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1. Pereira RM, Michalkiewicz E, Pianovski MA, França SN, Boguszewski MC, Cat I, Lacerda Filho Ld, Sandrini R: [Treatment of childhood adrenocortical tumor]. Arq Bras Endocrinol Metabol; 2005 Oct;49(5):747-52
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Treatment of childhood adrenocortical tumor].
  • [Transliterated title] Tratamento do tumor do córtex adrenal na infância.
  • Adrenocortical tumors (ACT) in children are uncommon.
  • However, the incidence of these tumors in Paraná is 15 times higher than that worldwide.
  • In our experience, disease stage I, absence of spillage during surgery and absence of intravenous thrombus are associated with better survival rates.
  • Preliminary data with the combination of etoposide, doxorubicin, cisplatin, and mitotane have shown that in some patients a complete remission is observed both of the tumor and metastasis.
  • Side effects due to these drugs are common and adrenal insufficiency may occur.
  • [MeSH-major] Adrenal Cortex Neoplasms. Adrenocortical Carcinoma
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Child, Preschool. Combined Modality Therapy. Female. Humans. Infant. Male. Neoplasm Staging. Prognosis. Survival Analysis

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  • (PMID = 16444357.001).
  • [ISSN] 0004-2730
  • [Journal-full-title] Arquivos brasileiros de endocrinologia e metabologia
  • [ISO-abbreviation] Arq Bras Endocrinol Metabol
  • [Language] por
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Brazil
  • [Number-of-references] 36
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2. Rosenkrantz AB, Do RK, Hajdu CH: Imaging appearance of bulk fat within an oncocytic adrenocortical neoplasm, a rare and potentially malignant tumour. Br J Radiol; 2010 Oct;83(994):e204-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Imaging appearance of bulk fat within an oncocytic adrenocortical neoplasm, a rare and potentially malignant tumour.
  • Oncocytic adrenocortical neoplasm is a rare adrenal tumour that usually follows a benign clinical course.
  • In some cases, however, these tumours have exhibited malignant behaviour.
  • Here, we present the first published case showing bulk fat within an oncocytic adrenocortical neoplasm on CT and MRI, a finding that mimics fat within an adrenal myelolipoma.
  • The distinction between these entities is important, as the current suggested management of an oncocytic adrenocortical neoplasm is resection with subsequent imaging surveillance.
  • [MeSH-major] Adenoma, Oxyphilic / diagnosis. Adrenal Cortex Neoplasms / diagnosis
  • [MeSH-minor] Diagnosis, Differential. Humans. Magnetic Resonance Imaging. Male. Middle Aged. Neoplasm Proteins / metabolism. Tomography, X-Ray Computed

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  • (PMID = 20846977.001).
  • [ISSN] 1748-880X
  • [Journal-full-title] The British journal of radiology
  • [ISO-abbreviation] Br J Radiol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Neoplasm Proteins
  • [Other-IDs] NLM/ PMC3473746
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3. Szajerka A, Dziegiel P, Szajerka T, Zabel M, Winowski J, Grzebieniak Z: Immunohistochemical evaluation of metallothionein, Mcm-2 and Ki-67 antigen expression in tumors of the adrenal cortex. Anticancer Res; 2008 Sep-Oct;28(5B):2959-65

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Immunohistochemical evaluation of metallothionein, Mcm-2 and Ki-67 antigen expression in tumors of the adrenal cortex.
  • BACKGROUND: The aim of this study was to assess the metallothionein (MT), maintenance protein 2 (Mcm-2) and Ki-67 expressions in adrenocortical adenomas and carcinomas in comparison to normal tissue and evaluate the correlations between these markers of proliferation and between these markers and tumor diameter.
  • MATERIALS AND METHODS: The expression of MT, Mcm-2 and Ki-67 was assessed by immunochemistry in forty-eight adrenocortical adenomas, six adrenocortical carcinomas and eleven normal adrenal cortex tissue samples.
  • RESULTS: The expressions of MT, Mcm-2 and Ki-67 in the adrenocortical carcinomas were significantly higher than in the adenomas and normal tissue (p<0.05).
  • The levels of Mcm-2 were also higher in the adrenocortical adenomas compared to the normal tissue (p<0.05).
  • The Mcm-2 expression showed a positive correlation to the expression of MT in the adrenocortical carcinomas (r=0.773; p<0.05) and to the expression of Ki-67 in the adrenocortical adenomas (r=0.432; p<0.05).
  • The malignant tumor diameter was positively correlated with the MT and Mcm-2 expressions (r=0.766, p<0.05 and r=0.620, p<0.05, respectively).
  • CONCLUSION: The assessment of Mcm-2 expression seems to be of special importance as a marker of adrenocortical dysplasia and a reliable indicator of malignancy in suspicious masses of the adrenal cortex.
  • [MeSH-major] Adrenal Cortex Neoplasms / metabolism. Adrenocortical Adenoma / metabolism. Adrenocortical Carcinoma / metabolism. Biomarkers, Tumor / biosynthesis. Cell Cycle Proteins / biosynthesis. Ki-67 Antigen / biosynthesis. Metallothionein / biosynthesis. Nuclear Proteins / biosynthesis

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  • (PMID = 19031940.001).
  • [ISSN] 0250-7005
  • [Journal-full-title] Anticancer research
  • [ISO-abbreviation] Anticancer Res.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Greece
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Cell Cycle Proteins; 0 / Ki-67 Antigen; 0 / Nuclear Proteins; 9038-94-2 / Metallothionein; EC 3.6.4.12 / MCM2 protein, human; EC 3.6.4.12 / Minichromosome Maintenance Complex Component 2
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4. Kabayegit OY, Soysal D, Oruk G, Ustaoglu B, Kosan U, Solmaz S, Avci A: Adrenocortical oncocytic neoplasm presenting with Cushing's syndrome: a case report. J Med Case Rep; 2008;2:228

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Adrenocortical oncocytic neoplasm presenting with Cushing's syndrome: a case report.
  • INTRODUCTION: Oncocytic neoplasms occur in several organs and are most commonly found in the thyroid, kidneys and salivary glands.
  • Oncocytic neoplasms of the adrenal cortex are extremely rare and are usually non-functioning.
  • CASE PRESENTATION: We report the case of an adrenocortical oncocytic neoplasm with uncertain malignant potential in a 31-year-old man with Cushing's syndrome.
  • The patient had been operated on following diagnosis of a 7 cm adrenal mass.
  • CONCLUSION: Adrenocortical oncocytic neoplasms must be considered in the differential diagnosis of both functioning and non-functioning adrenal masses.

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  • [Cites] Am J Surg Pathol. 1998 May;22(5):603-14 [9591731.001]
  • [Cites] Ann Diagn Pathol. 2005 Oct;9(5):295-7 [16198960.001]
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  • (PMID = 18620603.001).
  • [ISSN] 1752-1947
  • [Journal-full-title] Journal of medical case reports
  • [ISO-abbreviation] J Med Case Rep
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC2481265
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5. Takehara K, Sakai H, Shono T, Irie J, Kanetake H: Proliferative activity and genetic changes in adrenal cortical tumors examined by flow cytometry, fluorescence in situ hybridization and immunohistochemistry. Int J Urol; 2005 Feb;12(2):121-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Proliferative activity and genetic changes in adrenal cortical tumors examined by flow cytometry, fluorescence in situ hybridization and immunohistochemistry.
  • BACKGROUND: To determine differences in biological features among different adrenal tumors, we investigated the DNA ploidy, numerical chromosomal aberration and proliferative activity in human adrenal cortical neoplasms.
  • METHODS: Our study included six adrenal cortical adenomas with Cushing syndrome, 12 adenomas with hyperaldosteronism, three non-functioning adenomas and three adrenal cortical carcinomas.
  • RESULTS: The mean Ki-67 labeling index (LI) of adrenal cortical carcinomas was markedly higher than that of adrenal cortical adenomas (209.4 vs 8.7).
  • In functional adrenal cortical adenomas, the LI was significantly lower in adenomas with hyperaldosteronism than in those with Cushing syndrome (P = 0.004), although FCM results indicated that tetraploid patterns were more frequently observed in the former type.
  • Tumor size was significantly smaller in adenomas with hyperaldosteronism than in those with Cushing syndrome (P = 0.004).
  • Chromosome 17 showed disomy in all adrenal cortical adenomas, whereas chromosome 17 abnormalities were found in two of three adrenal cortical carcinomas.
  • CONCLUSIONS: Our study characterized various biological features of benign and malignant adrenal cortical tumors.
  • The use of a combination of markers might provide additional information to assist our understanding of the clinical behavior of an individual adrenal cortical tumor.
  • [MeSH-major] Adrenal Cortex Neoplasms / genetics. Adrenal Cortex Neoplasms / metabolism. Flow Cytometry. Immunohistochemistry. In Situ Hybridization, Fluorescence
  • [MeSH-minor] Adenoma / genetics. Adenoma / metabolism. Adenoma / pathology. Adult. Aged. Biomarkers, Tumor / genetics. Biomarkers, Tumor / metabolism. Carcinoma / genetics. Carcinoma / metabolism. Carcinoma / pathology. Cell Proliferation. Chromosomes, Human, Pair 17. Cushing Syndrome / genetics. Cushing Syndrome / metabolism. Cushing Syndrome / pathology. DNA, Neoplasm / genetics. Female. Humans. Hyperaldosteronism / genetics. Hyperaldosteronism / metabolism. Hyperaldosteronism / pathology. Ki-67 Antigen / metabolism. Male. Middle Aged. Ploidies. Tumor Suppressor Protein p53 / metabolism

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  • (PMID = 15733104.001).
  • [ISSN] 0919-8172
  • [Journal-full-title] International journal of urology : official journal of the Japanese Urological Association
  • [ISO-abbreviation] Int. J. Urol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Australia
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / DNA, Neoplasm; 0 / Ki-67 Antigen; 0 / Tumor Suppressor Protein p53
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6. Jarral OA, Todd C, Willson PD: Hemorrhagic shock secondary to spontaneous rupture of a non-secretory adrenal cortical tumour: A case report. Can Urol Assoc J; 2010 Dec;4(6):E161-3

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Hemorrhagic shock secondary to spontaneous rupture of a non-secretory adrenal cortical tumour: A case report.
  • After emergency laparotomy, angiography, embolisation and histological investigation, a diagnosis of spontaneous rupture of a benign non-secretory adrenal cortical tumour was made.
  • To our knowledge, this is the only reported case of this diagnosis.

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  • (PMID = 21749812.001).
  • [ISSN] 1920-1214
  • [Journal-full-title] Canadian Urological Association journal = Journal de l'Association des urologues du Canada
  • [ISO-abbreviation] Can Urol Assoc J
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Canada
  • [Other-IDs] NLM/ PMC3038376
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7. Jain M, Kapoor S, Mishra A, Gupta S, Agarwal A: Weiss criteria in large adrenocortical tumors: a validation study. Indian J Pathol Microbiol; 2010 Apr-Jun;53(2):222-6

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Weiss criteria in large adrenocortical tumors: a validation study.
  • BACKGROUND: Several systems including pathologic criteria alone or in combination with clinical features have been proposed to differentiate between benign and malignant adrenocortical tumors and assess their prognosis.
  • Since we see large adrenocortical carcinoma (ACC), we attempt to evaluate the diagnostic power of Weiss system in large ACC.
  • MATERIALS AND METHODS: In this study clinicopathological characteristics of 42 adrenocortical neoplasms are studied and classified into adrenocortical adenoma (ACA) and ACC based on Weiss score of less than or equal to three or greater than three.
  • RESULTS: The histological criteria of Weiss appeared to predict tumor prognosis accurately.
  • CONCLUSION: Weiss score was found to be a good prognostic factor for tumors of the adrenal cortex.
  • [MeSH-major] Adrenocortical Adenoma / diagnosis. Adrenocortical Adenoma / pathology. Adrenocortical Carcinoma / diagnosis. Adrenocortical Carcinoma / pathology. Histocytochemistry / methods
  • [MeSH-minor] Adolescent. Adult. Aged. Child. Child, Preschool. Diagnosis, Differential. Female. Humans. Infant. Male. Middle Aged. Prognosis. Young Adult

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  • (PMID = 20551521.001).
  • [ISSN] 0974-5130
  • [Journal-full-title] Indian journal of pathology & microbiology
  • [ISO-abbreviation] Indian J Pathol Microbiol
  • [Language] eng
  • [Publication-type] Evaluation Studies; Journal Article
  • [Publication-country] India
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8. Opocher G, Schiavi F, Cicala MV, Patalano A, Mariniello B, Boaretto F, Zovato S, Pignataro V, Macino B, Negro I, Mantero F: Genetics of adrenal tumors. Minerva Endocrinol; 2009 Jun;34(2):107-21
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  • [Title] Genetics of adrenal tumors.
  • Endocrinology pioneered the development of molecular medicine, but also the study of adrenal tumors had a great impact in this field.
  • Particularly important was the detection of genetics of tumors derived from the adrenal medulla, as well as that of those derived from the sympathetic and parasympathetic paraganglia.
  • Less well understood is the genetics of adrenal cortex tumors, in particular adrenocortical carcinoma, a rare and particularly aggressive disease.
  • There are only a few examples of hereditary transmission of adrenocortical carcinoma, but the analysis of low penetrance genes by genome wide association study may enable us to discover new genetic mechanisms responsible for adrenocortical-derived tumors.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Biomarkers, Tumor / genetics. Mutation. Pheochromocytoma / genetics
  • [MeSH-minor] Adrenal Cortex Neoplasms / genetics. Adrenocortical Carcinoma / genetics. Genetic Predisposition to Disease. Genomics. Humans. Neoplasm Proteins / genetics. Paraganglioma / genetics

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  • (PMID = 19471236.001).
  • [ISSN] 0391-1977
  • [Journal-full-title] Minerva endocrinologica
  • [ISO-abbreviation] Minerva Endocrinol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Neoplasm Proteins
  • [Number-of-references] 81
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9. Lau SK, Weiss LM: The Weiss system for evaluating adrenocortical neoplasms: 25 years later. Hum Pathol; 2009 Jun;40(6):757-68

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] The Weiss system for evaluating adrenocortical neoplasms: 25 years later.
  • The evaluation and categorization of adrenocortical neoplasms remain among the most challenging areas in adrenal pathology.
  • The Weiss system, first introduced 25 years ago, provides specific guidelines for differentiating adrenocortical adenoma from adrenocortical carcinoma and is considered the standard for determining malignancy in tumors of the adrenal cortex.
  • Considerable advances in the understanding of the pathology of adrenocortical neoplasia have occurred since delineation of the Weiss system, offering alternative approaches to the contemporary assessment of adrenocortical tumors.
  • [MeSH-major] Adrenal Cortex Neoplasms / pathology
  • [MeSH-minor] Adrenal Cortex / pathology. Adrenocortical Adenoma / pathology. Adrenocortical Carcinoma / pathology. Adult. Biopsy, Fine-Needle. Cell Nucleus / pathology. Child. Genes, Neoplasm. Humans. Immunohistochemistry. Mitosis. Necrosis. Neoplasm Invasiveness / pathology. Prognosis

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  • (PMID = 19442788.001).
  • [ISSN] 1532-8392
  • [Journal-full-title] Human pathology
  • [ISO-abbreviation] Hum. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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10. Palazzo FF, Sebag F, Sierra M, Ippolito G, Souteyrand P, Henry JF: Long-term outcome following laparoscopic adrenalectomy for large solid adrenal cortex tumors. World J Surg; 2006 May;30(5):893-8

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Long-term outcome following laparoscopic adrenalectomy for large solid adrenal cortex tumors.
  • INTRODUCTION: Laparoscopic adrenalectomy (LA) is the procedure of choice for small benign adrenal tumors.
  • In the absence of local invasion or metastases, the preoperative diagnosis of an adrenocortical carcinoma (ACC) is difficult, often leaving size as the principal predictor of malignancy.
  • Large tumors are resectable laparoscopically, but the long-term outcome and therefore appropriateness of LA for cortical tumors > 6 cm is not known.
  • METHODS: We reviewed the LA experience in our institution since its introduction in June 1994.
  • Patients who underwent LA for solid cortical tumors > or = 60 mm in diameter without preoperative or intraoperative evidence of malignancy were reviewed.
  • Follow-up data, including clinical examination, biochemical analysis, and repeat scans, were reviewed for evidence of local or systemic recurrent disease.
  • Among them, 19 were solid cortical tumors > or = 60 mm in diameter with no overt malignant preoperative or intraoperative characteristics: 9 nonsecreting tumors, 8 Cushing's syndrome tumors (including 2 virilizing variants), 1 virilizing tumor, and 1 aldosteronoma.
  • The mean age of the patients was 49.9 years (range 22-77 years), and the mean tumor size was 69.0 mm (range 60-80 mm).
  • Histology confirmed a cortical adenoma in eight patients, malignant tumors in three, and indeterminate tumors in eight.
  • Two patients died of systemic recurrent disease (liver metastases) at 10 and 19 months, respectively, following surgery; two other patients died 12 and 21 months, respectively following surgery owing to unrelated cardiovascular and cerebrovascular pathology.
  • One patient underwent surgery for local recurrence 54 months after primary surgery; the remaining 14 patients are well with no clinical or radiologic evidence of recurrent disease.
  • CONCLUSIONS: Laparoscopic adrenalectomy for large solid cortical tumors without pre- or intraoperative evidence of malignancy is not contraindicated, and it is unlikely to have a deleterious effect on long-term outcome.
  • We provide an algorithm for the approach to adrenocortical tumors > or = 6 cm.
  • [MeSH-major] Adrenal Cortex Neoplasms / surgery. Adrenalectomy. Adrenocortical Adenoma / surgery. Adrenocortical Carcinoma / surgery
  • [MeSH-minor] Adult. Aged. Algorithms. Humans. Laparoscopy. Middle Aged. Neoplasm Staging. Retrospective Studies. Time Factors. Treatment Outcome

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  • (PMID = 16680605.001).
  • [ISSN] 0364-2313
  • [Journal-full-title] World journal of surgery
  • [ISO-abbreviation] World J Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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11. Al-Zaid T, Alroy J, Pfannl R, Strissel KJ, Powers JF, Layer A, Carpinito G, Tischler AS: Oncocytic adrenal cortical tumor with cytoplasmic inclusions and hyaline globules. Virchows Arch; 2008 Sep;453(3):301-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Oncocytic adrenal cortical tumor with cytoplasmic inclusions and hyaline globules.
  • Adrenal cortical tumors, particularly oncocytic tumors, have been reported to contain a variety of intracytoplasmic and intramitochondrial inclusions.
  • Oncocytic cortical tumors can also morphologically mimic pheochromocytomas.
  • We report an unusual, partially oncocytic cortical neoplasm with nesting architecture, intranuclear inclusions, and hyaline globules reminiscent of pheochromocytoma, together with numerous, small, brightly eosinophilic, periodic acid-Schiff-positive cytoplasmic inclusions and typical cytoplasmic lipid droplets.
  • Immunohistochemistry and immunoblots were utilized to further characterize the tumor.
  • Immunohistochemistry demonstrated immunoreactivity of both the eosinophilic inclusions and the hyaline globules for adipose differentiation-related protein (ADRP), which is one of a group of proteins associated with storage of neutral lipids in many cell types.
  • Immunoblots confirmed the presence of ADRP and demonstrated an imbalance between ADRP and perilipin, another neutral lipid-associated protein, in tumor tissue compared to normal adrenal cortex.
  • The findings suggest that mitochondrial dysfunction in oncocytic cortical tumors may lead to abnormal processing of proteins related to the lipid-storing functions of the adrenal cortex, resulting in unusual cytoplasmic inclusions and extracellular globules resembling the globules in pheochromocytomas.
  • The finding of ADRP as a constituent of inclusions in adrenal cortical tumors has not been previously reported.
  • [MeSH-major] Adrenal Cortex Neoplasms / pathology. Inclusion Bodies / pathology. Oxyphil Cells / pathology
  • [MeSH-minor] Adrenal Cortex / pathology. Humans. Hyalin / metabolism. Male. Membrane Proteins / metabolism. Microscopy, Electron. Middle Aged. Perilipin-2. Pheochromocytoma / pathology

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  • (PMID = 18688642.001).
  • [ISSN] 0945-6317
  • [Journal-full-title] Virchows Archiv : an international journal of pathology
  • [ISO-abbreviation] Virchows Arch.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Membrane Proteins; 0 / PLIN2 protein, human; 0 / Perilipin-2
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12. Pianovski MA, Maluf EM, de Carvalho DS, Ribeiro RC, Rodriguez-Galindo C, Boffetta P, Zancanella P, Figueiredo BC: Mortality rate of adrenocortical tumors in children under 15 years of age in Curitiba, Brazil. Pediatr Blood Cancer; 2006 Jul;47(1):56-60
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Mortality rate of adrenocortical tumors in children under 15 years of age in Curitiba, Brazil.
  • BACKGROUND: Several reports refer to an increased frequency of adrenal cortex tumors (ACT) among children in Southern Brazil, yet all data have been derived from hospital-based registries.
  • PROCEDURE: We reviewed all death certificates that mentioned ACT or adrenal neuroblastoma (NB) and which were reported to the Paraná State Department of Health between 1998 and 2003, for individuals younger than 15 years who resided in the Curitiba metropolitan region.
  • The ratio of the adrenal NB and ACT age-adjusted mortality rates was 1.43.
  • CONCLUSIONS: Our investigation of population-based mortality confirms the evidence from hospital-based registries of a clustering of ACT in Southern Brazil.
  • [MeSH-major] Adrenal Cortex Neoplasms / mortality

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  • [Copyright] Copyright 2006 Wiley-Liss, Inc.
  • (PMID = 16200634.001).
  • [ISSN] 1545-5009
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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13. Gross MD, Gauger PG, Djekidel M, Rubello D: The role of PET in the surgical approach to adrenal disease. Eur J Surg Oncol; 2009 Nov;35(11):1137-45
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  • [Title] The role of PET in the surgical approach to adrenal disease.
  • BACKGROUND: Appropriate surgical approach to diseases of the adrenal requires a diagnosis sufficient to determine the biochemical status of adrenal dysfunction and anatomic evaluation sufficient to differentiate unilateral from bilateral disease, intra-adrenal from extra-adrenal neoplasm, adrenal tumor recurrence or adrenal metastases.
  • High resolution computed tomography (CT) and magnetic resonance have been the primary imaging modalities for the evaluation of anatomy, while scintigraphic studies have played a secondary role in diagnosis.
  • The recent availability of functional imaging provided by positron emission tomography (PET) with radiopharmaceuticals designed to depict substrate precursor uptake, cellular metabolism or receptor binding in neoplasms and CT as a single modality, hybrid PET/CT, to directly correlate function and anatomy has had a significant impact upon the diagnostic and therapeutic approach to many cancers and has been applied to adrenal disease with some early success that we describe in this review.
  • METHODS: In addition to the authors' experience, a search of Medline and PubMed databases was performed using search terms: 'adrenal scintigraphy', 'positron tomography', 'computed tomography', 'adrenal surgery', 'adrenal mass', '(18)F-fluorodeoxyglucose', 'adrenal carcinoma', 'adrenal medulla' and 'pheochromocytoma'.
  • CONCLUSIONS: Present PET radiopharmaceuticals and their use in hybrid PET/CT have demonstrated efficacy in the preoperative and follow-up evaluation of neoplasms of the adrenal cortex and medulla that hopefully will continue to improve with the development of newer tracers that continue to exploit unusual characteristics of the adrenals.
  • [MeSH-major] Adrenal Gland Neoplasms / radionuclide imaging. Adrenal Gland Neoplasms / surgery. Radiopharmaceuticals. Tomography, Emission-Computed
  • [MeSH-minor] Diagnosis, Differential. Humans. Tomography, X-Ray Computed

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  • (PMID = 19243910.001).
  • [ISSN] 1532-2157
  • [Journal-full-title] European journal of surgical oncology : the journal of the European Society of Surgical Oncology and the British Association of Surgical Oncology
  • [ISO-abbreviation] Eur J Surg Oncol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Radiopharmaceuticals
  • [Number-of-references] 75
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14. Carden CP, Frentzas S, Langham M, Casamayor I, Stephens AW, Poondru S, Wheaton J, Lippman SM, Kaye SB, Kim ES: Preliminary activity in adrenocortical tumor (ACC) in phase I dose escalation study of intermittent oral dosing of OSI-906, a small-molecule insulin-like growth factor-1 receptor (IGF-1R) tyrosine kinase inhibitor in patients with advanced solid tumors. J Clin Oncol; 2009 May 20;27(15_suppl):3544

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  • [Title] Preliminary activity in adrenocortical tumor (ACC) in phase I dose escalation study of intermittent oral dosing of OSI-906, a small-molecule insulin-like growth factor-1 receptor (IGF-1R) tyrosine kinase inhibitor in patients with advanced solid tumors.
  • IGF-1R blockade increases apoptosis and reduces tumor growth in preclinical models.
  • METHODS: Patients (pt) with advanced solid tumours were enrolled to determine safety, tolerability, maximum tolerated dose, pharmacokinetics (PK), pharmacodynamics (PD) and preliminary anti-tumor activity.

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  • (PMID = 27961354.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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15. Gonzalez RJ, Shapiro S, Sarlis N, Vassilopoulou-Sellin R, Perrier ND, Evans DB, Lee JE: Laparoscopic resection of adrenal cortical carcinoma: a cautionary note. Surgery; 2005 Dec;138(6):1078-85; discussion 1085-6
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  • [Title] Laparoscopic resection of adrenal cortical carcinoma: a cautionary note.
  • BACKGROUND: While laparoscopic removal of small, benign, functioning adrenal tumors is accepted, laparoscopic resection of adrenal tumors that may be adrenal cortical carcinoma (ACC) remains controversial.
  • Open adrenalectomy remains the standard of care for patients presenting with an adrenal cortical tumor for which ACC is in the differential diagnosis.
  • [MeSH-major] Adrenal Cortex Neoplasms / surgery. Adrenalectomy. Carcinoma / surgery. Laparoscopy. Neoplasm Recurrence, Local / epidemiology

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  • (PMID = 16360394.001).
  • [ISSN] 0039-6060
  • [Journal-full-title] Surgery
  • [ISO-abbreviation] Surgery
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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16. Komissarenko IV, Rybakov SI, Kvacheniuk AN, Lazar' SI, Fedorova TI, Kovalenko AE, Mel'nik ND, Negrienko KV: [The role of computer tomography in differential diagnosis of benign and malignant tumors of the adrenals cortex]. Klin Khir; 2005 Jul;(7):42-5

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  • [Title] [The role of computer tomography in differential diagnosis of benign and malignant tumors of the adrenals cortex].
  • Possibilities of computer tomography (CT) application for differential diagnosis of tumors of the adrenal cortex (TAC) were studied.
  • While the trustworthy signs of malignancy are absent, it is necessary to conduct morphological verification of the adrenal tumors nature using puncture biopsy and/or intraoperative express-cytological investigation.

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  • (PMID = 16255222.001).
  • [ISSN] 0023-2130
  • [Journal-full-title] Klinichna khirurhiia
  • [ISO-abbreviation] Klin Khir
  • [Language] RUS
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Ukraine
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17. Ranganathan S, Lynshue K, Hunt JL, Kane T, Jaffe R: Unusual adrenal cortical tumor of unknown biologic potential: a nodule in a nodule in a nodule. Pediatr Dev Pathol; 2005 Jul-Aug;8(4):483-8
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  • [Title] Unusual adrenal cortical tumor of unknown biologic potential: a nodule in a nodule in a nodule.
  • Adrenocortical tumors are uncommon neoplasms in childhood.
  • Most pediatric adrenal tumors are virilizing and carcinomas are more common than adenomas.
  • Recent molecular data suggest an adenoma-to-carcinoma progression sequence in adrenal cortical neoplasms.
  • We report a case of a 5-year-old boy who presented with virilizing symptoms secondary to an adrenal tumor that was resected laparoscopically.
  • The bulk of the tumor was a large, yellow mass with typical features of an adrenal cortical adenoma.
  • [MeSH-major] Adrenal Cortex Neoplasms / pathology. Adrenocortical Adenoma / pathology
  • [MeSH-minor] Adrenalectomy. Child, Preschool. Cushing Syndrome / etiology. Cushing Syndrome / pathology. DNA, Neoplasm / analysis. Hirsutism / etiology. Hirsutism / pathology. Humans. Loss of Heterozygosity. Male. Neoplasms, Multiple Primary / complications. Neoplasms, Multiple Primary / genetics. Neoplasms, Multiple Primary / pathology. Polymerase Chain Reaction. Treatment Outcome

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  • (PMID = 16010500.001).
  • [ISSN] 1093-5266
  • [Journal-full-title] Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society
  • [ISO-abbreviation] Pediatr. Dev. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / DNA, Neoplasm
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18. Hantel C, Beuschlein F: Mouse models of adrenal tumorigenesis. Best Pract Res Clin Endocrinol Metab; 2010 Dec;24(6):865-75
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  • [Title] Mouse models of adrenal tumorigenesis.
  • Adrenocortical carcinomas (ACCs) are heterogeneous tumors with a poor prognosis.
  • The rarity of this disorder causes a lack of treatment experience and material availability which is necessary to optimize existing treatments and to develop novel therapeutic strategies.
  • Although surgery is still the treatment of choice, adjuvant therapies are urgently needed as the rate of recurrence for these tumors is high.
  • In recent years molecular characterization of surgical tumor specimen has aided in the understanding of disease mechanisms and definition of therapeutic targets also in adrenocortical carcinoma.
  • Here we give an overview on rodent models that have been described to either have adrenocortical tumors as part of their phenotype or have been utilized for therapeutic screens as adrenocortical tumor models.
  • [MeSH-major] Adrenal Cortex Neoplasms / therapy. Adrenal Gland Neoplasms / physiopathology. Adrenal Gland Neoplasms / therapy. Disease Models, Animal
  • [MeSH-minor] Animals. Animals, Genetically Modified. Mice. Neoplasm Transplantation

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  • [Copyright] 2010 Elsevier Ltd. All rights reserved.
  • (PMID = 21115155.001).
  • [ISSN] 1878-1594
  • [Journal-full-title] Best practice & research. Clinical endocrinology & metabolism
  • [ISO-abbreviation] Best Pract. Res. Clin. Endocrinol. Metab.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Netherlands
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19. Subhawong AP, Subhawong TK, Li QK: Fine needle aspiration of metastatic prostate carcinoma simulating a primary adrenal cortical neoplasm: a case report and review of the literature. Diagn Cytopathol; 2010 Feb;38(2):147-53
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  • [Title] Fine needle aspiration of metastatic prostate carcinoma simulating a primary adrenal cortical neoplasm: a case report and review of the literature.
  • Adrenal metastases usually occur in prostate cancer patients with widespread bone and visceral disease.
  • Autopsy studies have shown that adrenal metastases may be found in up to 23% of these patients.
  • However, the finding of an isolated adrenal metastasis without the involvement of other organs in a patient with prostate cancer is exceedingly rare.
  • We report a patient with a history of prostate cancer, status post radiation, and hormonal therapy 4 years before, who presented with a new, single adrenal mass on abdominal imaging studies.
  • The ultrasound-guided FNA cytology of the adrenal mass revealed cytomorphological features that were suggestive of a primary adrenal cortical neoplasm, but overlapped with those of a prostate metastasis.
  • To our knowledge, FNA findings of metastatic prostate cancer simulating an adrenal cortical neoplasm have not been previously reported in the English literature.
  • The purpose of our study is to discuss the differential diagnosis of these entities.
  • The accurate diagnosis is important because of different prognosis and treatment implications for the various diseases.
  • [MeSH-major] Adenocarcinoma / secondary. Adrenal Cortex Neoplasms / secondary. Biopsy, Fine-Needle. Prostatic Neoplasms / pathology
  • [MeSH-minor] Aged. Antineoplastic Agents, Hormonal. Diagnosis, Differential. Humans. Immunohistochemistry. Male. Prostate-Specific Antigen / blood. Radiotherapy. Tomography, X-Ray Computed. Ultrasonography, Interventional

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  • (PMID = 19693937.001).
  • [ISSN] 1097-0339
  • [Journal-full-title] Diagnostic cytopathology
  • [ISO-abbreviation] Diagn. Cytopathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Hormonal; EC 3.4.21.77 / Prostate-Specific Antigen
  • [Number-of-references] 29
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20. Collins BR: Endocrine diseases of rodents. Vet Clin North Am Exot Anim Pract; 2008 Jan;11(1):153-62, vii-viii
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  • The clinical diagnosis of endocrine diseases almost never occurs in free-ranging animals in their native habitat.
  • Feral animals that have clinical endocrine disease, such as neoplasia, adrenal cortical hyperplasia, or diabetes, would exhibit clinical signs of altered behavior that would result in their removal by predators.
  • The diagnosis of endocrine disease thus takes place in the relatively protective environment of captivity.
  • [MeSH-major] Animals, Domestic. Endocrine System Diseases / veterinary. Rodent Diseases / diagnosis
  • [MeSH-minor] Animals. Cricetinae. Diagnosis, Differential. Female. Gerbillinae. Guinea Pigs. Male. Mice. Rats. Rodentia. Species Specificity

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  • (PMID = 18165143.001).
  • [ISSN] 1094-9194
  • [Journal-full-title] The veterinary clinics of North America. Exotic animal practice
  • [ISO-abbreviation] Vet Clin North Am Exot Anim Pract
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 39
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21. Welsh SJ, Khan S: Radiological localizing techniques in adrenal tumors. Minerva Endocrinol; 2009 Jun;34(2):161-9
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  • [Title] Radiological localizing techniques in adrenal tumors.
  • The characterisation of adrenal lesions is a common radiological dilemma.
  • Incidental adrenal lesions are commonly detected with computed tomography (CT), and lesion characterisation is critical.
  • The prevalence of incidental adrenal lesions has been reported to be 2.3% at autopsy and 0.5-2% with abdominal CT.
  • Although the majority of adrenal lesions are benign, in patients with an extra-adrenal primary cancer the probability of an adrenal mass being a metastasis is 52%.
  • Unfortunately, there may be significant overlap between the imaging appearances of benign lesions such as lipid-poor adenomas and malignant lesions, particularly metastases and small adrenal carcinomas.
  • This review highlights recent advances in radiological imaging of adrenal lesions and we discuss the relative merits of CT and magnetic resonance imaging to aid the identification of benign and malignant adrenal lesions and their roles, in combination with biochemical and clinical data, in recognizing common pathologies such as adrenal adenoma, phaeochromocytoma, carcinoma and metastases.
  • We also discuss the radiological characteristics of rarer adrenal lesions including lymphoma, neuroblastic tumours (neuroblastoma, ganglioneuroblastoma, and ganglioneuroma), lipomatous tumours (myelolipoma, angiolipoma, teratoma, lipoma and liposarcoma), in addition to hemangioma, hemangiosarcoma and leiomyosarcoma.
  • [MeSH-major] Adrenal Gland Neoplasms / radiography. Incidental Findings. Tomography, X-Ray Computed
  • [MeSH-minor] Adrenal Cortex Neoplasms / radiography. Adrenocortical Adenoma / radiography. Adrenocortical Carcinoma / radiography. Diagnosis, Differential. Ganglioneuroblastoma / radiography. Ganglioneuroma / radiography. Humans. Lymphoma / radiography. Magnetic Resonance Imaging. Neoplasm Metastasis. Neoplasms, Adipose Tissue / radiography. Neoplasms, Vascular Tissue / radiography. Neuroblastoma / radiography. Pheochromocytoma / radiography. Predictive Value of Tests. Sensitivity and Specificity. Teratoma / radiography

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  • (PMID = 19471240.001).
  • [ISSN] 0391-1977
  • [Journal-full-title] Minerva endocrinologica
  • [ISO-abbreviation] Minerva Endocrinol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Italy
  • [Number-of-references] 42
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22. Stephenson TJ: Prognostic and predictive factors in endocrine tumours. Histopathology; 2006 May;48(6):629-43
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  • [Title] Prognostic and predictive factors in endocrine tumours.
  • This review encompasses the diagnostic features of malignancy, the routinely observable prognostic features and the prognostic and predictive features emerging from research techniques in the principal endocrine neoplasms: pancreatic and extrapancreatic endocrine cell tumours, thyroid and parathyroid neoplasia, adrenal cortical neoplasms and adrenal and extra-adrenal paragangliomas.
  • While each endocrine tissue has its own set of diagnostic features for malignancy, and prognostic features once a diagnosis of malignancy has been established, there are a few common themes.
  • For several endocrine neoplasms, definite recognition of malignancy can be difficult and may depend upon frank invasive or metastatic growth at presentation.
  • The accurate documentation of routinely observable histological features interpreted in the light of current literature has not been superseded by special techniques in the statement of diagnosis or prognosis in the vast majority of endocrine neoplasms.
  • [MeSH-major] Endocrine Gland Neoplasms / pathology. Peptide Hormones / analysis
  • [MeSH-minor] Diagnosis, Differential. Glucagon / analysis. Humans. Insulin / analysis. Predictive Value of Tests. Prognosis

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  • (PMID = 16681678.001).
  • [ISSN] 0309-0167
  • [Journal-full-title] Histopathology
  • [ISO-abbreviation] Histopathology
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Insulin; 0 / Peptide Hormones; 9007-92-5 / Glucagon
  • [Number-of-references] 123
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23. Khan AA, Shergill IS, Hamid R, Gujral SS: Giant nonfunctioning carcinoma of the adrenal cortex mimicking renal cell carcinoma: a diagnostic dilemma. Urology; 2007 Jul;70(1):178.e1-2
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  • [Title] Giant nonfunctioning carcinoma of the adrenal cortex mimicking renal cell carcinoma: a diagnostic dilemma.
  • Adrenocortical carcinoma is a rare, highly malignant neoplasm that originates in the adrenal cortex and is difficult to differentiate from renal cell carcinoma, especially if it is gigantic and nonfunctional.
  • We report the case of a 40-year-old man with an incidental mass in the right upper abdomen.
  • However, histologic examination after radical nephrectomy confirmed the mass to be an adrenocortical carcinoma compressing the kidney.
  • We discuss the obscurity and implications of such a diagnosis.
  • [MeSH-major] Adrenal Cortex Neoplasms / diagnosis. Adrenocortical Carcinoma / diagnosis. Carcinoma, Renal Cell / diagnosis. Diagnostic Errors. Kidney Neoplasms / diagnosis
  • [MeSH-minor] Adult. Diagnosis, Differential. Humans. Male

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  • (PMID = 17656234.001).
  • [ISSN] 1527-9995
  • [Journal-full-title] Urology
  • [ISO-abbreviation] Urology
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 9
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24. Gut P, Huber K, Lohr J, Brühl B, Oberle S, Treier M, Ernsberger U, Kalcheim C, Unsicker K: Lack of an adrenal cortex in Sf1 mutant mice is compatible with the generation and differentiation of chromaffin cells. Development; 2005 Oct;132(20):4611-9
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  • [Title] Lack of an adrenal cortex in Sf1 mutant mice is compatible with the generation and differentiation of chromaffin cells.
  • The diversification of neural-crest-derived sympathoadrenal (SA) progenitor cells into sympathetic neurons and neuroendocrine adrenal chromaffin cells was thought to be largely understood.
  • In-vitro studies with isolated SA progenitor cells had suggested that chromaffin cell differentiation depends crucially on glucocorticoids provided by adrenal cortical cells.
  • However, analysis of mice lacking the glucocorticoid receptor gene had revealed that adrenal chromaffin cells develop mostly normally in these mice.
  • Alternative cues from the adrenal cortex that may promote chromaffin cell determination and differentiation have not been identified.
  • We therefore investigated whether the chromaffin cell phenotype can develop in the absence of an adrenal cortex, using mice deficient for the nuclear orphan receptor steroidogenic factor-1 (SF1), which lack adrenal cortical cells and gonads.
  • However, already at E12.5 the 'adrenal' region in Sf1-/- mice is occupied by fewer PHOX2B+ and TH+ SA cells as well as SOX10+ neural crest cells.
  • Our results suggest that cortical cues are not essential for determining chromaffin cell fate, but may be required for proper migration of SA progenitors to and/or colonization of the adrenal anlage.
  • [MeSH-major] Adrenal Cortex / cytology. Adrenal Cortex / metabolism. Cell Differentiation. Chromaffin Cells / cytology. Chromaffin Cells / metabolism. Homeodomain Proteins / genetics. Homeodomain Proteins / metabolism. Receptors, Cytoplasmic and Nuclear / genetics. Receptors, Cytoplasmic and Nuclear / metabolism. Transcription Factors / genetics. Transcription Factors / metabolism
  • [MeSH-minor] Animals. Gene Expression Regulation, Developmental. High Mobility Group Proteins / metabolism. Mice. Mice, Knockout. Microscopy, Electron. Mutation / genetics. Neoplasm Proteins / metabolism. SOXE Transcription Factors. Steroidogenic Factor 1

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  • (PMID = 16176945.001).
  • [ISSN] 0950-1991
  • [Journal-full-title] Development (Cambridge, England)
  • [ISO-abbreviation] Development
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / High Mobility Group Proteins; 0 / Homeodomain Proteins; 0 / NBPhox protein; 0 / Neoplasm Proteins; 0 / Receptors, Cytoplasmic and Nuclear; 0 / SOXE Transcription Factors; 0 / Sox10 protein, mouse; 0 / Steroidogenic Factor 1; 0 / Transcription Factors; 0 / steroidogenic factor 1, mouse
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25. Fulmer BR: Diagnosis and management of adrenal cortical carcinoma. Curr Urol Rep; 2007 Jan;8(1):77-82

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Diagnosis and management of adrenal cortical carcinoma.
  • Adrenal cortical carcinoma is a relatively uncommon malignancy that represents a significant clinical challenge for the development of optimal treatment strategies.
  • Although the framework of a successful treatment paradigm still relies on these steps, advances in diagnostic imaging have led to increased accuracy in diagnosis, and advances in laparoscopic surgical technique have served to reduce morbidity for patients facing treatment.
  • This review focuses on a discussion of advances in modalities for the diagnosis and treatment of adrenal cortical carcinoma amenable to curative therapy.
  • Patients that present with metastatic or locally advanced disease generally are treated with mitotane-based chemotherapy with or without the addition of cytotoxic drugs.
  • Contemporary results of this treatment approach are presented in this review as well as a discussion of further directions for the treatment of patients with advanced disease.
  • [MeSH-major] Adrenal Cortex Neoplasms / diagnosis. Adrenal Cortex Neoplasms / therapy. Adrenocortical Carcinoma / diagnosis. Adrenocortical Carcinoma / therapy
  • [MeSH-minor] Adrenalectomy / methods. Chemotherapy, Adjuvant. Combined Modality Therapy. Female. Humans. Laparoscopy / methods. Male. Neoplasm Staging. Prognosis. Radiotherapy, Adjuvant. Risk Assessment. Survival Analysis

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  • (PMID = 17239320.001).
  • [ISSN] 1534-6285
  • [Journal-full-title] Current urology reports
  • [ISO-abbreviation] Curr Urol Rep
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 50
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26. Berthon A, Sahut-Barnola I, Lambert-Langlais S, de Joussineau C, Damon-Soubeyrand C, Louiset E, Taketo MM, Tissier F, Bertherat J, Lefrançois-Martinez AM, Martinez A, Val P: Constitutive beta-catenin activation induces adrenal hyperplasia and promotes adrenal cancer development. Hum Mol Genet; 2010 Apr 15;19(8):1561-76
SciCrunch. OMIM: Data: Gene Annotation .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Constitutive beta-catenin activation induces adrenal hyperplasia and promotes adrenal cancer development.
  • Adrenocortical carcinoma is a rare but aggressive cancer with unknown aetiology.
  • Constitutive activation of beta-catenin is the most frequent alteration in benign and malignant adrenocortical tumours in patients.
  • Here, we show that constitutive activation of beta-catenin in the adrenal cortex of transgenic mice resulted in progressive steroidogenic and undifferentiated spindle-shaped cells hyperplasia as well as dysplasia of the cortex and medulla.
  • Altogether these observations demonstrate that constitutively active beta-catenin is an adrenal oncogene which triggers benign aldosterone-secreting tumour development and promotes malignancy.
  • [MeSH-major] Adrenal Cortex / pathology. Adrenal Gland Neoplasms / metabolism. Adrenal Gland Neoplasms / pathology. beta Catenin / metabolism
  • [MeSH-minor] Aldosterone / metabolism. Animals. Cell Proliferation. Disease Models, Animal. Humans. Hyperplasia. Mice. Mice, Inbred C57BL. Mice, Transgenic. Neoplasm Metastasis


27. Matsumoto K, Egawa S, Satoh T, Okuno N, Kaseda S, Baba S: Thoracoscopic transdiaphragmatic adrenalectomy for isolated locally recurrent adrenal carcinoma. Int J Urol; 2005 Dec;12(12):1055-7

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Thoracoscopic transdiaphragmatic adrenalectomy for isolated locally recurrent adrenal carcinoma.
  • A 58-year-old man who had undergone left adrenalectomy 2 years previously for adrenocortical carcinoma was diagnosed to have a left suprarenal solid mass.
  • Thoracoscopic transdiaphragmatic excision of the tumor was conducted under the diagnosis of isolated local recurrence of adrenal carcinoma.
  • There have been no signs of tumor recurrence during 3 years follow up after surgery.
  • [MeSH-major] Adrenal Cortex Neoplasms / surgery. Adrenalectomy / methods. Adrenocortical Carcinoma / surgery. Neoplasm Recurrence, Local / surgery. Thoracoscopy

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  • (PMID = 16409610.001).
  • [ISSN] 0919-8172
  • [Journal-full-title] International journal of urology : official journal of the Japanese Urological Association
  • [ISO-abbreviation] Int. J. Urol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Australia
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28. Xiao ZJ, Li CL: [Combined therapy of advanced adrenal cortical adenocarcinoma]. Zhonghua Yi Xue Za Zhi; 2010 Aug 10;90(30):2123-5

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Combined therapy of advanced adrenal cortical adenocarcinoma].
  • OBJECTIVE: To analyze the clinical efficacy of combined therapy in the treatment of advanced adrenal cortical adenocarcinoma.
  • METHODS: The clinical data of 12 cases with advanced adrenal cortical adenocarcinoma at our hospital from 1986 - 2006 were analyzed.
  • Pathological diagnosis was all of adrenal cortical adenocarcinoma.
  • According to evaluation criterion of chemotherapeutic effect by WHO in 1987, the results were: CR (complete remission) (n = 0), PR (partial remission) (n = 7), SD (stable disease) (n = 3) and PD (progressive disease) (n = 2).
  • CONCLUSION: Combined therapy of adrenal cortical adenocarcinoma is effective to prolong the patient lifespan.
  • Making an early diagnosis and offering a novel therapy yield a better outcome.
  • [MeSH-major] Adrenal Cortex Neoplasms / pathology. Adrenal Cortex Neoplasms / therapy. Adrenocortical Carcinoma / pathology. Adrenocortical Carcinoma / therapy
  • [MeSH-minor] Adult. Combined Modality Therapy. Female. Humans. Male. Middle Aged. Neoplasm Staging. Retrospective Studies. Treatment Outcome

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  • (PMID = 21029628.001).
  • [ISSN] 0376-2491
  • [Journal-full-title] Zhonghua yi xue za zhi
  • [ISO-abbreviation] Zhonghua Yi Xue Za Zhi
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
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29. Paton BL, Novitsky YW, Zerey M, Harrell AG, Norton HJ, Asbun H, Kercher KW, Heniford BT: Outcomes of adrenal cortical carcinoma in the United States. Surgery; 2006 Dec;140(6):914-20; discussion 919-20
NCI CPTAC Assay Portal. NCI CPTAC Assay Portal .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Outcomes of adrenal cortical carcinoma in the United States.
  • BACKGROUND: Improvements in the sensitivity of radiographic imaging have lead to an increase in the number of adrenal masses diagnosed.
  • The purpose of this study is to determine if technologic advancements have resulted in the diagnosis of earlier-staged adrenal cortical cancer (ACC) and to determine if the survival of patients with ACC has improved over the past 15 years.
  • RESULTS: We identified 602 patients with a mean age of 53 years and an average tumor size of 11.8 cm.
  • Two hundred thirty-eight (39.5%) patients presented with localized disease (stages I and II), and 311 (52%) patients presented with advanced disease (stages III and IV).
  • Patients with masses less than 5 cm were statistically more likely to have localized disease (P <. 001).
  • Age (P = .10), tumor size (P = .85), tumor stage (P = .45), and 5-year survival (P = .5) did not change over the 15-year study.
  • CONCLUSIONS: Over the 15-year study, patients with ACC were not diagnosed at an earlier stage or with tumors smaller, and survival did not improve.
  • [MeSH-major] Adrenal Cortex Neoplasms / diagnosis. Adrenal Cortex Neoplasms / pathology. Neoplasm Staging. SEER Program / statistics & numerical data
  • [MeSH-minor] Adrenal Glands / pathology. Adult. Aged. Aged, 80 and over. Female. Humans. Male. Middle Aged. Prognosis. Survival Rate. United States / epidemiology

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  • (PMID = 17188138.001).
  • [ISSN] 0039-6060
  • [Journal-full-title] Surgery
  • [ISO-abbreviation] Surgery
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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30. Komorowski J, Jurczynska J, Stepien T, Kolomecki K, Kuzdak K, Stepien H: Serum concentrations of TNF α and its soluble receptors in patients with adrenal tumors treated by surgery. Int J Mol Sci; 2010;11(6):2281-90
NCI CPTC Antibody Characterization Program. NCI CPTC Antibody Characterization Program .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Serum concentrations of TNF α and its soluble receptors in patients with adrenal tumors treated by surgery.
  • The peripheral blood levels of TNF alpha and its soluble receptors were studied in 39 patients with malignant and benign adrenal tumors treated by adrenalectomy.
  • The concentrations of TNF alpha were significantly elevated in patients with malignant tumors of the adrenal cortex and in patients with Conn's syndrome compared to control.
  • After adrenalectomy, the levels of TNF alpha were decreased in patients with malignant tumors and in patients with Conn's syndrome, nonfunctioniong adenomas and pheochromocytomas compared to the concentration before surgery.
  • However, to confirm practicality of the evaluation of TNF alpha and its soluble receptors in differential diagnosis in patients with adrenal tumors, a larger study group is needed.
  • [MeSH-major] Adrenal Gland Neoplasms / blood. Adrenal Gland Neoplasms / surgery. Receptors, Tumor Necrosis Factor / blood. Tumor Necrosis Factor-alpha / blood
  • [MeSH-minor] Adrenalectomy. Adult. Aged. Aged, 80 and over. Female. Humans. Male. Middle Aged. Receptors, Tumor Necrosis Factor, Type I / blood. Receptors, Tumor Necrosis Factor, Type II / blood

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  • (PMID = 20640152.001).
  • [ISSN] 1422-0067
  • [Journal-full-title] International journal of molecular sciences
  • [ISO-abbreviation] Int J Mol Sci
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / Receptors, Tumor Necrosis Factor; 0 / Receptors, Tumor Necrosis Factor, Type I; 0 / Receptors, Tumor Necrosis Factor, Type II; 0 / Tumor Necrosis Factor-alpha
  • [Other-IDs] NLM/ PMC2904916
  • [Keywords] NOTNLM ; TNF α / TNF α R1 / TNF α R2 / adrenal tumors / adrenalectomy
  • [General-notes] NLM/ Original DateCompleted: 20110714
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31. Gruschwitz T, Breza J, Wunderlich H, Junker K: Improvement of histopathological classification of adrenal gland tumors by genetic differentiation. World J Urol; 2010 Jun;28(3):329-34

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Improvement of histopathological classification of adrenal gland tumors by genetic differentiation.
  • PURPOSE: There are often problems in differentiating between benign and malignant adrenal gland tumors by imaging and histopathology.
  • On account of considerable differences in the therapy and aftercare of benign and malignant adrenal tumors, correct classification of tumor type is of greatest importance.
  • METHODS: DNA was isolated from tumor areas in paraffin sections and amplified by a modified protocol for DOP-PCR.
  • After labeling of tumor-DNA and normal DNA with biotin-dUTP and digoxigenin-dUTP, respectively, comparative genomic hybridization (CGH) was carried out according to standard protocols.
  • Retrospectively, 26 (16 adenomas and 10 carcinomas) tumors of the adrenal cortex were analyzed.
  • RESULTS: Genetic alterations were found in 5/16 adenomas (31.25%) and in all adrenocortical carcinomas.
  • The mean number of genetic changes per tumor was 8.7 (range 6-12) in carcinomas.
  • The benign cortical tumors present 1.6 changes (range 0-3) per tumor.
  • Only a moderate correlation between number of alterations and size of tumor was seen.
  • CONCLUSIONS: Genetic evaluation facilitates differentiation between adrenal gland tumors.
  • Genetic tests should be used in routine diagnostics of adrenal specimens.
  • Potentially, fine-needle biopsy can be established as standard diagnostics of adrenal tumors with unknown genesis.
  • [MeSH-major] Adenoma / classification. Adenoma / genetics. Adrenal Cortex Neoplasms / classification. Adrenal Cortex Neoplasms / genetics. Carcinoma / genetics
  • [MeSH-minor] Adrenalectomy / methods. Adult. Aged. Biomarkers, Tumor / analysis. Biopsy, Fine-Needle. Chromosome Aberrations. Chromosome Mapping. Cohort Studies. DNA, Neoplasm / analysis. Female. Humans. Immunohistochemistry. Male. Middle Aged. Polymerase Chain Reaction. Retrospective Studies. Sensitivity and Specificity. Statistics, Nonparametric. Young Adult

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  • (PMID = 20364258.001).
  • [ISSN] 1433-8726
  • [Journal-full-title] World journal of urology
  • [ISO-abbreviation] World J Urol
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / DNA, Neoplasm
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32. Cardoso CC, Bornstein SR, Hornsby PJ: Optimizing orthotopic cell transplantation in the mouse adrenal gland. Cell Transplant; 2010;19(5):565-72
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Optimizing orthotopic cell transplantation in the mouse adrenal gland.
  • Orthotopic cell transplantation models are important for a complete understanding of cell-cell interactions as well as tumor biology.
  • In published studies of orthotopic transplantation in the mouse adrenal gland, human neuroblastoma cells have been shown to invade and occupy the adrenal, but in these investigations a true orthotopic model was not established.
  • Here we show an orthotopic model in which transplanted cells are retained within the adrenal gland by formation of a fibrin clot.
  • To establish an appropriate technique, we used brightly fluorescent 10 microm polystyrene microspheres injected into the mouse adrenal gland.
  • When the microspheres were injected in a fibrinogen/thrombin mixture, fluorescence was confined to the adrenal gland.
  • As a model neoplastic cell originating from the cortex of the gland, we used a tumorigenic bovine adrenocortical cell line.
  • When 3 x 10(5) cells were implanted orthotopically, by 16 days the cell mass had expanded and had invaded the cortex, whereas when 1 x 10(5) cells were used, tumor masses were much smaller.
  • When mice were sacrificed at different time points, we found that tumor growth resulting was progressive and that by 26 days cells there was extensive invasion into the cortex or almost complete replacement of the cortex with tumor cells.
  • In summary, the present orthotopic model for intra-adrenal cell transplantation is valuable for investigation of growth of neoplastic cells of both cortical and medullary origin and should be useful for future studies of cortex-medulla interactions.

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  • (PMID = 20525431.001).
  • [ISSN] 1555-3892
  • [Journal-full-title] Cell transplantation
  • [ISO-abbreviation] Cell Transplant
  • [Language] ENG
  • [Grant] United States / NIA NIH HHS / AG / AG012287-14; United States / NIA NIH HHS / AG / P01 AG020752-020006; United States / NIA NIH HHS / AG / AG020752-020006; United States / NIA NIH HHS / AG / P01 AG020752; United States / NIA NIH HHS / AG / R37 AG012287-14; United States / NIA NIH HHS / AG / R37 AG012287
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 9001-31-4 / Fibrin; 9001-32-5 / Fibrinogen; EC 3.4.21.5 / Thrombin
  • [Other-IDs] NLM/ NIHMS246503; NLM/ PMC3735364
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33. Coli A, Di Giorgio A, Castri F, Destito C, Marin AW, Bigotti G: Sarcomatoid carcinoma of the adrenal gland: A case report and review of literature. Pathol Res Pract; 2010 Jan 15;206(1):59-65
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Sarcomatoid carcinoma of the adrenal gland: A case report and review of literature.
  • Reports about adrenocortical carcinomas (AC) mixed with sarcomatous areas are very rare.
  • The terminology and pathogenesis of such biphasic tumors remain controversial.
  • Herein, we report a case of sarcomatoid carcinoma of the adrenal gland in a 75-year-old woman who presented with left abdominal pain of one month's standing.
  • The results of abdominal ultrasonography and computed tomography (CT) showed the presence of a large heterogeneous adrenal mass.
  • Histologically, the neoplasm showed areas of adrenocortical carcinoma and areas of sarcomatoid spindle cell proliferation.
  • Twelve months after removal of the primary tumor, the patient died of her disease.
  • To the best of our knowledge, only seven cases of adrenal sarcomatoid carcinoma have been reported in the medical literature.
  • We review the reported cases according to the 2004 classification of the World Health Organization (WHO) of lung tumors, and highlight the histogenesis, diagnosis, and clinical course of this very aggressive tumor.
  • [MeSH-major] Adrenal Cortex Neoplasms / pathology. Carcinosarcoma / secondary. Liver Neoplasms / secondary
  • [MeSH-minor] Adrenalectomy. Aged. Fatal Outcome. Female. Humans. Neoplasm Proteins. Splenectomy

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  • [Copyright] Copyright 2009 Elsevier GmbH. All rights reserved.
  • (PMID = 19369012.001).
  • [ISSN] 1618-0631
  • [Journal-full-title] Pathology, research and practice
  • [ISO-abbreviation] Pathol. Res. Pract.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Neoplasm Proteins
  • [Number-of-references] 16
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34. McNicol AM: Assessment of malignancy in adrenal cortical tumors. Endocr Pathol; 2006;17(2):131-6

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Assessment of malignancy in adrenal cortical tumors.
  • The roles of the pathologist in assessing adrenal cortical tumors are, first, to differentiate adenoma from carcinoma, and, second, to assess prognosis when the diagnosis of malignancy is made.
  • [MeSH-major] Adenoma / pathology. Adrenal Cortex Neoplasms / pathology. Carcinoma / pathology. Neoplasm Invasiveness / diagnosis
  • [MeSH-minor] Biomarkers, Tumor / analysis. Humans. Immunohistochemistry. Molecular Biology. Prognosis

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  • (PMID = 17159245.001).
  • [ISSN] 1046-3976
  • [Journal-full-title] Endocrine pathology
  • [ISO-abbreviation] Endocr. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
  • [Number-of-references] 36
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35. Abma EM, Kluin PM, Dullaart RP: Malignant aldosterone-producing adrenal tumour: reoccurrence with glucocorticoid excess without hyperaldosteronism. Neth J Med; 2008 Jun;66(6):252-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Malignant aldosterone-producing adrenal tumour: reoccurrence with glucocorticoid excess without hyperaldosteronism.
  • We describe a case of hypokalaemic hypertension due to hyperaldosteronism caused by a unilateral adrenocortical tumour with unfavourable histopathology suggestive of malignancy.
  • The patient's clinical course was uneventful, until she presented with extensive metastases of adrenal carcinoma four years later.
  • Although malignant aldosterone-producing adrenal tumours are very rare, the present case underscores that clinicians should be aware that primary hyperaldosteronism can occur in the context of adrenocortical carcinoma.
  • [MeSH-major] Adrenal Cortex Neoplasms / complications. Adrenocortical Carcinoma / complications. Hydrocortisone / blood. Hyperaldosteronism / complications. Hyperkalemia / etiology. Hypertension / etiology. Neoplasm Recurrence, Local / blood
  • [MeSH-minor] Blood Pressure. Diagnosis, Differential. Female. Humans. Magnetic Resonance Imaging. Middle Aged. Potassium / blood. Tomography, X-Ray Computed

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  • (PMID = 18689909.001).
  • [ISSN] 0300-2977
  • [Journal-full-title] The Netherlands journal of medicine
  • [ISO-abbreviation] Neth J Med
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Netherlands
  • [Chemical-registry-number] RWP5GA015D / Potassium; WI4X0X7BPJ / Hydrocortisone
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36. Tan HS, Thai AC, Nga ME, Mukherjee JJ: Development of ipsilateral adrenocortical carcinoma sixteen years after resection of an adrenal tumour causing Cushing's syndrome. Ann Acad Med Singapore; 2005 Apr;34(3):271-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Development of ipsilateral adrenocortical carcinoma sixteen years after resection of an adrenal tumour causing Cushing's syndrome.
  • INTRODUCTION: At times, it may be difficult to differentiate early stage, low-grade adrenocortical carcinoma from benign adrenal adenoma.
  • CLINICAL PICTURE: A 53-year-old lady underwent right adrenalectomy for a 4-cm adrenocortical tumour causing Cushing's syndrome.
  • Histology revealed an adrenocortical adenoma.
  • Sixteen years later, she presented with a 14-cm adrenal tumour, again on the right side.
  • TREATMENT: She underwent surgical removal of the tumour.
  • Histology confirmed adrenocortical carcinoma.
  • OUTCOME: She died of metastatic disease 17 months later.
  • CONCLUSIONS: This case highlights the importance of long-term, systematic follow-up of patients treated for benign adrenal adenomas, especially if the tumour size exceeds 4 cm.
  • [MeSH-major] Adrenal Cortex Neoplasms / pathology. Adrenocortical Carcinoma / pathology. Cushing Syndrome / etiology. Neoplasm Recurrence, Local / pathology
  • [MeSH-minor] Adrenocortical Adenoma / pathology. Diagnosis, Differential. Fatal Outcome. Female. Humans. Middle Aged

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  • (PMID = 15902349.001).
  • [ISSN] 0304-4602
  • [Journal-full-title] Annals of the Academy of Medicine, Singapore
  • [ISO-abbreviation] Ann. Acad. Med. Singap.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Singapore
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37. Giordano TJ: Molecular pathology of adrenal cortical tumors: separating adenomas from carcinomas. Endocr Pathol; 2006;17(4):355-63
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Molecular pathology of adrenal cortical tumors: separating adenomas from carcinomas.
  • Adrenal cortical carcinoma is a rare but interesting endocrine tumor.
  • Its diagnosis is usually straightforward using morphologic assessment and supplemental immunohistochemistry.
  • Here, the relevant molecular pathology of adrenal cortical tumors is reviewed with special reference to those methods (e.g., DNA microarrays) that hold promise for improved diagnosis and prognosis, and prediction of therapeutic response.
  • [MeSH-major] Adrenal Cortex Neoplasms / pathology. Adrenocortical Adenoma / pathology. Adrenocortical Carcinoma / pathology
  • [MeSH-minor] Biomarkers, Tumor / genetics. Biomarkers, Tumor / metabolism. DNA, Neoplasm / analysis. Diagnosis, Differential. Humans. Immunohistochemistry. Oligonucleotide Array Sequence Analysis. Principal Component Analysis

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  • (PMID = 17525484.001).
  • [ISSN] 1046-3976
  • [Journal-full-title] Endocrine pathology
  • [ISO-abbreviation] Endocr. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / DNA, Neoplasm
  • [Number-of-references] 59
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38. Bertagna X, Groussin L, Libe R, Bertherat J: [Adrenal cortical carcinoma: advances in the pathophysiology and management of this malignancy]. Bull Acad Natl Med; 2008 Jan;192(1):87-102; discussion 102-3

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Adrenal cortical carcinoma: advances in the pathophysiology and management of this malignancy].
  • [Transliterated title] Le corticosurrénalome: progrès dans la physiopathologie et la prise en charge d'un cancer rare.
  • Adrenal cortical carcinoma is a rare malignancy, with only one or two new cases being diagnosed per million subjects per year.
  • The best chances of cure are obtained when a "localized" tumor can undergo "complete" surgical removal.
  • Most often, however, the diagnosis is made when the tumor is already invasive and non secretory Clinical, hormonal and imaging features, including 18-fluorodeoxyglucose PET scan, can provide strong evidence of malignancy and indicate open surgical excision in expert hands.
  • Recent advances in the genetics of adrenal cortical carcinomas have identified molecular factors that can be used as diagnostic and prognostic markers.
  • A better understanding of the pathophysiology of these tumors is required in order to develop targeted therapies.
  • [MeSH-major] Adrenal Cortex Neoplasms / therapy. Carcinoma / therapy
  • [MeSH-minor] Humans. Neoplasm Staging. Prognosis

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  • (PMID = 18663984.001).
  • [ISSN] 0001-4079
  • [Journal-full-title] Bulletin de l'Académie nationale de médecine
  • [ISO-abbreviation] Bull. Acad. Natl. Med.
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Netherlands
  • [Number-of-references] 30
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39. Dehner LP, Hill DA: Adrenal cortical neoplasms in children: why so many carcinomas and yet so many survivors? Pediatr Dev Pathol; 2009 Jul-Aug;12(4):284-91

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Adrenal cortical neoplasms in children: why so many carcinomas and yet so many survivors?
  • Adrenal cortical neoplasms in children are represented by a disproportionate number of cases that have been diagnosed pathologically as adrenocortical carcinomas (ACCs)-as many as 90% of all cortical tumors in some pediatric series.
  • Most are sporadically occurring neoplasms, but ACCs are a manifestation of Beckwith-Wiedemann and Li-Fraumeni syndromes.
  • Tumor weight is seemingly a significant determinant in prognosis at a threshold of greater than 400 g.
  • A risk assessment system is proposed that incorporates tumor weight, localization of tumor to the gland without invasion into the surrounding tissues or organs, and absence of metastasis.
  • [MeSH-major] Adrenal Cortex Neoplasms / pathology. Adrenocortical Carcinoma / pathology
  • [MeSH-minor] Beckwith-Wiedemann Syndrome / genetics. Beckwith-Wiedemann Syndrome / pathology. Child, Preschool. Humans. Infant. Infant, Newborn. Li-Fraumeni Syndrome / genetics. Li-Fraumeni Syndrome / pathology. Neoplasm Staging. Prognosis. Risk Assessment. Survivors

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  • (PMID = 19326954.001).
  • [ISSN] 1093-5266
  • [Journal-full-title] Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society
  • [ISO-abbreviation] Pediatr. Dev. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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40. Gonzalez RJ, Tamm EP, Ng C, Phan AT, Vassilopoulou-Sellin R, Perrier ND, Evans DB, Lee JE: Response to mitotane predicts outcome in patients with recurrent adrenal cortical carcinoma. Surgery; 2007 Dec;142(6):867-75; discussion 867-75
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Response to mitotane predicts outcome in patients with recurrent adrenal cortical carcinoma.
  • BACKGROUND: Adrenal cortical carcinoma (ACC) is a rare disease in which recurrence after surgery is common.
  • RESULTS: The median disease-free survival for all 186 patients was 12 months and the median overall survival (OS) was 37 months.
  • Higher stage at presentation (P = .002) and tumor cortisol production (P = .007) were associated with a worse OS.
  • For patients with stable or responding disease to mitotane, the median OS from date of recurrence was 18 months, compared with 9 months (P = .01) for those who progressed.
  • CONCLUSIONS: Patients with recurrent ACC who have stable or responding disease to mitotane have a more favorable prognosis than those who progress.
  • Mitotane should be considered in most patients with recurrent ACC, including as preoperative therapy for those with recurrent disease considered for surgical resection.
  • [MeSH-major] Adrenal Cortex Neoplasms / drug therapy. Adrenal Cortex Neoplasms / surgery. Antineoplastic Agents, Hormonal / therapeutic use. Mitotane / therapeutic use. Neoplasm Recurrence, Local / drug therapy. Neoplasm Recurrence, Local / surgery
  • [MeSH-minor] Adolescent. Adult. Aged. Child. Child, Preschool. Combined Modality Therapy. Disease-Free Survival. Female. Follow-Up Studies. Humans. Kaplan-Meier Estimate. Male. Middle Aged. Predictive Value of Tests. Prognosis

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  • (PMID = 18063070.001).
  • [ISSN] 1532-7361
  • [Journal-full-title] Surgery
  • [ISO-abbreviation] Surgery
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Hormonal; 78E4J5IB5J / Mitotane
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41. Namour F, Ayav A, Lu X, Klein M, Muresan M, Bresler L, Tramoy D, Guéant JL, Brunaud L: Lack of association between microsatellite instability and benign adrenal tumors. World J Surg; 2006 Jul;30(7):1240-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Lack of association between microsatellite instability and benign adrenal tumors.
  • BACKGROUND: The adrenal gland may give rise to pheochromocytomas, which are catecholamine-producing tumors originating from the adrenal medulla, or to adrenocortical tumors, which derive from the adrenocortical cortex and may be secreting or not.
  • The genetic mechanisms underlying the formation of these tumors include somatic mutations in susceptibility genes, especially in the familial forms, and allelic loss, especially in chromosome 1.
  • Microsatellite loci were analyzed in 32 benign tumors, including 11 pheochromocytomas and 21 adrenocortical tumors, in patients with and without familial syndrome.
  • No microsatellite instability was detected in any tumor.
  • A second patient with a MEN-2A syndrome and a two-sided pheochromocytoma exhibited a loss of heterozygosity for D2S123 in the right tumor only and a retention of heterozygosity for all markers in the left tumor.
  • CONCLUSIONS: These results suggest that microsatellite instability, evaluated by the five reference markers of the National Cancer Institute, is not a feature of benign adrenal tumors.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Microsatellite Repeats / genetics. Pheochromocytoma / genetics
  • [MeSH-minor] Adult. Aged. Alleles. DNA, Neoplasm / analysis. Female. Humans. Loss of Heterozygosity. Male. Middle Aged. Polymerase Chain Reaction. Proto-Oncogene Proteins / genetics

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  • (PMID = 16715450.001).
  • [ISSN] 0364-2313
  • [Journal-full-title] World journal of surgery
  • [ISO-abbreviation] World J Surg
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / DNA, Neoplasm; 0 / Proto-Oncogene Proteins
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42. Hermsen IG, Gelderblom H, Kievit J, Romijn JA, Haak HR: Extremely long survival in six patients despite recurrent and metastatic adrenal carcinoma. Eur J Endocrinol; 2008 Jun;158(6):911-9

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Extremely long survival in six patients despite recurrent and metastatic adrenal carcinoma.
  • OBJECTIVE: Adrenal cortical carcinoma (ACC) is an aggressive tumour with a high mortality.
  • The patient is still alive 28 years after diagnosis.
  • The patient is still alive 25 years after diagnosis.
  • The tumour was resected followed by o,p'-DDD treatment.
  • She is still alive 18 years after diagnosis.
  • He is still alive 17 years after the initial diagnosis.
  • After diagnosis, she developed lung metastasis, which were treated with o,p'-DDD and chemotherapy.
  • The patient is still alive with slowly progressive disease 12 years after diagnosis.
  • The patient is still alive 28 years after diagnosis.
  • CONCLUSION: Some patients can have an extremely long survival of ACC, despite recurrent disease and metastases.

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  • (PMID = 18505909.001).
  • [ISSN] 1479-683X
  • [Journal-full-title] European journal of endocrinology
  • [ISO-abbreviation] Eur. J. Endocrinol.
  • [Language] ENG
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
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43. Ilias I, Sahdev A, Reznek RH, Grossman AB, Pacak K: The optimal imaging of adrenal tumours: a comparison of different methods. Endocr Relat Cancer; 2007 Sep;14(3):587-99
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] The optimal imaging of adrenal tumours: a comparison of different methods.
  • Computed tomography (CT; unenhanced, followed by contrast-enhanced examinations) is the cornerstone of imaging of adrenal tumours.
  • Functional nuclear medicine imaging is useful for adrenal lesions that are not adequately characterised with CT and MRI.
  • Scintigraphy with [(131)I]-6-iodomethyl norcholesterol (a labelled cholesterol analogue) can differentiate adrenal cortical adenomas from carcinomas.
  • The specific and useful roles of adrenal imaging include the characterisation of tumours, assessment of true tumour size, differentiation of adenomas from carcinomas and metastases, and differentiation of hyperfunctioning from non-functioning lesions.
  • Adrenal imaging complements and assists the clinical and hormonal evaluation of adrenal tumours.
  • [MeSH-major] Adenoma / diagnosis. Adrenal Gland Neoplasms / diagnosis. Diagnostic Imaging / methods
  • [MeSH-minor] Adrenal Cortex Neoplasms / diagnosis. Adrenal Cortex Neoplasms / pathology. Adrenal Medulla / pathology. Adrenocortical Hyperfunction / diagnosis. Diagnosis, Differential. Ganglioneuroma / diagnosis. Ganglioneuroma / pathology. Hemangioma / diagnosis. Hemangioma / pathology. Hemangiosarcoma / diagnosis. Hemangiosarcoma / pathology. Humans. Leiomyosarcoma / diagnosis. Leiomyosarcoma / pathology. Lymphoma / diagnosis. Lymphoma / pathology. Magnetic Resonance Imaging. Myelolipoma / diagnosis. Myelolipoma / pathology. Neoplasm Metastasis. Neuroblastoma / diagnosis. Neuroblastoma / pathology. Pheochromocytoma / diagnosis. Pheochromocytoma / pathology. Tomography, X-Ray Computed. Whole Body Imaging

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  • (PMID = 17914090.001).
  • [ISSN] 1351-0088
  • [Journal-full-title] Endocrine-related cancer
  • [ISO-abbreviation] Endocr. Relat. Cancer
  • [Language] eng
  • [Publication-type] Comparative Study; Evaluation Studies; Journal Article; Review
  • [Publication-country] England
  • [Number-of-references] 61
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44. Cohen MM Jr: Beckwith-Wiedemann syndrome: historical, clinicopathological, and etiopathogenetic perspectives. Pediatr Dev Pathol; 2005 May-Jun;8(3):287-304
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Complications include neonatal hypoglycemia and an increased risk for Wilms tumor, adrenal cortical carcinoma, hepatoblastoma, rhabdomyosarcoma, and neuroblastoma, among others.
  • [MeSH-minor] Diagnosis, Differential. Female. Humans. Infant, Newborn. Male

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  • (PMID = 16010495.001).
  • [ISSN] 1093-5266
  • [Journal-full-title] Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society
  • [ISO-abbreviation] Pediatr. Dev. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 140
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45. Tian L, Guo Y, Wu YP, Liu LZ: [CT features of adrenal cortical adenoma: a report of 109 cases]. Ai Zheng; 2008 Jan;27(1):66-70

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [CT features of adrenal cortical adenoma: a report of 109 cases].
  • BACKGROUND & OBJECTIVE: Adrenal cortical adenoma (ACA) is a common disease, and can be diagnosed easily with CT examination.
  • The maximal diameter of the tumor was <5 cm in 95 patients.
  • The maximal diameter of the tumor was > 5 cm in 14 patients.
  • [MeSH-major] Adrenal Cortex Neoplasms / radiography. Adrenocortical Adenoma / radiography. Radiographic Image Enhancement / methods. Tomography, Spiral Computed / methods
  • [MeSH-minor] Adolescent. Adult. Aged. Child. Child, Preschool. Diagnosis, Differential. Female. Humans. Infant. Male. Middle Aged. Neoplasm Recurrence, Local. Tumor Burden. Young Adult

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  • (PMID = 18184467.001).
  • [Journal-full-title] Ai zheng = Aizheng = Chinese journal of cancer
  • [ISO-abbreviation] Ai Zheng
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
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46. Dell'Avanzato R, Castaldi F, Giovannini C, Mercadante E, Cianciulli P, Carlini M: Giant symptomatic myelolipoma of the right adrenal gland: a case report. Chir Ital; 2009 Mar-Apr;61(2):231-6

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Giant symptomatic myelolipoma of the right adrenal gland: a case report.
  • Adrenal myelolipoma is an uncommon tumour of the adrenal gland, usually unilateral, frequently associated with hypertension and obesity, with a benign biological behaviour and without hormonal activity, first described in 1905.
  • The neoplasm consists of adipose tissue and myelopoietic cells of the bone marrow.
  • These tumours have a very slow but continued growth and their volume and weight vary significantly from small lesions of a few grams to huge masses weighing up to several kilograms.
  • In this report a case of a 43-year-old male with a 22 x 18 x 9 cm giant myelolipoma, weighing 3500 g and originating from the right adrenal gland is described.
  • The different aetiological hypotheses of this rare neoplasm and its clinical features, diagnosis and treatment are discussed.
  • [MeSH-major] Adrenal Cortex Neoplasms / pathology. Myelolipoma / pathology
  • [MeSH-minor] Adult. Diagnosis, Differential. Humans. Male. Treatment Outcome

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  • (PMID = 19536999.001).
  • [ISSN] 0009-4773
  • [Journal-full-title] Chirurgia italiana
  • [ISO-abbreviation] Chir Ital
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Italy
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47. Kirshtein B, Yelle JD, Moloo H, Poulin E: Laparoscopic adrenalectomy for adrenal malignancy: a preliminary report comparing the short-term outcomes with open adrenalectomy. J Laparoendosc Adv Surg Tech A; 2008 Feb;18(1):42-6

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Laparoscopic adrenalectomy for adrenal malignancy: a preliminary report comparing the short-term outcomes with open adrenalectomy.
  • BACKGROUND: The laparoscopic approach to adrenal malignancy remains a topic of debate.
  • RESULTS: Twenty-six cases were identified: 19 women and 7 men with a median age of 48 years (range, 20-81) underwent 12 open (8 adrenocortical carcinoma [ACC] and 4 metastases) and 14 laparoscopic adrenalectomies (5 ACC, 8 metastases, and 1 lymphoma).
  • There was no difference in age, sex, American Society of Anesthesiologists status or diagnosis between the two groups, but patients in the laparoscopic group had a higher body mass index.
  • The size of tumors removed by open surgery was larger than by laparoscopy (8 vs. 4 cm; P = 0.003).
  • CONCLUSIONS: Laparoscopic adrenalectomy is both feasible and safe for some malignant tumors of the adrenal gland in experienced hands.
  • Careful selection, preoperative staging, and respect for oncologic principles are important considerations in choosing laparoscopic surgery for primary and secondary adrenal malignancy.
  • [MeSH-major] Adrenal Cortex Neoplasms / surgery. Adrenalectomy / methods. Carcinoma / surgery. Laparoscopy
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Blood Loss, Surgical. Female. Humans. Length of Stay. Lymphoma / surgery. Male. Middle Aged. Neoplasm Metastasis. Retrospective Studies. Treatment Outcome

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  • (PMID = 18266573.001).
  • [ISSN] 1092-6429
  • [Journal-full-title] Journal of laparoendoscopic & advanced surgical techniques. Part A
  • [ISO-abbreviation] J Laparoendosc Adv Surg Tech A
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] United States
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48. Sturgeon C, Shen WT, Clark OH, Duh QY, Kebebew E: Risk assessment in 457 adrenal cortical carcinomas: how much does tumor size predict the likelihood of malignancy? J Am Coll Surg; 2006 Mar;202(3):423-30
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Risk assessment in 457 adrenal cortical carcinomas: how much does tumor size predict the likelihood of malignancy?
  • BACKGROUND: Laparoscopic adrenalectomy for tumors > 6 cm is controversial because of the risk of malignancy, but data to support this position are mostly from small series.
  • STUDY DESIGN: Adrenocortical carcinomas (ACC) recorded in the Surveillance, Epidemiology, and End Results (SEER) database (1988 to 2000) were compared with benign functional or nonfunctional adrenal cortical adenomas (excluding aldosteronomas) operated on at our institution between January 1, 1993, and July 1, 2003.
  • RESULTS: We identified 457 patients with ACC and 47 patients with adrenal cortical adenomas; 376 and 44 neoplasms, respectively, had tumor size data available.
  • Tumor size was larger in ACC (12.0 +/- 5.6 versus 4.2 +/- 1.9 cm, mean +/- SD, p < 0.05).
  • For ACC presenting with local disease, the sensitivity, specificity, and likelihood ratios of tumor size to predict malignancy were 96%, 52%, and 2.0, respectively, for tumors > or = 4 cm; 90%, 80%, and 4.4 for tumors > or = 6 cm; 77%, 95%, and 16.9 for tumors > or = 8 cm; and 55%, 98%, and 24.4 for tumors > or = 10 cm.
  • Assuming a pretest probability of malignancy of 5%, the likelihood ratios derived from this study yield a posttest probability of 10%, 19%, and 47% for cancer in adrenal cortical tumors > or = 4 cm, > or = 6 cm, and > or = 8 cm, respectively.
  • CONCLUSIONS: These data suggest that size is useful for predicting malignancy, and that at a size threshold of > or = 4 cm, the likelihood of malignancy doubles (to 10%) and it is more than ninefold higher for tumors > or = 8 cm (47%).
  • [MeSH-major] Adrenal Cortex Neoplasms / pathology. Adrenocortical Carcinoma / pathology
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Child. Child, Preschool. Diagnosis, Differential. Female. Humans. Infant. Infant, Newborn. Male. Middle Aged. Neoplasm Staging. Prevalence. Probability. Prognosis. Retrospective Studies. Risk Assessment. SEER Program / statistics & numerical data. Severity of Illness Index

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  • (PMID = 16500246.001).
  • [ISSN] 1072-7515
  • [Journal-full-title] Journal of the American College of Surgeons
  • [ISO-abbreviation] J. Am. Coll. Surg.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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49. Li SH, Huang CH, Ko SF, Chou FF, Huang SC: Extended survival in a patient with recurrent and metastatic adrenal cortical carcinoma by aggressive transarterial embolization--a case report. J Surg Oncol; 2005 May 1;90(2):101-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Extended survival in a patient with recurrent and metastatic adrenal cortical carcinoma by aggressive transarterial embolization--a case report.
  • The prognosis of inoperable recurrent or metastatic adrenal cortical carcinoma is poor due to lack of effective treatment modalities.
  • We report a case of recurrent and metastatic adrenal cortical carcinoma in which prolonged survival of 58 months was achieved with aggressive three sequential transarterial embolization.
  • A 60-year-old man received operation for left adrenal cortical carcinoma.
  • Liver metastases, tumor bed recurrence, and spleen metastasis were noted during follow-up.
  • Three sequential transarterial embolization for metastatic liver tumors, tumor bed recurrence, and metastatic spleen tumor were performed and resulted in relief of symptoms and prolonged survival of 58 months after recurrence verified.
  • Aggressive transarterial embolization seems to be a safe and effective procedure for symptoms relief, and may prolong survival in the management of inoperable adrenal cortical carcinoma.
  • It can be considered in any patient with inoperable adrenal cortical carcinoma if not contraindicated.
  • [MeSH-major] Adrenal Cortex Neoplasms / therapy. Adrenocortical Carcinoma / therapy. Embolization, Therapeutic. Liver Neoplasms / secondary. Neoplasm Recurrence, Local / therapy. Splenic Neoplasms / secondary

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  • (PMID = 15844181.001).
  • [ISSN] 0022-4790
  • [Journal-full-title] Journal of surgical oncology
  • [ISO-abbreviation] J Surg Oncol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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50. Karikari IO, Uschold TD, Selznick LA, Carter JH, Cummings TJ, Friedman AH: Primary spinal intramedullary adrenal cortical adenoma associated with spinal dysraphism: case report. Neurosurgery; 2006 Nov;59(5):E1144; discussion E1144
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Primary spinal intramedullary adrenal cortical adenoma associated with spinal dysraphism: case report.
  • OBJECTIVE: The authors report a primary spinal intramedullary adrenal cortical adenoma in a patient with spinal dysraphism presenting with bilateral leg pain and urinary frequency.
  • METHODS: Magnetic resonance imaging, L2 laminectomy with resection of mass, and pathological and immunohistochemical analysis of resected mass revealed the diagnosis.
  • RESULTS: Microscopic and immunohistochemical findings confirmed the diagnosis as a primary intramedullary tumor of adrenal cortical origin.
  • CONCLUSION: The occurrence of a primary adrenal tumor in the spinal cord is rare and difficult to explain based on our understanding of embryology.
  • [MeSH-major] Adrenal Cortex Neoplasms / diagnosis. Adrenal Cortex Neoplasms / surgery. Adrenocortical Adenoma / diagnosis. Adrenocortical Adenoma / surgery. Spinal Cord Neoplasms / diagnosis. Spinal Cord Neoplasms / surgery. Spinal Dysraphism / complications

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  • (PMID = 17143207.001).
  • [ISSN] 1524-4040
  • [Journal-full-title] Neurosurgery
  • [ISO-abbreviation] Neurosurgery
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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51. Korzeniewska M, Kołomecki K, Stepień H, Naze M, Stepień T, Kuzdak K: [Assessment of pro- and antiangiogenic factors blood serum concentrations in patients with hormonal inactive adrenal tumors]. Endokrynol Pol; 2005 Jan-Feb;56(1):39-44

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Assessment of pro- and antiangiogenic factors blood serum concentrations in patients with hormonal inactive adrenal tumors].
  • INTRODUCTION: The growth and persistence of solid tumors and their metastases is connected with angiogenesis.
  • THE AIM OF THE STUDY: Evaluation the value of serum VEGF and soluble forms of VEGF receptors concentration as a marker of malignancy in patients with hormonal inactive adrenal tumors.
  • MATERIAL AND METHODS: Twenty seven patients (18 female, 9 male; mean age 48+/-4.3 years) with adrenocortical carcinoma (N=8), adrenal metastases (N=4) and adrenocortical adenoma (N=15) were included in this study.
  • Patients with adrenocortical carcinoma had the levels of VEGF (1263.8 pg/ml) significantly higher and of sVEGFR-2 (5893.7 pg/ml) significantly lower in comparison to control group (p<0.05).
  • On the other hand the mean VEGF (334.2 pg/ml) concentration in patients with benign adrenocortical adenoma wasn't significant different than in control group (p>0.05) but mean sVEGFR-1 (21.7 pg/ml) and sVEGFR-2 (7106.4 pg/ml) concentrations were significantly lower than in the control (p<0.05).
  • CONCLUSION: These data suggest that determination of VEGF and sVEGFR concentration in the serum of patients with hormonal inactive adrenal tumors may be applied as an additional marker of malignancy.
  • [MeSH-major] Adrenal Cortex Neoplasms / blood. Adrenal Cortex Neoplasms / pathology. Biomarkers, Tumor / blood. Vascular Endothelial Growth Factor A / blood
  • [MeSH-minor] Adrenocortical Adenoma / blood. Adrenocortical Adenoma / pathology. Adrenocortical Carcinoma / blood. Adrenocortical Carcinoma / pathology. Adrenocortical Carcinoma / secondary. Adult. Aged. Female. Humans. Male. Middle Aged. Neoplasm Staging. Vascular Endothelial Growth Factor Receptor-1 / blood. Vascular Endothelial Growth Factor Receptor-2 / blood

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  • (PMID = 16335673.001).
  • [ISSN] 0423-104X
  • [Journal-full-title] Endokrynologia Polska
  • [ISO-abbreviation] Endokrynol Pol
  • [Language] pol
  • [Publication-type] Controlled Clinical Trial; English Abstract; Journal Article
  • [Publication-country] Poland
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Vascular Endothelial Growth Factor A; EC 2.7.10.1 / Vascular Endothelial Growth Factor Receptor-1; EC 2.7.10.1 / Vascular Endothelial Growth Factor Receptor-2
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52. Tissier F: Classification of adrenal cortical tumors: what limits for the pathological approach? Best Pract Res Clin Endocrinol Metab; 2010 Dec;24(6):877-85

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Classification of adrenal cortical tumors: what limits for the pathological approach?
  • Most adrenocortical tumors are benign; adrenocortical carcinomas are rare but their prognosis is poor and their therapeutics are sparse.
  • In most adrenocortical tumors, the morphological approach in particular by Weiss system, brings sufficient elements to establish the differential diagnosis between a benign and a malignant tumor.
  • But some tumors of Weiss score of 2 or 3 can raise problems: are they benign, malignant or are they of uncertain malignant potential?
  • These genetics findings already have repercussions for the patients in the development of molecular markers for diagnosis and prognosis and in the future they could help in the development of new morphological approaches, in particular immunohistochemical approaches.
  • [MeSH-major] Adrenal Cortex Neoplasms / classification. Adrenal Cortex Neoplasms / pathology
  • [MeSH-minor] Adrenal Cortex / metabolism. Adrenal Cortex / pathology. Animals. Biomarkers, Tumor / metabolism. Diagnosis, Differential. Humans. Neoplasm Staging. Prognosis

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  • [Copyright] 2010 Elsevier Ltd. All rights reserved.
  • (PMID = 21115156.001).
  • [ISSN] 1878-1594
  • [Journal-full-title] Best practice & research. Clinical endocrinology & metabolism
  • [ISO-abbreviation] Best Pract. Res. Clin. Endocrinol. Metab.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
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53. Wagner AS, Fleitz JM, Kleinschmidt-Demasters BK: Pediatric adrenal cortical carcinoma: brain metastases and relationship to NF-1, case reports and review of the literature. J Neurooncol; 2005 Nov;75(2):127-33
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Pediatric adrenal cortical carcinoma: brain metastases and relationship to NF-1, case reports and review of the literature.
  • Adrenal cortical carcinoma (ACC) is a rare childhood neoplasm that seldom manifests brain metastases; hence few papers in the literature focus on neurological manifestations associated with ACC.
  • Although ACC is known to be a signature tumor type in several inherited cancer predisposition syndromes, particularly Li Fraumeni, ACC has not been previously associated with neurofibromatosis, type 1 (NF-1), an inherited disorder with frequent CNS lesions that might prompt concern for metastatic disease by neuroimaging studies.
  • The first child developed metastasis to the brain 4 years after resection of his adrenal primary and 2 and 1 years, respectively, after metastases to the liver and lungs.
  • Disseminated disease prompted concern that her complex intracranial lesions identified by neuroimaging studies might represent brain metastases, but this proved to be NF1-related hamartomatous lesions.
  • [MeSH-major] Adrenal Cortex Neoplasms / diagnosis. Adrenal Cortex Neoplasms / pathology. Adrenocortical Carcinoma / diagnosis. Adrenocortical Carcinoma / pathology. Brain Neoplasms / secondary. Neurofibromatosis 1
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Child, Preschool. Chromosome Aberrations. Chylothorax / complications. Chylothorax / surgery. Diagnosis, Differential. Fatal Outcome. Female. Follow-Up Studies. Hamartoma / diagnosis. Humans. Liver Neoplasms / secondary. Lung Neoplasms / secondary. Magnetic Resonance Imaging. Male. Neoplasm Metastasis. Receptor, Epidermal Growth Factor / metabolism. Time Factors. Treatment Outcome

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  • (PMID = 16132517.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Netherlands
  • [Chemical-registry-number] EC 2.7.10.1 / Receptor, Epidermal Growth Factor
  • [Number-of-references] 28
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54. Puech-Bret N, Bret J, Bennet A, Huyghe E, Mazerolles C, Zabraniecki L, Fournie B: Maffucci syndrome and adrenal cortex tumor. Joint Bone Spine; 2009 Oct;76(5):556-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Maffucci syndrome and adrenal cortex tumor.
  • We report the second known case of Maffucci syndrome associated with an adrenal cortex tumor.
  • Endocrine tumors have been reported in patients with multiple enchondromas, although the underlying mechanism of this combination is unknown.
  • Therefore, routine evaluation for involvement of the adrenal cortex may be warranted to improve our knowledge of this syndrome and of its pathophysiology.
  • [MeSH-major] Adrenal Cortex Neoplasms / complications. Enchondromatosis / complications
  • [MeSH-minor] Abdomen / pathology. Adrenocorticotropic Hormone / blood. Adult. Amputation. Bone Neoplasms / pathology. Bone Neoplasms / radiography. Female. Femoral Neoplasms / pathology. Femoral Neoplasms / radiography. Fingers / surgery. Hemangioma / complications. Hemangioma / pathology. Humans. Hydrocortisone / blood. Leg / pathology. Magnetic Resonance Imaging. Sarcoma / pathology. Sarcoma / surgery. Uterine Neoplasms / pathology

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  • (PMID = 19782627.001).
  • [ISSN] 1778-7254
  • [Journal-full-title] Joint, bone, spine : revue du rhumatisme
  • [ISO-abbreviation] Joint Bone Spine
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] France
  • [Chemical-registry-number] 9002-60-2 / Adrenocorticotropic Hormone; WI4X0X7BPJ / Hydrocortisone
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55. Soga H, Takenaka A, Ooba T, Nakano Y, Miyake H, Takeda M, Tanaka K, Hara I, Fujisawa M: A twelve-year experience with adrenal cortical carcinoma in a single institution: long-term survival after surgical treatment and transcatheter arterial embolization. Urol Int; 2009;82(2):222-6

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A twelve-year experience with adrenal cortical carcinoma in a single institution: long-term survival after surgical treatment and transcatheter arterial embolization.
  • INTRODUCTION: We retrospectively analyzed 6 cases of adrenal cortical carcinoma (ACC) treated during a 12-year period at a single institution.
  • The mean age at diagnosis was 53.3 years (range 36-72).
  • [MeSH-major] Adrenal Cortex Neoplasms / therapy. Adrenalectomy. Adrenocortical Carcinoma / therapy. Chemoembolization, Therapeutic
  • [MeSH-minor] Adult. Aged. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Neoplasms / secondary. Bone Neoplasms / therapy. Female. Humans. Japan. Liver Neoplasms / secondary. Liver Neoplasms / therapy. Male. Middle Aged. Neoplasm Staging. Reoperation. Retrospective Studies. Survival Analysis. Time Factors. Tomography, X-Ray Computed. Treatment Outcome

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  • [Copyright] Copyright 2009 S. Karger AG, Basel.
  • (PMID = 19322014.001).
  • [ISSN] 1423-0399
  • [Journal-full-title] Urologia internationalis
  • [ISO-abbreviation] Urol. Int.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Switzerland
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56. Cham E, Watkin W, Goldschmidt R, Liu L: Fine needle aspiration cytology of adrenocortical oncocytic neoplasm: a case report. Acta Cytol; 2010 Jul-Aug;54(4):627-34

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Fine needle aspiration cytology of adrenocortical oncocytic neoplasm: a case report.
  • BACKGROUND: Adrenocortical oncocytic neoplasms (AONs) are rare tumors that typically show marked nuclear pleomorphism and eosinophilic cytoplasm and are highly cellular on fine needle aspiration (FNA) smears.
  • These features, worrisome in conventional adrenocortical tumors, are not necessarily signs of malignancy in AONs.
  • Computed tomography showed a 10-cm, solid, left adrenal mass and a 21-cm complex cystic mass in the pelvis.
  • FNA of the adrenal mass showed hypercellular smears with dyscohesive cells having pleomorphic nuclei and abundant, granular cytoplasm.
  • Resection of the adrenal mass demonstrated an AON without definite malignant features.
  • CONCLUSION: On FNA, cells from an AON can be hypercellular and cytologically atypical, which can be pitfalls for a malignant diagnosis.
  • [MeSH-major] Adenoma, Oxyphilic / pathology. Adrenal Cortex Neoplasms / pathology
  • [MeSH-minor] Biopsy, Fine-Needle. Female. Fibroma / pathology. Humans. Middle Aged. Neoplasms, Multiple Primary. Ovarian Neoplasms / pathology. Tomography, X-Ray Computed. Treatment Outcome

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  • (PMID = 20715669.001).
  • [ISSN] 0001-5547
  • [Journal-full-title] Acta cytologica
  • [ISO-abbreviation] Acta Cytol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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57. Hahner S, Fassnacht M, Hammer F, Schammann M, Weismann D, Hansen IA, Allolio B: Evidence against a role of human airway trypsin-like protease--the human analogue of the growth-promoting rat adrenal secretory protease--in adrenal tumourigenesis. Eur J Endocrinol; 2005 Jan;152(1):143-53

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Evidence against a role of human airway trypsin-like protease--the human analogue of the growth-promoting rat adrenal secretory protease--in adrenal tumourigenesis.
  • OBJECTIVE: A serine protease from rat adrenal cortex was recently characterized and named adrenal secretory protease (AsP).
  • AsP is expressed in the adrenal cortex and is capable of cleaving pro-gamma-melanocyte-stimulating hormone (1-76 N-terminus of pro-opiomelanocortin) into fragments that act as adrenal mitogens.
  • AsP may therefore play a crucial role in adrenal growth and tumourigenesis.
  • The aim of this study was to further characterize the human homologue of AsP and its possible role in adrenal tumourigenesis.
  • Further analysis revealed that the HAT gene is the human homologue of a long splice variant of AsP, which we recently described as rat airway trypsin-like serine protease 1.
  • In contrast to rodents, no short isoform of HAT was found in humans due to a stop codon in exon 6 which prevents the expression of a short isoform.
  • While high expression of HAT mRNA was found in the trachea and in the gastrointestinal tract, expression in the adrenal was only very weak.
  • We further investigated HAT expression in five normal adrenal glands, 15 adrenocortical adenomas (five hormonally inactive adenomas, five aldosterone-producing adenomas and five cortisol-producing adenomas), nine adrenocortical carcinomas, five phaeochromocytomas and two adrenal hyperplasias.
  • Weak HAT expression was detectable in only two out of five normal adrenal glands, in one out of twenty-four adrenocortical tumours and four out of five phaeochromocytomas.
  • However, the expression in the adrenal tissue was several orders of magnitude lower than in the trachea.
  • In addition, we could not detect any HAT transcripts in a sample of fetal adrenal.
  • CONCLUSION: Gene structure and tissue distribution of HAT, the human homologue of the rat adrenal secretory protease AsP, reveal major interspecies differences.
  • The observation of very low expression levels in normal adrenal tissue and adrenocortical tumours casts doubt about a role for HAT in the physiological and pathological growth of adrenocortical cells.

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  • (PMID = 15762198.001).
  • [ISSN] 0804-4643
  • [Journal-full-title] European journal of endocrinology
  • [ISO-abbreviation] Eur. J. Endocrinol.
  • [Language] ENG
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Protein Isoforms; 0 / RNA, Neoplasm; EC 3.4.21.- / Serine Endopeptidases; EC 3.4.21.- / human airway trypsin-like protease
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58. Tissier F: [Pathology of adrenocortical tumors: review and recent data]. Ann Endocrinol (Paris); 2009 Jun;70(3):179-85

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Pathology of adrenocortical tumors: review and recent data].
  • [Transliterated title] Anatomie pathologique des tumeurs corticosurrénaliennes de l'adulte : état des lieux et données récentes.
  • Most adrenocortical tumors are benign; adrenocortical carcinomas are rare but their prognosis is poor and their therapeutic is sparse.
  • In most adrenocortical tumors, the morphological approach brings sufficient elements to establish the differential diagnosis between a benign and a malignant tumor but in few cases, it is insufficient.
  • Moreover, morphology is limited for predicting prognosis of adrenocortical carcinomas.
  • These genetics findings already have repercussions for the patients in the development of molecular markers for diagnosis and prognosis and in the future they could help in the development of new morphological approaches, in particular immunohistochemical approaches.
  • [MeSH-major] Adrenal Cortex Neoplasms / pathology
  • [MeSH-minor] Cyclin E / genetics. Gene Expression Regulation, Neoplastic. Insulin-Like Growth Factor II / genetics. Mitosis. Mutation. Necrosis / pathology. Neoplasm Metastasis / pathology. Pheochromocytoma / genetics. Pheochromocytoma / pathology. Prognosis. Tumor Suppressor Protein p53 / genetics. beta Catenin / genetics

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  • (PMID = 19298951.001).
  • [ISSN] 0003-4266
  • [Journal-full-title] Annales d'endocrinologie
  • [ISO-abbreviation] Ann. Endocrinol. (Paris)
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] France
  • [Chemical-registry-number] 0 / Cyclin E; 0 / Tumor Suppressor Protein p53; 0 / beta Catenin; 67763-97-7 / Insulin-Like Growth Factor II
  • [Number-of-references] 77
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59. Sbiera S, Schmull S, Assie G, Voelker HU, Kraus L, Beyer M, Ragazzon B, Beuschlein F, Willenberg HS, Hahner S, Saeger W, Bertherat J, Allolio B, Fassnacht M: High diagnostic and prognostic value of steroidogenic factor-1 expression in adrenal tumors. J Clin Endocrinol Metab; 2010 Oct;95(10):E161-71

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] High diagnostic and prognostic value of steroidogenic factor-1 expression in adrenal tumors.
  • CONTEXT: No immunohistochemical marker has been established to reliably differentiate adrenocortical tumors from other adrenal masses.
  • We hypothesized that expression of steroidogenic factor-1 (SF-1), a transcription factor involved in adrenal development, is of value for the differential diagnosis of adrenal masses and predicts prognosis in adrenocortical carcinoma (ACC).
  • PATIENTS AND METHODS: SF-1 protein expression was assessed by immunohistochemistry on tissue samples from 167 ACC, 52 adrenocortical adenomas (ACA), six normal adrenal glands, six normal ovaries and 73 neoplastic nonsteroidogenic tissues.
  • In addition, SF-1 mRNA expression was present in all 91 analyzed adrenocortical tumors.
  • In contrast, SF-1 expression was absent in all nonsteroidogenic tumors.
  • Strong SF-1 protein expression significantly correlated with poor clinical outcome: tumor stage-adjusted hazard ratio for death 2.46 [95% confidence interval (CI) = 1.30-4.64] and for recurrence 3.91 (95% CI = 1.71-8.94).
  • Similar results were obtained in the independent cohort using RNA analysis [tumor stage-adjusted hazard ratio for death 4.69 (95% CI = 1.44-15.30)].
  • CONCLUSION: SF-1 is a highly valuable immunohistochemical marker to determine the adrenocortical origin of an adrenal mass with high sensitivity and specificity.
  • [MeSH-major] Adrenal Cortex Neoplasms / diagnosis. Adrenocortical Adenoma / diagnosis. Adrenocortical Carcinoma / diagnosis. Steroidogenic Factor 1 / genetics
  • [MeSH-minor] Adult. Biomarkers, Tumor / genetics. Biomarkers, Tumor / metabolism. Cohort Studies. Diagnosis, Differential. Female. Gene Expression Regulation, Neoplastic. Humans. Immunohistochemistry. Male. Middle Aged. Neoplasm Metastasis. Neoplasm Staging. Predictive Value of Tests. Prognosis. Sensitivity and Specificity

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  • (PMID = 20660055.001).
  • [ISSN] 1945-7197
  • [Journal-full-title] The Journal of clinical endocrinology and metabolism
  • [ISO-abbreviation] J. Clin. Endocrinol. Metab.
  • [Language] eng
  • [Publication-type] Evaluation Studies; Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / NR5A1 protein, human; 0 / Steroidogenic Factor 1
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60. Rossbach HC, Baschinsky D, Wynn T, Obzut D, Sutcliffe M, Tebbi C: Composite adrenal anaplastic neuroblastoma and virilizing adrenocortical tumor with germline TP53 R248W mutation. Pediatr Blood Cancer; 2008 Mar;50(3):681-3
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Composite adrenal anaplastic neuroblastoma and virilizing adrenocortical tumor with germline TP53 R248W mutation.
  • Composite tumors are extremely rare.
  • Such tumors in adrenal glands are usually of neuroendocrine-neural type and occur mostly in adults.
  • We report a patient with composite neuroblastoma (NB), adrenocortical tumor (ACT), and Li-Fraumeni syndrome (LFS) with germline TP53 R248W mutation.
  • LFS predisposes to the development of leukemia, sarcomas, adrenocortical and breast carcinomas, brain tumors and, questionably, NB.
  • [MeSH-major] Adrenal Cortex Neoplasms / pathology. Adrenal Gland Neoplasms / pathology. Adrenocortical Adenoma / pathology. Genes, p53. Germ-Line Mutation. Li-Fraumeni Syndrome / genetics. Mutation, Missense. Neoplasms, Multiple Primary / pathology. Neuroblastoma / pathology. Point Mutation. Virilism / etiology
  • [MeSH-minor] Amino Acid Substitution. Aneuploidy. Brain Neoplasms / genetics. Breast Neoplasms / genetics. Carcinoma / genetics. Choroid Plexus Neoplasms / genetics. Female. Glioblastoma / genetics. Humans. Infant. Loss of Heterozygosity. Male. Pedigree

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  • [Copyright] (c) 2007 Wiley-Liss, Inc.
  • (PMID = 17427234.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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61. Geller JL, Azer PC, Weiss LM, Mertens RB: Pigmented adrenocortical carcinoma: case report and review. Endocr Pathol; 2006;17(3):297-304
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Pigmented adrenocortical carcinoma: case report and review.
  • Darkly pigmented adrenocortical neoplasms are rare tumors that are often referred to as "black adenomas," indicative of both their pigmented nature and their invariably benign clinical behavior in previously reported cases.
  • We herein describe an exceptional case of a malignant pigmented adrenocortical neoplasm, with late recurrence and metastasis.
  • At age 53, this female patient was diagnosed with Cushing's syndrome and underwent a laparoscopic right adrenalectomy, revealing a 3 cm well-circumscribed, darkly pigmented adrenocortical tumor.
  • The tumor exhibited several atypical histologic features and was diagnosed as an atypical pigmented adrenal cortical neoplasm of uncertain malignant potential.
  • At subsequent exploratory laparotomy, three separate tumor nodules exhibiting varying degrees of pigmentation and ranging from 2.2 to 3.3 cm maximum dimension were excised.
  • Histologically, the tumor nodules were consistent with local recurrence/metastasis of the patient's previously excised pigmented adrenocortical neoplasm.
  • [MeSH-major] Adrenal Cortex Neoplasms / pathology. Adrenocortical Carcinoma / secondary. Pigmentation. Retroperitoneal Neoplasms / secondary
  • [MeSH-minor] Adrenalectomy. Cushing Syndrome / etiology. Female. Humans. Middle Aged. Neoplasm Recurrence, Local / pathology. Neoplasm Recurrence, Local / surgery

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  • (PMID = 17308367.001).
  • [ISSN] 1046-3976
  • [Journal-full-title] Endocrine pathology
  • [ISO-abbreviation] Endocr. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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62. Bernini GP, Moretti A, Mannelli M, Ercolino T, Bardini M, Caramella D, Taurino C, Salvetti A: Unique association of non-functioning pheochromocytoma, ganglioneuroma, adrenal cortical adenoma, hepatic and vertebral hemangiomas in a patient with a new intronic variant in the VHL gene. J Endocrinol Invest; 2005 Dec;28(11):1032-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Unique association of non-functioning pheochromocytoma, ganglioneuroma, adrenal cortical adenoma, hepatic and vertebral hemangiomas in a patient with a new intronic variant in the VHL gene.
  • This was characterized by right adrenal pheochromocytoma associated with homolateral ganglioneuroma and controlateral adrenal cortical adenoma.
  • The three tumors, incidentally discovered, proved to be non-functioning (normal secretion of catecholamines and of other neuroendocrine peptides, glucocorticoids, mineralcorticoids and androgens).
  • Accordingly, the patient showed no sign or symptom of endocrine disease.
  • Computed tomography (CT) and magnetic resonance (MR) demonstrated a typical adenomatous lesion on the left adrenal gland with precocious uptake of the radiotracer on radioidine (131I)-norcholesterol adrenal scintigraphy, while the controlateral gland showed hyperdensity on CT, hyperintensity on MR and no uptake at adrenal scintigraphy.
  • The right adrenal gland was surgically removed and, microscopically, pheochromocytoma and ganglioneuroma areas appeared intermixed without a predominant component.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Adrenocortical Adenoma / genetics. Ganglioneuroma / genetics. Hemangioma / genetics. Liver Neoplasms / genetics. Neoplasms, Multiple Primary. Pheochromocytoma / genetics. Spinal Neoplasms / genetics. Von Hippel-Lindau Tumor Suppressor Protein / genetics
  • [MeSH-minor] Adrenal Cortex Neoplasms / genetics. Adrenal Cortex Neoplasms / pathology. Aged. DNA, Neoplasm / analysis. Female. Genetic Variation. Humans. Introns / genetics. Magnetic Resonance Imaging. Tomography, X-Ray Computed


63. Lombardi CP, Raffaelli M, Pani G, Maffione A, Princi P, Traini E, Galeotti T, Rossi ED, Fadda G, Bellantone R: Gene expression profiling of adrenal cortical tumors by cDNA macroarray analysis. Results of a preliminary study. Biomed Pharmacother; 2006 May;60(4):186-90
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  • [Title] Gene expression profiling of adrenal cortical tumors by cDNA macroarray analysis. Results of a preliminary study.
  • Adrenocortical carcinoma (ACC) are highly malignant tumors with poor prognosis.
  • To verify if it is possible to assess their differential gene expression by a cDNA macroarray analysis using RNA extracted from paraffin sections, we analyzed two different cohorts of adrenal cortical adenoma (ACA) and ACC.
  • Heat shock protein 60 (HSP-60) (ratio>2), Ciclin D1 and topoisomerase I (ratio>1.5) were overexpressed in the ACC cohort, while jun proto-oncogene was down-regulated. cDNA macroarray analysis from paraffin sections of adrenal tumors is feasible, despite with a low success rate.
  • [MeSH-major] Adrenal Cortex Neoplasms / genetics. Adrenocortical Carcinoma / genetics. Gene Expression Profiling. Gene Expression Regulation, Neoplastic / genetics. Oligonucleotide Array Sequence Analysis
  • [MeSH-minor] Down-Regulation. Humans. Neoplasm Proteins / genetics. Up-Regulation


64. Alsultan A, Lovell MA, Hayes KL, Allshouse MJ, Garrington TP: Simultaneous occurrence of right adrenocortical tumor and left adrenal neuroblastoma in an infant with Beckwith-Wiedemann syndrome. Pediatr Blood Cancer; 2008 Nov;51(5):695-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Simultaneous occurrence of right adrenocortical tumor and left adrenal neuroblastoma in an infant with Beckwith-Wiedemann syndrome.
  • Children with Beckwith-Wiedemann syndrome (BWS) have increased risk for development of embryonal tumors.
  • We present the case of an infant with BWS who has hypomethylation of LIT1 gene in the 11p15.5 chromosomal region and at 6 months of age presented with simultaneous occurrence of neuroblastoma arising from the left adrenal gland and a right adrenocortical tumor.
  • She underwent surgical resection of both tumors and remains tumor free 18 months after surgery.
  • [MeSH-major] Adrenal Cortex Neoplasms / complications. Adrenal Gland Neoplasms / complications. Beckwith-Wiedemann Syndrome / complications. Neoplasms, Multiple Primary / complications. Neuroblastoma / complications


65. Mann MW, Ellis SS, Mallory SB: Infantile acne as the initial sign of an adrenocortical tumor. J Am Acad Dermatol; 2007 Feb;56(2 Suppl):S15-8
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  • [Title] Infantile acne as the initial sign of an adrenocortical tumor.
  • Ultrasound and abdominal computed tomographic scan revealed a large adrenal mass consistent with an adrenocortical tumor.
  • The patient underwent surgical excision of the well-encapsulated tumor with normalization of his hormones and no subsequent recurrence.
  • Although rare, childhood adrenocortical tumors have a poor prognosis, with the majority of tumors having regional and metastatic disease.
  • Because early diagnosis and complete surgical excision improve prognosis, children with refractory infantile acne should be evaluated for signs of virilization and accelerated growth.
  • Elevated levels of DHEA and DHEA-S should prompt an aggressive diagnostic evaluation for an adrenocortical tumor.
  • [MeSH-major] Acne Vulgaris / etiology. Adrenal Cortex Neoplasms / complications. Adrenocortical Adenoma / complications

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  • (PMID = 17097383.001).
  • [ISSN] 1097-6787
  • [Journal-full-title] Journal of the American Academy of Dermatology
  • [ISO-abbreviation] J. Am. Acad. Dermatol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 3XMK78S47O / Testosterone; 459AG36T1B / Dehydroepiandrosterone
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66. Lichtenauer UD, Shapiro I, Geiger K, Quinkler M, Fassnacht M, Nitschke R, Rückauer KD, Beuschlein F: Side population does not define stem cell-like cancer cells in the adrenocortical carcinoma cell line NCI h295R. Endocrinology; 2008 Mar;149(3):1314-22
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Side population does not define stem cell-like cancer cells in the adrenocortical carcinoma cell line NCI h295R.
  • Recent evidence suggests the existence of a stem cell-like subpopulation of cells in hematological and solid tumor entities, which determine the malignant phenotype of a given tumor through their proliferative potential and chemotherapy resistance.
  • Herein we demonstrate the presence of SP cells in a variety of adrenal specimens, including primary cultures of human adrenocortical tumors and normal adrenal glands as well as established human and murine adrenocortical cancer cell lines by fluorescence-activated cell sorter analysis and confocal microscopy.
  • On a functional level, SP cells from the human adrenocortical tumor cell line NCI h295R revealed an expression pattern consistent with a less differentiated phenotype, including lower expression of steroidogenic enzymes such as steroid acute regulatory protein (StAR) and side-chain cleavage enzyme (P450scc) in comparison with non-SP cells.
  • Similarly to the baseline growth kinetics, no survival benefit was evident in SP cells after treatment with cytotoxic agents commonly used in adrenocortical carcinomas.
  • Taken together, these findings provide evidence that Hoechst dye exclusion, in contrast to what has been reported for other tumor entities, is not a major tumor stem cell defining marker in adrenocortical NCI h295R tumor cells.
  • [MeSH-major] Adrenal Cortex Neoplasms / pathology. Adrenocortical Carcinoma / pathology. Neoplastic Stem Cells / cytology
  • [MeSH-minor] Adrenal Glands / cytology. Antineoplastic Agents / pharmacology. Antineoplastic Agents / therapeutic use. Cell Cycle / physiology. Cell Differentiation / physiology. Cell Line, Tumor. Cell Proliferation. Cholesterol Side-Chain Cleavage Enzyme / metabolism. Coloring Agents. Drug Resistance, Neoplasm / physiology. Humans. Phenotype. Phosphoproteins / metabolism. Tumor Cells, Cultured

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  • (PMID = 18063677.001).
  • [ISSN] 0013-7227
  • [Journal-full-title] Endocrinology
  • [ISO-abbreviation] Endocrinology
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Coloring Agents; 0 / Phosphoproteins; 0 / steroidogenic acute regulatory protein; EC 1.14.15.6 / Cholesterol Side-Chain Cleavage Enzyme
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67. Sane T: [Mitotane in the treatment of adrenal carcinoma]. Duodecim; 2010;126(17):2040-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Mitotane in the treatment of adrenal carcinoma].
  • Mitotane has been used for 50 years as the first-line drug in the treatment of disseminated adrenocortical carcinoma.
  • It reduces local recurrence of the disease and development of metastases even after a seemingly total surgical removal of the tumor.
  • [MeSH-major] Adrenal Cortex Neoplasms / drug therapy. Adrenocortical Carcinoma / drug therapy. Antineoplastic Agents, Hormonal / therapeutic use. Mitotane / therapeutic use
  • [MeSH-minor] Humans. Neoplasm Metastasis / prevention & control. Neoplasm Recurrence, Local / prevention & control

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  • (PMID = 21053521.001).
  • [ISSN] 0012-7183
  • [Journal-full-title] Duodecim; lääketieteellinen aikakauskirja
  • [ISO-abbreviation] Duodecim
  • [Language] fin
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Finland
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Hormonal; 78E4J5IB5J / Mitotane
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68. Wright CB, Brennan L, Brophy P, Kirsh G, Shapiro M, Potter B, Giss S, Lindeman KE, Obial R, Fannin E: Adrenocortical tumor with left renal vein, vena cava and intrahepatic venous extension. J Cardiovasc Surg (Torino); 2008 Feb;49(1):79-81
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Adrenocortical tumor with left renal vein, vena cava and intrahepatic venous extension.
  • She proved, however, to have an adrenal cortical carcinoma which displaced the kidney, exhibiting vascular invasion within the gland and non-adherent extension into the vena cava, atrium, common hepatic vein and left renal vein, where some adherence was present.
  • This unusual tumor required extensive surgery for removal, including use of cardiopulmonary bypass, with good results.
  • [MeSH-major] Adrenal Cortex Neoplasms / pathology. Adrenocortical Carcinoma / pathology. Carcinoma, Renal Cell / diagnosis. Hepatic Veins / pathology. Kidney Neoplasms / diagnosis. Renal Veins / pathology. Vena Cava, Inferior / pathology
  • [MeSH-minor] Adrenalectomy. Adult. Cardiopulmonary Bypass. Diagnosis, Differential. Female. Heart Atria / pathology. Humans. Neoplasm Invasiveness. Nephrectomy. Treatment Outcome. Vascular Surgical Procedures

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  • (PMID = 18212691.001).
  • [ISSN] 0021-9509
  • [Journal-full-title] The Journal of cardiovascular surgery
  • [ISO-abbreviation] J Cardiovasc Surg (Torino)
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Italy
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69. Miyoshi Y, Oue T, Oowari M, Soh H, Tachibana M, Kimura S, Kiyohara Y, Yamada H, Bessyo K, Mushiake S, Homma K, Hasegawa T, Sasano H, Ozono K: A case of pediatric virilizing adrenocortical tumor resulting in hypothalamic-pituitary activation and central precocious puberty following surgical removal. Endocr J; 2009;56(8):975-82
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  • [Title] A case of pediatric virilizing adrenocortical tumor resulting in hypothalamic-pituitary activation and central precocious puberty following surgical removal.
  • We present a 6-year-old boy with a virilizing adrenocortical tumor who initially presented with peripheral precocious puberty.
  • Both serum and urinary levels of adrenal androgens were elevated.
  • The histological diagnosis was adrenocortical carcinoma according to the criteria of Weiss.
  • Following surgical removal of the androgen-producing tumor, the patient subsequently developed hypothalamic-pituitary activation and demonstrated central precocious puberty.
  • Clinical follow-up of potential secondary effects of excess hormone secretion after removal is important in some pediatric patients with virilizing adrenocortical tumor.
  • [MeSH-major] Adrenal Cortex Neoplasms / complications. Adrenal Cortex Neoplasms / surgery. Adrenocortical Carcinoma / complications. Adrenocortical Carcinoma / surgery. Hypothalamo-Hypophyseal System / physiopathology. Puberty, Precocious / etiology

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  • (PMID = 19671995.001).
  • [ISSN] 1348-4540
  • [Journal-full-title] Endocrine journal
  • [ISO-abbreviation] Endocr. J.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
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70. Misić M, Vidas Z, Skegro D, Kocman B, Jelić-Puskarić B, Kardum-Skelin I: Fine needle aspiration cytology of adrenocortical carcinoma--case report. Coll Antropol; 2010 Jun;34(2):665-9
Genetic Alliance. consumer health - Adrenocortical Carcinoma.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Fine needle aspiration cytology of adrenocortical carcinoma--case report.
  • Ultrasonography (US) revealed a solitary tumor mass, eight cm in size, of the right adrenal gland.
  • Laboratory tests showed it to be a hormonally active, androgen secreting tumor (elevated testosterone level), which was consistent with the clinical picture of the disease.
  • After histopathological analysis tumor was signed out as adrenocortical carcinoma, a low risk carcinoma according to Weiss' classification.
  • The finding was verified by computerized tomography and the patient was reoperated on.
  • Cytologic opinion was recidive of primary malignant disease.
  • ACC is a rare malignant epithelial tumor of adrenal cortical cells, with high malignant potential.
  • Morphologically (histopathology and cytology), differential diagnosis includes adenoma on the one hand, and renal cell carcinoma (RCC) and hepatocellular carcinoma (HCC) on the other hand.
  • A combined evaluation of clinical features, size or weight, microscopic appearance, immunohistochemical and molecular genetic data is necessary to ensure a correct diagnosis.
  • The purpose of this case report is to present clinical and cytomorphologic features of our case of adrenocortical carcinoma which is very rare in cytology practice.
  • [MeSH-major] Adrenal Cortex Neoplasms / pathology. Carcinoma / pathology

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  • (PMID = 20698150.001).
  • [ISSN] 0350-6134
  • [Journal-full-title] Collegium antropologicum
  • [ISO-abbreviation] Coll Antropol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Croatia
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71. Markou A, Tsigou K, Papadogias D, Kossyvakis K, Vamvakidis K, Kounadi T, Piaditis G: A unique case of a benign adrenocortical tumor with triple secretion of cortisol, androgens, and aldosterone: development of multiple sclerosis after surgical removal of the tumor. Hormones (Athens); 2005 Oct-Dec;4(4):226-30
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  • [Title] A unique case of a benign adrenocortical tumor with triple secretion of cortisol, androgens, and aldosterone: development of multiple sclerosis after surgical removal of the tumor.
  • We present a 39-year old female with a benign adrenal tumor characterized by autonomous secretion of cortisol, androgens, and aldosterone.
  • CT of the adrenals revealed a 2.5 x 3.0 cm tumor with characteristics of an adenoma on the left adrenal gland.
  • Further investigations revealed suppressed basal ACTH levels, loss of diurnal rhythm of cortisol, and failure to suppress on low dose dexamethasone suppression test, suggesting autonomous cortisol secretion by the tumor.
  • Complete clinical and biochemical remission of the disease was observed after left adrenalectomy.
  • Histology confirmed the presence of an adrenocortical adenoma.
  • The flare-up of an autoimmune disease (multiple sclerosis) postoperatively could be coincidental or possibly related to the high normalization of the high cortisol levels acting as a precipitating factor.
  • [MeSH-major] Adrenal Cortex Neoplasms / pathology. Adrenal Cortex Neoplasms / surgery. Adrenalectomy / adverse effects. Adrenocortical Adenoma / pathology. Adrenocortical Adenoma / surgery. Multiple Sclerosis / etiology
  • [MeSH-minor] Adult. Aldosterone / secretion. Androgens / secretion. Biopsy, Needle. Female. Follow-Up Studies. Humans. Hydrocortisone / secretion. Immunohistochemistry. Neoplasm Staging. Risk Assessment

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  • (PMID = 16613821.001).
  • [ISSN] 1109-3099
  • [Journal-full-title] Hormones (Athens, Greece)
  • [ISO-abbreviation] Hormones (Athens)
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Greece
  • [Chemical-registry-number] 0 / Androgens; 4964P6T9RB / Aldosterone; WI4X0X7BPJ / Hydrocortisone
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72. Grubbs EG, Callender GG, Xing Y, Perrier ND, Evans DB, Phan AT, Lee JE: Recurrence of adrenal cortical carcinoma following resection: surgery alone can achieve results equal to surgery plus mitotane. Ann Surg Oncol; 2010 Jan;17(1):263-70
Hazardous Substances Data Bank. MITOTANE .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Recurrence of adrenal cortical carcinoma following resection: surgery alone can achieve results equal to surgery plus mitotane.
  • BACKGROUND: A recent nonrandomized interinstitutional study reported that adjuvant mitotane following surgery for adrenocortical carcinoma (ACC) was associated with decreased recurrence.
  • SI patients had a superior disease-free survival compared with SO patients (median 25 versus 12 months, P = 0.003), and SI patients also had a superior overall survival compared with SO patients (median not reached versus 44 months, P = 0.02).
  • [MeSH-major] Adrenal Cortex Neoplasms / surgery. Adrenocortical Carcinoma / surgery. Antineoplastic Agents, Hormonal / therapeutic use. Mitotane / therapeutic use. Neoplasm Recurrence, Local / diagnosis
  • [MeSH-minor] Adolescent. Adult. Aged. Chemotherapy, Adjuvant. Child. Child, Preschool. Combined Modality Therapy. Female. Follow-Up Studies. Humans. Male. Middle Aged. Neoplasm Staging. Retrospective Studies. Survival Rate. Treatment Outcome. Young Adult

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  • (PMID = 19851811.001).
  • [ISSN] 1534-4681
  • [Journal-full-title] Annals of surgical oncology
  • [ISO-abbreviation] Ann. Surg. Oncol.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / P30 CA016672
  • [Publication-type] Clinical Trial; Comparative Study; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Hormonal; 78E4J5IB5J / Mitotane
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73. Betz MJ, Shapiro I, Fassnacht M, Hahner S, Reincke M, Beuschlein F, German and Austrian Adrenal Network: Peroxisome proliferator-activated receptor-gamma agonists suppress adrenocortical tumor cell proliferation and induce differentiation. J Clin Endocrinol Metab; 2005 Jul;90(7):3886-96
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Peroxisome proliferator-activated receptor-gamma agonists suppress adrenocortical tumor cell proliferation and induce differentiation.
  • Moreover, recent evidence has suggested that TZDs might have favorable effects in the treatment of a variety of tumors as differentiation-inducing agents.
  • Adrenocortical carcinoma (ACC) is a rare tumor entity with poor prognosis due to its highly malignant phenotype and lack of effective treatment options.
  • OBJECTIVE: The purpose of this study was to investigate effects of TZDs on adrenocortical cancer cells.
  • RESULTS: PPARgamma mRNA expression was detectable in all adrenocortical tumors including ACCs at similar levels.
  • Furthermore, incubation of the adrenocortical tumor cell line NCI h295 with the PPARgamma agonist rosiglitazone led to a decrease in cell viability, a decrease of cellular proliferation, and an increase in apoptosis as well as steroidogenesis.
  • On the molecular level, NCI h295 cells expressed higher levels of ACTH receptor (melanocortin receptor-2) mRNA upon treatment, whereas cyclin E mRNA was reduced, thus reflecting a shift toward an expression pattern found in less aggressive adrenocortical tumors in vivo.
  • [MeSH-major] Adrenal Cortex Neoplasms / drug therapy. PPAR gamma / agonists. Thiazolidinediones / pharmacology
  • [MeSH-minor] Adult. Aged. Anilides / pharmacology. Apoptosis / drug effects. Cell Differentiation. Cell Line, Tumor. Cell Proliferation / drug effects. Cyclin E / genetics. Dose-Response Relationship, Drug. Female. Humans. Insulin-Like Growth Factor II / genetics. Male. Middle Aged. Promoter Regions, Genetic. RNA, Messenger / analysis. Receptor, Melanocortin, Type 2 / genetics

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  • (PMID = 15886257.001).
  • [ISSN] 0021-972X
  • [Journal-full-title] The Journal of clinical endocrinology and metabolism
  • [ISO-abbreviation] J. Clin. Endocrinol. Metab.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / 2-chloro-5-nitrobenzanilide; 0 / Anilides; 0 / Cyclin E; 0 / PPAR gamma; 0 / RNA, Messenger; 0 / Receptor, Melanocortin, Type 2; 0 / Thiazolidinediones; 05V02F2KDG / rosiglitazone; 67763-97-7 / Insulin-Like Growth Factor II
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74. Aubert S, Buob D, Leroy X, Devos P, Carnaille B, Do Cao C, Wemeau JL, Leteurtre E: [Weiss system: a still in-use diagnostic tool for the assessment of adrenocortical malignancy]. Ann Pathol; 2005 Dec;25(6):545-54

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Weiss system: a still in-use diagnostic tool for the assessment of adrenocortical malignancy].
  • [Transliterated title] Le système de Weiss: un outil toujours d'actualité pour le diagnostic de malignité des tumeurs de la corticosurrenale.
  • Adrenocortical carcinomas are rare tumors.
  • In the absence of the gold standard that constitutes metastases, local invasion or recurrence, the diagnosis of malignancy may represent a great challenge for both clinicians and pathologists.
  • A more precise definition of the different microscopic criteria and the proposition of a modified Weiss system based on the most reliable criteria could help.
  • Besides, the Weiss system is of limited value for the diagnosis of malignancy in oxyphil tumors and children tumors of the adrenal cortex.
  • [MeSH-major] Adrenal Cortex Neoplasms / pathology
  • [MeSH-minor] Adult. Child. Humans. Mitotic Index. Necrosis. Neoplasm Invasiveness

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  • (PMID = 16735978.001).
  • [ISSN] 0242-6498
  • [Journal-full-title] Annales de pathologie
  • [ISO-abbreviation] Ann Pathol
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] France
  • [Number-of-references] 55
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75. Fudge EB, von Allmen D, Volmar KE, Calikoglu AS: Cushing Syndrome in a 6-Month-Old Infant due to Adrenocortical Tumor. Int J Pediatr Endocrinol; 2009;2009:168749

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Cushing Syndrome in a 6-Month-Old Infant due to Adrenocortical Tumor.
  • Cushing syndrome is rare in infancy and usually due to an adrenocortical tumor (ACT).
  • We report an infant with Cushing syndrome due to adrenocortical carcinoma.
  • Abdominal MRI revealed a heterogeneous right adrenal mass extending into the inferior vena cava.
  • The tumor was removed surgically en bloc.
  • In conclusion, adrenocortical neoplasms in children are rare, but should be considered in the differential diagnosis of Cushing syndrome.

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  • (PMID = 20049152.001).
  • [ISSN] 1687-9856
  • [Journal-full-title] International journal of pediatric endocrinology
  • [ISO-abbreviation] Int J Pediatr Endocrinol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Egypt
  • [Other-IDs] NLM/ PMC2798106
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76. Delhanty PJ, van Koetsveld PM, Gauna C, van de Zande B, Vitale G, Hofland LJ, van der Lely AJ: Ghrelin and its unacylated isoform stimulate the growth of adrenocortical tumor cells via an anti-apoptotic pathway. Am J Physiol Endocrinol Metab; 2007 Jul;293(1):E302-9
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  • [Title] Ghrelin and its unacylated isoform stimulate the growth of adrenocortical tumor cells via an anti-apoptotic pathway.
  • Ghrelin is expressed in normal human adrenocortical cells and induces their proliferation through growth hormone secretagogue receptor 1a (GHS-R1a).
  • Consequently, it was of interest to us to determine whether acylated ghrelin and its predominant serum isoform, unacylated ghrelin, also act as factors for adrenocortical carcinoma cell growth.
  • To examine a potential ghrelin-regulated system in adrenocortical tumors, we measured proliferative effects of acylated and unacylated ghrelin in the adrenocortical carcinoma cell lines SW-13 and NCI-H295R.
  • Acylated and unacylated ghrelin in the nanomolar range dose-dependently induced adrenocortical cell growth up to 200% of untreated controls, as measured by thymidine uptake and WST1 assay.
  • Acylated and unacylated ghrelin are potential auto/paracrine factors acting through an antiapoptotic pathway to stimulate adrenocortical tumor cell growth.
  • [MeSH-major] Adrenal Cortex Neoplasms / pathology. Adrenocortical Carcinoma / pathology. Apoptosis / drug effects. Cell Proliferation / drug effects. Peptide Hormones / pharmacology
  • [MeSH-minor] Acetylation. Cell Cycle / drug effects. Ghrelin. Humans. Protein Isoforms / pharmacology. Receptors, Corticotropin-Releasing Hormone / antagonists & inhibitors. Receptors, Corticotropin-Releasing Hormone / genetics. Receptors, Corticotropin-Releasing Hormone / metabolism. Receptors, G-Protein-Coupled / antagonists & inhibitors. Receptors, G-Protein-Coupled / genetics. Receptors, G-Protein-Coupled / metabolism. Receptors, Ghrelin. Signal Transduction / drug effects. Tumor Cells, Cultured

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  • (PMID = 17405826.001).
  • [ISSN] 0193-1849
  • [Journal-full-title] American journal of physiology. Endocrinology and metabolism
  • [ISO-abbreviation] Am. J. Physiol. Endocrinol. Metab.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / CRF receptor type 2; 0 / Ghrelin; 0 / Peptide Hormones; 0 / Protein Isoforms; 0 / Receptors, Corticotropin-Releasing Hormone; 0 / Receptors, G-Protein-Coupled; 0 / Receptors, Ghrelin
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77. Gaujoux S, Tissier F, Groussin L, Libé R, Ragazzon B, Launay P, Audebourg A, Dousset B, Bertagna X, Bertherat J: Wnt/beta-catenin and 3',5'-cyclic adenosine 5'-monophosphate/protein kinase A signaling pathways alterations and somatic beta-catenin gene mutations in the progression of adrenocortical tumors. J Clin Endocrinol Metab; 2008 Oct;93(10):4135-40

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Wnt/beta-catenin and 3',5'-cyclic adenosine 5'-monophosphate/protein kinase A signaling pathways alterations and somatic beta-catenin gene mutations in the progression of adrenocortical tumors.
  • BACKGROUND: The Wnt/beta-catenin and cAMP signaling pathways play an important role in adrenal cortex tumorigenesis.
  • Somatic activating mutations of the beta-catenin gene (CTNNB1) are the most frequent genetic defects identified both in adrenocortical adenomas (ACAs) and adrenocortical cancers (ACCs).
  • PRKAR1A mutations leading to cAMP pathway dysregulation are observed in primary pigmented nodular adrenocortical diseases (PPNADs) and some sporadic ACAs.
  • OBJECTIVE: The objective of the investigation was to study Wnt/beta-catenin dysregulation in adrenocortical tumors (ACTs) with cAMP pathway genetic alteration and search for secondary CTNNB1 somatic mutations in heterogeneous tumors.
  • PATIENTS AND METHODS: Nine PPNADs, including five with macronodules, three ACAs with PRKAR1A somatic mutations, and one heterogeneous tumor with ACC developed within an ACA, were studied by immunohistochemistry and DNA sequencing.
  • RESULTS: beta-Catenin accumulation was observed in all PPNADs, ACAs with PRKAR1A mutations, and the ACC component of the heterogeneous tumor.
  • In addition, the occurrence as an additional hit of a CTNNB1 somatic mutation is associated with larger or more aggressive ACTs.
  • This underlines the importance of the Wnt/beta-catenin pathway in adrenal cortex tumorigenesis and the importance of genetic accumulation in the progression of ACTs.
  • [MeSH-major] Adenoma / genetics. Adrenal Cortex Neoplasms / genetics. Cyclic AMP-Dependent Protein Kinase RIalpha Subunit / genetics. Mutation. Signal Transduction / genetics. Wnt Proteins / genetics. beta Catenin / genetics
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Child. Cyclic AMP / metabolism. DNA Mutational Analysis. Disease Progression. Female. Gene Expression Regulation, Neoplastic. Humans. Male. Middle Aged. Neoplasm Invasiveness. Tumor Burden / genetics

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  • (PMID = 18647815.001).
  • [ISSN] 0021-972X
  • [Journal-full-title] The Journal of clinical endocrinology and metabolism
  • [ISO-abbreviation] J. Clin. Endocrinol. Metab.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Cyclic AMP-Dependent Protein Kinase RIalpha Subunit; 0 / PRKAR1A protein, human; 0 / Wnt Proteins; 0 / beta Catenin; E0399OZS9N / Cyclic AMP
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78. Gil J, Kalembkiewicz M, Polak E, Kostecka-Matyja M: [Disseminated adrenocortical carcinoma: case report]. Pol Arch Med Wewn; 2007 Jul;117(7):317-21
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  • [Title] [Disseminated adrenocortical carcinoma: case report].
  • [Transliterated title] Rozsiany rak kory nadnercza.
  • Adrenocortical carcinoma is a rare neoplasm occurring with a frequency of 1-2 cases per million.
  • This type of a tumor is slightly more frequent in women (58.6%) than in men (41.4%).
  • Etiology of adrenocortical carcinoma is still unclear, but a role of genetic and environmental factors has been largely considered.
  • The tumor size is still the best single predictor of prognosis.
  • Histopathology specimen from biopsy or obtained during operation should be stained for Melan A, which can confirm the adrenal origin of the tumor.
  • We presented the case of functioning adrenocortical cancer in 37-year-old patient who at time of diagnosis had 12 cm in diameter tumor of the left adrenal gland and metastases to the liver and lung.
  • In the article the symptoms associated with hormones produced by the carcinoma, diagnostics and treatment with regard to the progression of the disease have also been discussed.
  • [MeSH-major] Adrenal Cortex Neoplasms / pathology. Adrenocortical Carcinoma / pathology. Liver Neoplasms / secondary. Lung Neoplasms / secondary

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  • (PMID = 17966598.001).
  • [Journal-full-title] Polskie Archiwum Medycyny Wewnetrznej
  • [ISO-abbreviation] Pol. Arch. Med. Wewn.
  • [Language] pol
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Poland
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79. Slater EP, Diehl SM, Langer P, Samans B, Ramaswamy A, Zielke A, Bartsch DK: Analysis by cDNA microarrays of gene expression patterns of human adrenocortical tumors. Eur J Endocrinol; 2006 Apr;154(4):587-98
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  • [Title] Analysis by cDNA microarrays of gene expression patterns of human adrenocortical tumors.
  • OBJECTIVES: Adrenocortical carcinoma (ACC) is a rare malignant neoplasm with extremely poor prognosis.
  • The molecular mechanisms of adrenocortical tumorigenesis are still not well understood.
  • The comparative analysis by cDNA microarrays of gene-expression patterns of benign and malignant adrenocortical tumors allows us to identify new tumor-suppressor genes and proto-oncogenes underlying adrenocortical tumorigenesis.
  • DESIGN AND METHODS: Total RNA from fresh-frozen tissue of 10 ACC and 10 benign adrenocortical adenomas was isolated after histologic confirmation of neoplastic cellularity of at least 85%.
  • The reference consisted of pooled RNA of 10 normal adrenal cortex samples.
  • Amplified RNA of tumor and reference was used to synthesize Cy3- and Cy5-fluorescently labeled cDNA in a flip-color technique.
  • RESULTS: The comparative analysis of gene expression revealed many genes with more than fourfold expression difference between ACC and normal tissue (42 genes), cortical adenoma and normal tissue (11 genes), and ACC and cortical adenoma (21 genes) respectively.
  • As confirmed by real-time PCR, the IGF2 gene was significantly upregulated in ACCs versus cortical adenomas and normal cortical tissue.
  • Genes that were downregulated in adrenocortical tumors included chromogranin B and early growth response factor 1.
  • CONCLUSIONS: Comprehensive expression profiling of adrenocortical tumors by the cDNA microarray technique is a very powerful tool to elucidate the molecular steps associated with the tumorigenesis of these ill-defined neoplasms.

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  • (PMID = 16556722.001).
  • [ISSN] 0804-4643
  • [Journal-full-title] European journal of endocrinology
  • [ISO-abbreviation] Eur. J. Endocrinol.
  • [Language] ENG
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / RNA, Messenger; 4964P6T9RB / Aldosterone; WI4X0X7BPJ / Hydrocortisone
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80. Mazzuco TL, Chabre O, Sturm N, Feige JJ, Thomas M: Ectopic expression of the gastric inhibitory polypeptide receptor gene is a sufficient genetic event to induce benign adrenocortical tumor in a xenotransplantation model. Endocrinology; 2006 Feb;147(2):782-90
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  • [Title] Ectopic expression of the gastric inhibitory polypeptide receptor gene is a sufficient genetic event to induce benign adrenocortical tumor in a xenotransplantation model.
  • Aberrant expression of ectopic G protein-coupled receptors (GPCRs) in adrenal cortex tissue has been observed in several cases of ACTH-independent macronodular adrenal hyperplasias and adenomas associated with Cushing's syndrome.
  • Although there is clear clinical evidence for the implication of these ectopic receptors in abnormal regulation of cortisol production, whether this aberrant GPCR expression is the cause or the consequence of the development of an adrenal hyperplasia is still an open question.
  • To answer it, we genetically engineered primary bovine adrenocortical cells to have them express the gastric inhibitory polypeptide receptor.
  • We observed the formation of an enlarged and hyperproliferative adenomatous adrenocortical tissue that secreted cortisol in a gastric inhibitory polypeptide-dependent manner and induced a mild Cushing's syndrome with hyperglycemia.
  • Moreover, we show that tumor development was ACTH independent.
  • Thus, a single genetic event, inappropriate expression of a nonmutated GPCR gene, is sufficient to initiate the complete phenotypic alterations that ultimately lead to the formation of a benign adrenocortical tumor.
  • [MeSH-major] Adenoma / genetics. Adrenal Cortex / metabolism. Adrenal Gland Neoplasms / genetics. Cell Transformation, Neoplastic / metabolism. Gene Expression Regulation, Neoplastic. Receptors, Gastrointestinal Hormone / metabolism

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  • (PMID = 16254030.001).
  • [ISSN] 0013-7227
  • [Journal-full-title] Endocrinology
  • [ISO-abbreviation] Endocrinology
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Homeodomain Proteins; 0 / Receptors, G-Protein-Coupled; 0 / Receptors, Gastrointestinal Hormone; 0 / gastric inhibitory polypeptide receptor; 128559-51-3 / RAG-1 protein; WI4X0X7BPJ / Hydrocortisone
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81. Allolio B, Fassnacht M: Clinical review: Adrenocortical carcinoma: clinical update. J Clin Endocrinol Metab; 2006 Jun;91(6):2027-37
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  • [Title] Clinical review: Adrenocortical carcinoma: clinical update.
  • CONTEXT: Adrenocortical carcinoma (ACC) is a rare and heterogeneous malignancy with incompletely understood pathogenesis and poor prognosis.
  • Patients present with hormone excess (e.g. virilization, Cushing's syndrome) or a local mass effect (median tumor size at diagnosis > 10 cm).
  • The following search terms were used in varying combinations: adrenal, adrenocortical, cancer, carcinoma, tumor, diagnosis, imaging, treatment, radiotherapy, mitotane, cytotoxic, surgery.
  • EVIDENCE SYNTHESIS: Tumors typically appear inhomogeneous in both computerized tomography and magnetic resonance imaging with necroses and irregular borders and differ from benign adenomas by their low fat content.
  • Hormonal analysis reveals evidence of steroid hormone secretion by the tumor in the majority of cases, even in seemingly hormonally inactive lesions.
  • Histopathology is crucial for the diagnosis of malignancy and may also provide important prognostic information.
  • Local recurrence is frequent, particularly after violation of the tumor capsule.
  • Surgery also plays a role in local tumor recurrence and metastatic disease.
  • In advanced disease, the most promising therapeutic options (etoposide, doxorubicin, cisplatin plus mitotane, and streptozotocin plus mitotane) are currently being compared in an international phase III trial (www.firm-act.org).
  • Adjuvant treatment options after complete tumor removal (e.g. mitotane, radiotherapy) are urgently needed because postoperative disease-free survival at 5 yr is only around 30%, but options have still not been convincingly established.
  • [MeSH-major] Adrenal Cortex Neoplasms / therapy
  • [MeSH-minor] Follow-Up Studies. Humans. Neoplasm Staging. Prognosis

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  • (PMID = 16551738.001).
  • [ISSN] 0021-972X
  • [Journal-full-title] The Journal of clinical endocrinology and metabolism
  • [ISO-abbreviation] J. Clin. Endocrinol. Metab.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Number-of-references] 156
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82. De Groot PC, Van Kamp IL, Zweers EJ, De Kroon CD, Van Wijngaarden WJ: Oligohydramnios in a pregnant woman with Cushing's syndrome caused by an adrenocortical adenoma. J Matern Fetal Neonatal Med; 2007 May;20(5):431-4
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  • [Title] Oligohydramnios in a pregnant woman with Cushing's syndrome caused by an adrenocortical adenoma.
  • A 28-year-old primigravida with severe oligohydramnios and pre-eclamptic symptoms was found to have Cushing's syndrome caused by an adrenocortical tumor.
  • As clinical features of pre-eclampsia and Cushing's syndrome may overlap, diagnosis can be delayed or even missed.
  • [MeSH-major] Adrenal Cortex Neoplasms / diagnosis. Cushing Syndrome / diagnosis. Oligohydramnios / diagnosis. Pregnancy Complications, Neoplastic / diagnosis
  • [MeSH-minor] Adenoma / complications. Adenoma / diagnosis. Adenoma / surgery. Adrenalectomy. Adult. Diagnosis, Differential. Female. Fetal Death. Humans. Hydrocortisone / therapeutic use. Pre-Eclampsia. Pregnancy

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  • (PMID = 17674251.001).
  • [ISSN] 1476-7058
  • [Journal-full-title] The journal of maternal-fetal & neonatal medicine : the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians
  • [ISO-abbreviation] J. Matern. Fetal. Neonatal. Med.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] WI4X0X7BPJ / Hydrocortisone
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83. Lee R, Al-Ahmadie HA, Boorjian SA, Gonzalez RR, Badillo C, Badillo F, Reuter VE, Steckel J: A case of incidental adrenocortical oncocytoma. Nat Clin Pract Urol; 2006 Nov;3(11):618-21

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  • [Title] A case of incidental adrenocortical oncocytoma.
  • BACKGROUND: A 39-year-old female presented with an incidentally discovered left adrenal mass.
  • DIAGNOSIS: Oncocytic adrenocortical tumor, or adrenal oncocytoma.
  • [MeSH-major] Adenoma, Oxyphilic / diagnosis. Adenoma, Oxyphilic / surgery. Adrenal Cortex Neoplasms / diagnosis. Adrenal Cortex Neoplasms / surgery

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  • (PMID = 17088930.001).
  • [ISSN] 1743-4289
  • [Journal-full-title] Nature clinical practice. Urology
  • [ISO-abbreviation] Nat Clin Pract Urol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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84. Tissier F: [Sporadic adrenocortical tumors: genetics and perspectives for the pathologist]. Ann Pathol; 2008 Oct;28(5):409-16
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  • [Title] [Sporadic adrenocortical tumors: genetics and perspectives for the pathologist].
  • [Transliterated title] Tumeurs corticosurrénaliennes sporadiques de l'adulte : aspects génétiques et perspectives pour le pathologiste.
  • Most adrenocortical tumors are benign; adrenocortical carcinomas are rare but their prognosis is poor and few therapeutic options are available.
  • In most adrenocortical tumors, the morphological approach provides enough elements to establish the differential diagnosis between a benign and a malignant tumor but in few cases, it is insufficient.
  • Moreover, morphology is limited for predicting prognosis of adrenocortical carcinomas.
  • The comprehension of the genetic syndromes associated with adrenocortical tumors led to progress in the identification of genetic abnormalities involved in sporadic adrenocortical tumorigenesis.
  • Thus, in sporadic adrenocortical tumorigenesis, IGF-II overexpression and cyclin E overproduction have been associated with 11p15 alterations which are observed in Bethwith-Wiedemann syndrome and TP53 inactivating mutations and 17p13 locus abnormalities which are observed in Li-Fraumeni syndrome.
  • Activation of the Wnt/ss-catenin signaling pathway which is observed in familial adenomatous polyposis has been found in adrenocortical adenomas and carcinomas associated to mutations of CTNNB1, the gene coding ss-catenin, suggesting a central role for this pathway in adrenocortical tumorigenesis.
  • These genetics findings already have had repercussions for patients via the development of molecular markers for diagnosis and prognosis; in the future they should be helpful in the development of new therapeutics.
  • [MeSH-major] Adrenal Cortex Neoplasms / genetics. Adrenal Cortex Neoplasms / pathology

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  • (PMID = 19068395.001).
  • [ISSN] 0242-6498
  • [Journal-full-title] Annales de pathologie
  • [ISO-abbreviation] Ann Pathol
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] France
  • [Chemical-registry-number] 0 / Cyclic AMP-Dependent Protein Kinase RIalpha Subunit; 0 / PRKAR1A protein, human; 67763-97-7 / Insulin-Like Growth Factor II; EC 3.1.4.- / Phosphoric Diester Hydrolases; EC 3.1.4.35 / PDE11A protein, human
  • [Number-of-references] 73
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85. Hunger-Battefeld W, Gajda M, Hansch A, Mandecka A, Müller UA, Wolf G: [Diagnostic pitfalls with Cushing's syndrome]. Internist (Berl); 2010 Mar;51 Suppl 1:293-302
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  • Adrenal cortical carcinoma is a rare diagnosis and may present with hormone secretion.
  • A histological differentiation between an adrenal cortical adenoma and carcinoma can be very difficult.
  • However, a fast diagnosis including staging and complete surgical resection is pivotal for the prognosis of an adrenal cortical carcinoma.
  • Metastasing adrenal cortical carcinoma should be treated with a mitotane based chemotherapy, and inclusion in the "firm-act study" is highly recommended.
  • The present case report demonstrates the diagnostic pitfalls in a female patients with Cushing's syndrome who suffered from metastasing adrenal cortical carcinoma.
  • [MeSH-major] Adrenal Cortex Neoplasms / diagnosis. Adrenocortical Adenoma / diagnosis. Carcinoma, Renal Cell / diagnosis. Cushing Syndrome / diagnosis. Kidney Neoplasms / diagnosis. Neoplasms, Multiple Primary / diagnosis
  • [MeSH-minor] Adrenal Cortex / pathology. Adrenalectomy. Circadian Rhythm / physiology. Diabetes Mellitus, Type 2 / etiology. Diagnosis, Differential. Disease Progression. Female. Follow-Up Studies. Humans. Hydrocortisone / blood. Hypertension / etiology. Kidney / pathology. Liver Neoplasms / pathology. Liver Neoplasms / secondary. Lung Neoplasms / pathology. Lung Neoplasms / secondary. Middle Aged. Neoplasm Recurrence, Local / diagnosis. Neoplasm Recurrence, Local / pathology. Obesity, Morbid / etiology. Weight Gain

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  • (PMID = 20012255.001).
  • [ISSN] 1432-1289
  • [Journal-full-title] Der Internist
  • [ISO-abbreviation] Internist (Berl)
  • [Language] ger
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] WI4X0X7BPJ / Hydrocortisone
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86. Sandrini F, Villani DP, Tucci S, Moreira AC, de Castro M, Elias LL: Inheritance of R337H p53 gene mutation in children with sporadic adrenocortical tumor. Horm Metab Res; 2005 Apr;37(4):231-5
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  • [Title] Inheritance of R337H p53 gene mutation in children with sporadic adrenocortical tumor.
  • Adrenocortical tumors (ACTs) are frequent in Brazil.
  • The mechanisms of adrenal tumorigenesis remain poorly established; the R337H germline mutation in the p53 gene has previously been associated with ACTs in Brazilian children.
  • DNA was extracted from peripheral blood cells and/or tumor tissue for sequencing exon 10 of the p53 gene.
  • Two members of the adult group showed asymptomatic adrenal incidentalomas, two showed virilization, and one presented with Cushing's syndrome.
  • The R337H mutation was found in fifteen samples of the nineteen tumor specimens available (78.9%).
  • None of the parents showed ACTs or any other neoplasia at the time of the study.
  • There was no association between the presence of germline or tissue R337H p53 mutation and malignancy at diagnosis.
  • The R337H p53 mutation was inherited from one of the parents of the patients, and there was no association between the presence of this mutation and tumor malignancy in children.
  • [MeSH-major] Adrenal Cortex Neoplasms / genetics. Genes, p53 / genetics. Mutation / genetics

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  • (PMID = 15952083.001).
  • [ISSN] 0018-5043
  • [Journal-full-title] Hormone and metabolic research = Hormon- und Stoffwechselforschung = Hormones et métabolisme
  • [ISO-abbreviation] Horm. Metab. Res.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Codon; 9007-49-2 / DNA
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87. Fernández-Guarino M, Harto A, Pérez-García B, Montull C, De Las Heras E, Jaén P: [Plaque-phase mycosis fungoides treated with photodynamic therapy: results from 12 patients]. Actas Dermosifiliogr; 2010 Nov;101(9):785-91
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  • [Transliterated title] Micosis fungoide en fase de placas tratada con terapia fotodinámica: resultados en 12 pacientes.
  • BACKGROUND: Recent years have seen an increase in the off-label use of photodynamic therapy for the treatment of a variety of skin diseases.
  • [MeSH-major] Aminolevulinic Acid / analogs & derivatives. Mycosis Fungoides / drug therapy. Photochemotherapy. Photosensitizing Agents / therapeutic use. Skin Neoplasms / drug therapy
  • [MeSH-minor] Adrenal Cortex Hormones / therapeutic use. Adult. Combined Modality Therapy. Female. Fluorescence. Humans. Male. Middle Aged. Neoplasm Recurrence, Local. Occlusive Dressings. Patient Acceptance of Health Care. Prospective Studies. Remission Induction. Treatment Outcome

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  • (PMID = 21034709.001).
  • [ISSN] 1578-2190
  • [Journal-full-title] Actas dermo-sifiliográficas
  • [ISO-abbreviation] Actas Dermosifiliogr
  • [Language] spa
  • [Publication-type] Clinical Trial; English Abstract; Journal Article
  • [Publication-country] Spain
  • [Chemical-registry-number] 0 / Adrenal Cortex Hormones; 0 / Photosensitizing Agents; 0 / methyl 5-aminolevulinate; 88755TAZ87 / Aminolevulinic Acid
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88. Reddy KS, Devarapalli Y, Mazur M, Hamawi K, Chakkera H, Moss A, Mekeel K, Post D, Heilman R, Mulligan D: Alemtuzumab with rapid steroid taper in simultaneous kidney and pancreas transplantation: comparison to induction with antithymocyte globulin. Transplant Proc; 2010 Jul-Aug;42(6):2006-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [MeSH-major] Antibodies, Monoclonal / therapeutic use. Antibodies, Neoplasm / therapeutic use. Antilymphocyte Serum / therapeutic use. Kidney Transplantation / immunology. Mycophenolic Acid / analogs & derivatives. Pancreas Transplantation / immunology
  • [MeSH-minor] Acute Disease. Adrenal Cortex Hormones / therapeutic use. Antibodies, Monoclonal, Humanized. Creatinine / blood. Drug Therapy, Combination. Glomerular Filtration Rate. Graft Rejection / epidemiology. Graft Rejection / prevention & control. Graft Survival. Humans. Immunosuppressive Agents / therapeutic use. Patient Selection. Retrospective Studies

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  • [Copyright] Copyright 2010 Elsevier Inc. All rights reserved.
  • (PMID = 20692393.001).
  • [ISSN] 1873-2623
  • [Journal-full-title] Transplantation proceedings
  • [ISO-abbreviation] Transplant. Proc.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Adrenal Cortex Hormones; 0 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Humanized; 0 / Antibodies, Neoplasm; 0 / Antilymphocyte Serum; 0 / Immunosuppressive Agents; 3A189DH42V / alemtuzumab; 9242ECW6R0 / mycophenolate mofetil; AYI8EX34EU / Creatinine; HU9DX48N0T / Mycophenolic Acid
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89. Szabó PM, Tamási V, Molnár V, Andrásfalvy M, Tömböl Z, Farkas R, Kövesdi K, Patócs A, Tóth M, Szalai C, Falus A, Rácz K, Igaz P: Meta-analysis of adrenocortical tumour genomics data: novel pathogenic pathways revealed. Oncogene; 2010 May 27;29(21):3163-72

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Meta-analysis of adrenocortical tumour genomics data: novel pathogenic pathways revealed.
  • Sporadic adrenocortical tumours are common, but their pathogenesis is poorly elucidated.
  • In this study, we present a meta-analysis and review of gene expression microarray and comparative genome hybridization (CGH) studies performed to date on these tumours, including our own data.
  • Data of whole genome microarray studies from altogether 164 tumours (97 benign, 67 malignant) and 18 normal tissues were reclassified and reanalysed.
  • Significant gene sets and cytogenetic changes from publications without available genomic data were also examined including 269 benign, 215 malignant tumour and 30 normal tissues.
  • In our experimental study, 11 tumour and four normal samples were analysed by parallel mRNA and CGH profiling.
  • These pathways include novel, previously undescribed pathomechanisms of adrenocortical tumours, and associated gene products may serve as diagnostic markers of malignancy and therapeutic targets.
  • [MeSH-major] Adrenal Cortex Neoplasms / genetics. Comparative Genomic Hybridization. Genomics. Meta-Analysis as Topic
  • [MeSH-minor] Chromosome Aberrations. Gene Expression Profiling. Humans. Oligonucleotide Array Sequence Analysis. RNA, Messenger / genetics. RNA, Neoplasm / genetics

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  • (PMID = 20305693.001).
  • [ISSN] 1476-5594
  • [Journal-full-title] Oncogene
  • [ISO-abbreviation] Oncogene
  • [Language] eng
  • [Publication-type] Journal Article; Meta-Analysis; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / RNA, Messenger; 0 / RNA, Neoplasm
  • [Number-of-references] 50
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90. Vivarelli M, Dazzi A, Zanello M, Cucchetti A, Cescon M, Ravaioli M, Del Gaudio M, Lauro A, Grazi GL, Pinna AD: Effect of different immunosuppressive schedules on recurrence-free survival after liver transplantation for hepatocellular carcinoma. Transplantation; 2010 Jan 27;89(2):227-31
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  • BACKGROUND: Tumor recurrence represents the main limitation of liver transplantation in patients with hepatocellular carcinoma (HCC) and can be favored by exposure to calcineurin inhibitors.
  • METHODS: We investigated the effect of an immunosuppressant schedule that minimizes the exposure to calcineurin inhibitors on patients transplanted for HCC to ascertain whether this can reduce the tumor recurrence rate.
  • [MeSH-major] Carcinoma, Hepatocellular / surgery. Immunosuppressive Agents / therapeutic use. Liver Neoplasms / surgery. Liver Transplantation / immunology. Neoplasm Recurrence, Local / epidemiology. Sirolimus / therapeutic use. Tacrolimus / therapeutic use
  • [MeSH-minor] Adrenal Cortex Hormones / therapeutic use. Adult. Aged. Cohort Studies. Disease-Free Survival. Drug Administration Schedule. Female. Hepatitis B / complications. Hepatitis C / complications. Humans. Male. Middle Aged. Risk Factors. Survivors. Young Adult

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  • (PMID = 20098287.001).
  • [ISSN] 1534-6080
  • [Journal-full-title] Transplantation
  • [ISO-abbreviation] Transplantation
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Adrenal Cortex Hormones; 0 / Immunosuppressive Agents; W36ZG6FT64 / Sirolimus; WM0HAQ4WNM / Tacrolimus
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91. Nguyen AT, Hashemi S, Castellano MR, Eisenbraun M, Coppa GF: A new subtype of Hodgkin's lymphoma, syncytial nodular sclerosing: first case report of primary small bowel lymphoma. J Gastrointest Cancer; 2009;40(1-2):38-40
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  • INTRODUCTION: Gastrointestinal lymphomas may arise as a component of systemic disease with GI involvement or primary neoplasm.
  • The diagnosis of primary GI lymphoma requires the lack of peripheral or mediastinal lymphadenopathy, normal white blood cell count, and differential on the peripheral blood smear; tumor involvement must be predominantly in the GI tract, and no evidence of liver or spleen involvement.
  • [MeSH-major] Hodgkin Disease / immunology. Hodgkin Disease / pathology. Immunocompromised Host. Intestinal Neoplasms / immunology. Intestinal Neoplasms / pathology. Intestine, Small / pathology
  • [MeSH-minor] Adrenal Cortex Hormones / therapeutic use. Adult. Graft Rejection / prevention & control. Humans. Kidney Transplantation. Male

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  • (PMID = 19731101.001).
  • [ISSN] 1941-6636
  • [Journal-full-title] Journal of gastrointestinal cancer
  • [ISO-abbreviation] J Gastrointest Cancer
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Adrenal Cortex Hormones
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92. Chinot OL: [Cerebral metastases]. Rev Prat; 2006 Oct 31;56(16):1799-804
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  • Improvement of diagnosis techniques and cancer treatment efficacy explain in part the increasing incidence of brain metastasis, observed in 20 to 30 % of cancer patients.
  • New techniques of MRI improve its sensibility and guide therapeutic decision, in conjunction with prognostic factors including age, Karnovsky performance score and control of the general disease.
  • [MeSH-major] Brain Neoplasms / secondary. Brain Neoplasms / therapy
  • [MeSH-minor] Administration, Oral. Adrenal Cortex Hormones / administration & dosage. Adrenal Cortex Hormones / therapeutic use. Age Factors. Aged. Breast Neoplasms. Colonic Neoplasms. Combined Modality Therapy. Humans. Karnofsky Performance Status. Kidney Neoplasms. Lung Neoplasms. Lymphoma / diagnosis. Magnetic Resonance Imaging. Magnetic Resonance Spectroscopy. Middle Aged. Neoplasm Recurrence, Local. Positron-Emission Tomography. Prognosis. Radiosurgery. Research

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  • (PMID = 17315506.001).
  • [ISSN] 0035-2640
  • [Journal-full-title] La Revue du praticien
  • [ISO-abbreviation] Rev Prat
  • [Language] fre
  • [Publication-type] Comparative Study; English Abstract; Journal Article; Review
  • [Publication-country] France
  • [Chemical-registry-number] 0 / Adrenal Cortex Hormones
  • [Number-of-references] 13
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93. Ohwada S, Izumi M, Kawate S, Hamada K, Toya H, Togo N, Horiguchi J, Koibuchi Y, Takahashi T, Yamada M: Surgical outcome of stage III and IV adrenocortical carcinoma. Jpn J Clin Oncol; 2007 Feb;37(2):108-13
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  • [Title] Surgical outcome of stage III and IV adrenocortical carcinoma.
  • BACKGROUND: Adrenocortical carcinoma (ACC) is a rare tumor usually diagnosed at an advanced stage on invasion of or adherence to adjacent organs.
  • The estimated 3-year disease-free and overall survivals for stage III were 20% and 40%, respectively, with a median follow-up of 32 months (range, 11-58).
  • The mean disease-free survival was 21.0 +/- 9.0 months (95% CI: 3.3-38.7).
  • The 3-year disease-free and overall survivals for stage III and IV were 14.3% and 28.6%, respectively.
  • The mean disease-free survival time was 18.6 +/- 6.7 months (95% CI: 5.4-31.8).
  • [MeSH-major] Adrenal Cortex Neoplasms / surgery. Adrenocortical Carcinoma / surgery
  • [MeSH-minor] Adult. Aged. Female. Hepatectomy. Humans. Male. Middle Aged. Neoplasm Invasiveness. Neoplasm Staging. Survival Analysis. Vena Cava, Inferior / surgery

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  • (PMID = 17277000.001).
  • [ISSN] 1465-3621
  • [Journal-full-title] Japanese journal of clinical oncology
  • [ISO-abbreviation] Jpn. J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Japan
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94. Ragazzon B, Lefrançois-Martinez AM, Val P, Sahut-Barnola I, Tournaire C, Chambon C, Gachancard-Bouya JL, Begue RJ, Veyssière G, Martinez A: Adrenocorticotropin-dependent changes in SF-1/DAX-1 ratio influence steroidogenic genes expression in a novel model of glucocorticoid-producing adrenocortical cell lines derived from targeted tumorigenesis. Endocrinology; 2006 Apr;147(4):1805-18
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  • [Title] Adrenocorticotropin-dependent changes in SF-1/DAX-1 ratio influence steroidogenic genes expression in a novel model of glucocorticoid-producing adrenocortical cell lines derived from targeted tumorigenesis.
  • We established the first adrenocortical tumor cell lines with complete zona fasciculata (ZF) cell phenotype from tumors induced in transgenic mice by large T-antigen of simian virus 40 under the control of the aldose reductase-like akr1b7 gene promoter.
  • Adrenocortical tumor cell lines produced high amounts of corticosterone and were responsive to ACTH.
  • Taking advantage of these cells, we have examined the effect of ACTH on DAX-1 (dosage-sensitive sex reversal-adrenal hypoplasia congenita critical region on X-chromosome, gene 1) and SF-1 (steroidogenic factor 1), two transcription factors known to respectively repress and activate adrenocortical steroidogenesis by acting on common target genes.
  • [MeSH-major] Adrenal Cortex Neoplasms / metabolism. Adrenocorticotropic Hormone / pharmacology. Corticosterone / biosynthesis. DNA-Binding Proteins / analysis. Homeodomain Proteins / analysis. Receptors, Cytoplasmic and Nuclear / analysis. Transcription Factors / analysis. Zona Fasciculata / metabolism
  • [MeSH-minor] Adrenal Cortex. Aldehyde Reductase / genetics. Animals. Cell Line, Tumor. DAX-1 Orphan Nuclear Receptor. Gene Expression Regulation. Humans. Male. Mice. Mice, Transgenic. Promoter Regions, Genetic. RNA, Messenger / analysis. Steroidogenic Factor 1

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  • (PMID = 16439455.001).
  • [ISSN] 0013-7227
  • [Journal-full-title] Endocrinology
  • [ISO-abbreviation] Endocrinology
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / DAX-1 Orphan Nuclear Receptor; 0 / DNA-Binding Proteins; 0 / Homeodomain Proteins; 0 / NR0B1 protein, human; 0 / NR5A1 protein, human; 0 / Nr0b1 protein, mouse; 0 / RNA, Messenger; 0 / Receptors, Cytoplasmic and Nuclear; 0 / Steroidogenic Factor 1; 0 / Transcription Factors; 0 / steroidogenic factor 1, mouse; 9002-60-2 / Adrenocorticotropic Hormone; EC 1.1.1.21 / Akr1b7 protein, mouse; EC 1.1.1.21 / Aldehyde Reductase; W980KJ009P / Corticosterone
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95. Hishiki T, Kazukawa I, Saito T, Terui K, Mitsunaga T, Nakata M, Matsuura G, Minagawa M, Kohno Y, Yoshida H: Diagnosis of adrenocortical tumor in a neonate by detection of elevated blood 17-hydroxyprogesterone measured as a routine neonatal screening for congenital adrenal hyperplasia: a case report. J Pediatr Surg; 2008 Oct;43(10):e19-22
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  • [Title] Diagnosis of adrenocortical tumor in a neonate by detection of elevated blood 17-hydroxyprogesterone measured as a routine neonatal screening for congenital adrenal hyperplasia: a case report.
  • We report herein a case of prenatally detected neonatal adrenocortical tumor (ACT).
  • Computed tomography and ultrasonography after birth revealed a round solid tumor 40 mm in diameter in the right suprarenal area.
  • The precise diagnosis of ACT was unexpectedly obtained based on results from the Japanese neonatal mass screening program.
  • Blood 17-hydroxyprogesterone is routinely measured as a part of this program for early detection of congenital adrenal hyperplasia in Japan.
  • Abnormally elevated level of 17-hydroxyprogesterone was reported in the patient and, thus, led to the diagnosis of ACT.
  • Adrenocortical tumors are extremely rare in childhood, particularly in the neonatal period.
  • Some of these tumors secrete abnormally high levels of cortisol, suppressing function of the contralateral adrenal gland and, thus, leading to life-threatening postoperative adrenal insufficiency.
  • Adrenocortical tumor should always be considered among the differential diagnoses for neonatal suprarenal mass because precise diagnosis will enable the physician to develop appropriate treatment strategies.
  • [MeSH-major] 17-alpha-Hydroxyprogesterone / blood. Adrenal Cortex Neoplasms / diagnosis. Adrenal Hyperplasia, Congenital / diagnosis. Carcinoma / diagnosis. Fetal Blood / chemistry. Neonatal Screening
  • [MeSH-minor] Adrenal Insufficiency / prevention & control. Adrenalectomy. Aldosterone / blood. Dehydroepiandrosterone Sulfate / blood. Early Diagnosis. Female. Humans. Hydrocortisone / administration & dosage. Hydrocortisone / therapeutic use. Infant, Newborn. Postoperative Complications / prevention & control. Premedication. Testosterone / blood. Tomography, X-Ray Computed. Ultrasonography, Prenatal


96. Rodriguez-Galindo C, Figueiredo BC, Zambetti GP, Ribeiro RC: Biology, clinical characteristics, and management of adrenocortical tumors in children. Pediatr Blood Cancer; 2005 Sep;45(3):265-73
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  • [Title] Biology, clinical characteristics, and management of adrenocortical tumors in children.
  • Childhood adrenocortical tumors (ACT) are very aggressive endocrine neoplasms whose incidence is quite low.
  • In recent years, however, new information has been derived from the International Pediatric Adrenocortical Tumor Registry (IPACTR), and new clues to its pathogenesis have emerged.
  • Two-thirds of patients have resectable tumors.
  • Cisplatin-based chemotherapy with mitotane is indicated for unresectable or metastatic disease, although its impact on overall outcome is slight.
  • In childhood ACT, age, tumor size, and tumor resectability are the most important prognostic indicators.
  • Outcome is stage-dependent; patients with small, resectable tumors have survival rates in excess of 80%, whereas the outcome for patients with unresectable disease is dismal.
  • Patients with large, resectable tumors have an intermediate outcome.
  • [MeSH-major] Adrenal Cortex Neoplasms
  • [MeSH-minor] Adolescent. Antineoplastic Agents, Hormonal / therapeutic use. Child. Child, Preschool. Genes, p53 / genetics. Humans. Infant. Mitotane / therapeutic use. Mutation. Neoplasm Staging. Research. Survival Rate

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  • [Copyright] (c) 2005 Wiley-Liss, Inc.
  • (PMID = 15747338.001).
  • [ISSN] 1545-5009
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA 21765; United States / NCI NIH HHS / CA / CA 71907
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Hormonal; 78E4J5IB5J / Mitotane
  • [Number-of-references] 83
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97. Gjerstorff MF, Harkness L, Kassem M, Frandsen U, Nielsen O, Lutterodt M, Møllgård K, Ditzel HJ: Distinct GAGE and MAGE-A expression during early human development indicate specific roles in lineage differentiation. Hum Reprod; 2008 Oct;23(10):2194-201
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  • In addition, GAGE family members were expressed in the peripheral adrenal cortex of 6-9-week-old embryos and fetuses, which specifically correlated with massive cellular proliferation and establishment of the definitive and fetal zones.
  • [MeSH-major] Antigens, Neoplasm / physiology. Cell Differentiation. Cell Lineage. Neoplasm Proteins / physiology
  • [MeSH-minor] Adrenal Cortex / embryology. Adrenal Cortex / metabolism. Cell Movement. Central Nervous System / embryology. Central Nervous System / metabolism. Ectoderm / embryology. Ectoderm / metabolism. Embryonic Development. Embryonic Stem Cells / metabolism. Female. Fetal Development. Fetus / metabolism. Germ Cells / metabolism. Humans. Immunohistochemistry. Male. Melanoma-Specific Antigens. Neural Plate / embryology. Neural Plate / metabolism. Reverse Transcriptase Polymerase Chain Reaction. Teratoma / metabolism

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  • (PMID = 18611917.001).
  • [ISSN] 1460-2350
  • [Journal-full-title] Human reproduction (Oxford, England)
  • [ISO-abbreviation] Hum. Reprod.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antigens, Neoplasm; 0 / Melanoma-Specific Antigens; 0 / Neoplasm Proteins
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98. Cotesta D, Petramala L, Serra V, Giustini S, Divona L, Calvieri S, De Toma G, Ciardi A, Corsi A, Massa R, Reale MG, Letizia C: Pheochromocytoma associated with adrenocortical tumor in the same gland. Two case reports and literature review. Minerva Endocrinol; 2006 Jun;31(2):183-9
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  • [Title] Pheochromocytoma associated with adrenocortical tumor in the same gland. Two case reports and literature review.
  • Pheochromocytomas are catecholamine-producing neuroendocrine tumours arising from chromaffin cells of the adrenal medulla or extra-adrenal paraganglionic system that show 2 distinctive features, rarity and clinical variability.
  • Pheochromocytoma occasionally is associated with pathological lesions of the adrenal cortex.
  • We present 2 cases of patients referred to our hospital with a finding of clinical suspected pheochromocytoma.
  • The diagnosis of pheochromocytoma was confirmed in both cases with laboratory analysis and the lesion was achieved by employing 3 imaging techniques: computed tomography (CT), magnetic resonance imaging (MRI) and scintigraphy with (123)I-metaiodobenzilguanidine (MIBG).
  • The patients underwent adrenalectomy and in the same adrenal gland we found a pheochromocytoma associated with a nonfunctioning cortical adenoma.
  • [MeSH-major] Adrenal Cortex Neoplasms / diagnosis. Adrenal Gland Neoplasms / diagnosis. Adrenocortical Adenoma / diagnosis. Neoplasms, Multiple Primary / diagnosis. Pheochromocytoma / diagnosis
  • [MeSH-minor] 3-Iodobenzylguanidine. Adrenalectomy. Adult. Humans. Male. Middle Aged. Neurofibromatosis 1 / diagnosis. Radiopharmaceuticals. Treatment Outcome

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  • (PMID = 16682942.001).
  • [ISSN] 0391-1977
  • [Journal-full-title] Minerva endocrinologica
  • [ISO-abbreviation] Minerva Endocrinol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; 35MRW7B4AD / 3-Iodobenzylguanidine
  • [Number-of-references] 41
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99. Szekeres M, Turu G, Orient A, Szalai B, Süpeki K, Cserzo M, Várnai P, Hunyady L: Mechanisms of angiotensin II-mediated regulation of aldosterone synthase expression in H295R human adrenocortical and rat adrenal glomerulosa cells. Mol Cell Endocrinol; 2009 Apr 29;302(2):244-53
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  • [Title] Mechanisms of angiotensin II-mediated regulation of aldosterone synthase expression in H295R human adrenocortical and rat adrenal glomerulosa cells.
  • In adrenal zona glomerulosa cells angiotensin II (Ang II) is a key regulator of steroidogenesis.
  • Our purpose was to compare the mechanisms of Ang II-induced changes in the expression level of early transcription factors NR4A1 (NGFIB) and NR4A2 (Nurr1) genes, and the CYP11B2 gene encoding aldosterone synthase in H295R human adrenocortical tumor cells and in primary rat adrenal glomerulosa cells.
  • Real-time PCR studies have demonstrated that Ang II increased the expression levels of NR4A1 and NR4A2 in H295R cells within 1 h after stimulation, which persisted up to 6 h; whereas in rat adrenal glomerulosa cells the kinetics of the expression of these genes were more rapid and transient.
  • Studies using MEK inhibitor (PD98059, 20 microM), protein kinase C inhibitor (BIM1, 3 microM) and calmodulin kinase (CAMK) inhibitor (KN93, 10 microM) revealed that in rat adrenal glomerulosa cells CAMK-mediated mechanisms play a predominant role in the regulation of CYP11B2.
  • These data suggest that the previously reported CAMK-mediated stimulation of early transcription factors NGFIB and Nurr1 has a predominant role in Ang II-induced CYP11B2 activation in rat adrenal glomerulosa cells, whereas in H295R cells ERK activation and G protein-independent mechanisms also contribute to this process.
  • [MeSH-major] Adrenal Cortex / cytology. Angiotensin II / pharmacology. Cytochrome P-450 CYP11B2 / genetics. Gene Expression Regulation / drug effects. Zona Glomerulosa / cytology

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  • (PMID = 19418629.001).
  • [ISSN] 1872-8057
  • [Journal-full-title] Molecular and cellular endocrinology
  • [ISO-abbreviation] Mol. Cell. Endocrinol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Ireland
  • [Chemical-registry-number] 0 / Transcription Factors; 11128-99-7 / Angiotensin II; EC 1.14.15.4 / Cytochrome P-450 CYP11B2; EC 2.7.11.17 / Calcium-Calmodulin-Dependent Protein Kinases; EC 2.7.11.24 / Extracellular Signal-Regulated MAP Kinases
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100. Rodriguez FJ, Scheithauer BW, Erickson LA, Jenkins RB, Giannini C: Ectopic low-grade adrenocortical carcinoma in the spinal region: immunohistochemical and molecular cytogenetic study of a pediatric case. Am J Surg Pathol; 2009 Jan;33(1):142-8
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  • [Title] Ectopic low-grade adrenocortical carcinoma in the spinal region: immunohistochemical and molecular cytogenetic study of a pediatric case.
  • Ectopic adrenocortical neoplasms arising in the nervous system are very rare.
  • We encountered an intradural, extramedullary case of an adrenocortical neoplasm of indeterminate malignant potential affecting a spinal nerve root in the distal lumbar region of a 5-month-old girl.
  • The tumor in both resections had increased mitotic activity (5/10 high power fields) and MIB-1 labeling indices of 23% and 33% at initial resection and recurrence, respectively.
  • Both tumors demonstrated gains of chromosomes 5 and 12 by interphase cytogenetics, whereas insulin growth factor 2 was identified in the recurrent tumor by immunohistochemistry.
  • This report demonstrates that ectopic adrenocortical tumors in the nervous system may exhibit clinicopathologic and cytogenetic features suggestive of adrenocortical carcinoma.

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  • (PMID = 18941403.001).
  • [ISSN] 1532-0979
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] ENG
  • [Grant] United States / NINDS NIH HHS / NS / T32 NS007494; United States / NINDS NIH HHS / NS / T32 NS07494-04
  • [Publication-type] Case Reports; Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] United States
  • [Other-IDs] NLM/ NIHMS396174; NLM/ PMC3427599
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