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1. Faria JF, Goldman SM, Szejnfeld J, Melo H, Kater C, Kenney P, Huayllas MP, Demarchi G, Francisco VV, Andreoni C, Srougi M, Ortiz V, Abdalla N: Adrenal masses: characterization with in vivo proton MR spectroscopy--initial experience. Radiology; 2007 Dec;245(3):788-97
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Adrenal masses: characterization with in vivo proton MR spectroscopy--initial experience.
  • PURPOSE: To prospectively determine the accuracy of in vivo proton ((1)H) magnetic resonance (MR) spectroscopy in distinguishing adrenal adenomas, pheochromocytomas, adrenocortical carcinomas, and metastases, with histologic or computed tomographic findings and follow-up data as the reference standards.
  • Sixty consecutive patients (24 male and 36 female patients; mean age, 53 years) harboring adrenal tumors larger than 2 cm in diameter (mean diameter, 4.6 cm +/- 3.4 [standard deviation]) entered the study and were examined with a 1.5-T MR imaging system and point-resolved multivoxel (1)H MR spectroscopy.
  • Thirty-eight patients had adenomas; 10, pheochromocytomas; five, carcinomas; and seven, metastases.
  • Metabolite ratios (choline-creatine, choline-lipid, lipid-creatine, and 4.0-4.3 ppm/creatine) and cutoff values (obtained by using receiver operating characteristic analyses) were obtained and compared for each type of adrenal mass, which was identified previously on the basis of clinical, hormonal, and pathologic evidence.
  • RESULTS: Cutoff values of 1.20 for the choline-creatine ratio (92% sensitivity, 96% specificity; P < .01), 0.38 for the choline-lipid ratio (92% sensitivity, 90% specificity; P < .01), and 2.10 for the lipid-creatine ratio (45% sensitivity, 100% specificity) enabled adenomas and pheochromocytomas to be distinguished from carcinomas and metastases.
  • A 4.0-4.3 ppm/creatine ratio greater than 1.50 enabled distinction of pheochromocytomas and carcinomas from adenomas and metastases (87% sensitivity, 98% specificity; P < .01).
  • CONCLUSION: (1)H MR spectroscopy can be used to characterize adrenal masses on the basis of spectral findings for benign adenomas, carcinomas, pheochromocytomas, and metastases.
  • [MeSH-major] Adenoma / diagnosis. Adrenal Gland Neoplasms / diagnosis. Adrenocortical Carcinoma / diagnosis. Magnetic Resonance Spectroscopy. Pheochromocytoma / diagnosis
  • [MeSH-minor] Adult. Diagnosis, Differential. Female. Humans. Male. Middle Aged. Prospective Studies. Reproducibility of Results

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  • [Copyright] (c) RSNA, 2007.
  • [CommentIn] Radiology. 2009 Mar;250(3):955-6; author reply 955-6; discussion 956 [19244058.001]
  • (PMID = 18024453.001).
  • [ISSN] 1527-1315
  • [Journal-full-title] Radiology
  • [ISO-abbreviation] Radiology
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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2. Haase M, Schott M, Bornstein SR, Malendowicz LK, Scherbaum WA, Willenberg HS: CITED2 is expressed in human adrenocortical cells and regulated by basic fibroblast growth factor. J Endocrinol; 2007 Feb;192(2):459-65
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  • [Title] CITED2 is expressed in human adrenocortical cells and regulated by basic fibroblast growth factor.
  • CITED2 gene deletion in mice leads to adrenal agenesis.
  • Therefore, we analyzed CITED2, a CBP/p300 interacting transactivator with transforming activity, in the human adrenal gland.
  • In this study, we examined CITED2 expression in human embryonic and adult adrenal glands as well as adrenocortical carcinomas.
  • As ACTH and basic fibroblast growth factor (bFGF) are connected to the physiology and growth of adrenocortical cells we studied the regulation of CITED2 by these factors in the NCI-H295R adrenocortical carcinoma cell line.
  • We found CITED2 expression in the adult adrenal cortex as well in adrenocortical carcinomas.
  • At an early stage of human adrenal organogenesis CITED2 could be located to the definitive zone of the developing adrenal gland using immunohistochemistry.
  • CITED2 is expressed in embryonic and adult human adrenal glands as well as in adrenocortical cancer.
  • This suggests a novel role for CITED2 in human adrenal growth and possibly in adrenal tumorigenesis.
  • [MeSH-major] Adrenal Cortex / metabolism. DNA-Binding Proteins / analysis. Fibroblast Growth Factor 2 / pharmacology. Gene Expression Regulation. Repressor Proteins / analysis. Trans-Activators / analysis
  • [MeSH-minor] Adrenocortical Carcinoma. Adrenocorticotropic Hormone / metabolism. Adrenocorticotropic Hormone / pharmacology. Adult. Cell Line, Tumor. Cells, Cultured. Colforsin / pharmacology. Dose-Response Relationship, Drug. Embryonic Development. Humans. Immunohistochemistry / methods. Microscopy, Fluorescence. RNA, Messenger / analysis. Reverse Transcriptase Polymerase Chain Reaction / methods. Transfection / methods

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  • (PMID = 17283246.001).
  • [ISSN] 0022-0795
  • [Journal-full-title] The Journal of endocrinology
  • [ISO-abbreviation] J. Endocrinol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / CITED2 protein, human; 0 / DNA-Binding Proteins; 0 / RNA, Messenger; 0 / Repressor Proteins; 0 / Trans-Activators; 103107-01-3 / Fibroblast Growth Factor 2; 1F7A44V6OU / Colforsin; 9002-60-2 / Adrenocorticotropic Hormone
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3. Yüksel M, Ozyurtkan MO, Bostanci K, Ahiskali R, Kodalli N: Acute exacerbation of interstitial fibrosis after pulmonary resection. Ann Thorac Surg; 2006 Jul;82(1):336-8
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  • Idiopathic pulmonary fibrosis is a chronic diffuse lung disease of unknown cause, and a risk factor for increasing morbidity and mortality after lung resection.
  • Acute exacerbation of idiopathic pulmonary fibrosis after lung surgery for lung cancer is rare.
  • We report two cases of acute exacerbation of idiopathic pulmonary fibrosis after resection for lung cancer.
  • [MeSH-minor] Acute Disease. Adenocarcinoma / complications. Adenocarcinoma / surgery. Adrenal Cortex Hormones / therapeutic use. Aged, 80 and over. Carcinoma, Squamous Cell / complications. Carcinoma, Squamous Cell / surgery. Fatal Outcome. Humans. Lung Neoplasms / surgery. Lymph Node Excision. Male. Middle Aged. Oxygen / therapeutic use. Respiratory Insufficiency / etiology. Smoking

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  • (PMID = 16798250.001).
  • [ISSN] 1552-6259
  • [Journal-full-title] The Annals of thoracic surgery
  • [ISO-abbreviation] Ann. Thorac. Surg.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / Adrenal Cortex Hormones; S88TT14065 / Oxygen
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4. White DA, Camus P, Endo M, Escudier B, Calvo E, Akaza H, Uemura H, Kpamegan E, Kay A, Robson M, Ravaud A, Motzer RJ: Noninfectious pneumonitis after everolimus therapy for advanced renal cell carcinoma. Am J Respir Crit Care Med; 2010 Aug 1;182(3):396-403
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Noninfectious pneumonitis after everolimus therapy for advanced renal cell carcinoma.
  • OBJECTIVES: To assess the incidence, radiographic patterns, management, and outcome of pneumonitis in patients with advanced renal cell carcinoma receiving everolimus.
  • Dedicated radiological review of available serial radiographic studies (245 patients receiving everolimus and 132 receiving placebo) found a higher percentage of new radiographic findings even in patients without a diagnosis of clinical pneumonitis who were receiving everolimus versus placebo (38.9 vs. 15.2%).
  • [MeSH-major] Carcinoma, Renal Cell / drug therapy. Immunosuppressive Agents / adverse effects. Kidney Neoplasms / drug therapy. Pneumonia / chemically induced. Sirolimus / analogs & derivatives
  • [MeSH-minor] Adrenal Cortex Hormones / therapeutic use. Adult. Aged. Aged, 80 and over. Everolimus. Female. Humans. Male. Middle Aged. Respiratory Function Tests. Severity of Illness Index. Tomography, X-Ray Computed


5. Fassnacht M, Wittekind C, Allolio B: [Current TNM classification systems for adrenocortical carcinoma]. Pathologe; 2010 Sep;31(5):374-8
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  • [Title] [Current TNM classification systems for adrenocortical carcinoma].
  • Adrenocortical carcinoma (ACC) is a rare malignancy and often difficult to diagnose.
  • Therefore, the European Network for the Study of Adrenal Tumours (ENSAT) developed a revised staging system, the superiority of which was recently confirmed in an independent American cohort.
  • [MeSH-major] Adrenal Cortex Neoplasms / pathology. Neoplasm Staging / methods
  • [MeSH-minor] Adrenal Cortex / pathology. Cohort Studies. Disease Progression. Humans. Lymphatic Metastasis / pathology. Neoplasm Invasiveness / pathology. Neoplastic Cells, Circulating. Prognosis. Registries

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  • (PMID = 20703482.001).
  • [ISSN] 1432-1963
  • [Journal-full-title] Der Pathologe
  • [ISO-abbreviation] Pathologe
  • [Language] ger
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Germany
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6. Adam P, Hahner S, Hartmann M, Heinrich B, Quinkler M, Willenberg HS, Saeger W, Sbiera S, Schmull S, Voelker HU, Ströbel P, Allolio B, Fassnacht M: Epidermal growth factor receptor in adrenocortical tumors: analysis of gene sequence, protein expression and correlation with clinical outcome. Mod Pathol; 2010 Dec;23(12):1596-604
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  • [Title] Epidermal growth factor receptor in adrenocortical tumors: analysis of gene sequence, protein expression and correlation with clinical outcome.
  • Adrenocortical carcinoma is a rare but highly malignant neoplasm with still limited treatment options.
  • Epidermal growth factor receptor (EGFR) has been shown to be overexpressed in many solid tumors, but its expression in adrenocortical carcinoma has been studied only in a limited number of cases.
  • Therefore, we analyzed the expression of EGFR in 169 adrenocortical carcinoma samples and compared it with 31 adrenocortical adenomas.
  • Additionally, in 30 cases of adrenocortical carcinoma, exons 18-21 of the EGFR gene were cloned and sequenced.
  • EGFR expression was found in 128 of 169 adrenocortical carcinoma samples (76%), and in 60 of these samples (=36%) strong membrane staining was detected.
  • In contrast, only 1 out of 31 adrenocortical adenomas weakly expressed the EGFR (3%).
  • In summary, EGFR was overexpressed in more than three-quarters of adrenocortical carcinoma cases of this series.
  • As EGFR is hardly expressed in adrenocortical adenomas, our results suggest that its expression in adrenocortical tumors indicates a malignant phenotype, which may be used in the differential diagnosis between adrenocortical adenomas and carcinomas.
  • [MeSH-major] Adrenal Cortex Neoplasms / metabolism. Adrenal Cortex Neoplasms / pathology. Adrenocortical Adenoma / metabolism. Adrenocortical Adenoma / pathology. Receptor, Epidermal Growth Factor / biosynthesis

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  • (PMID = 20693985.001).
  • [ISSN] 1530-0285
  • [Journal-full-title] Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
  • [ISO-abbreviation] Mod. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; EC 2.7.10.1 / Receptor, Epidermal Growth Factor
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7. 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.
  • 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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8. Mundt P, Mochmann HC, Ebhardt H, Zeitz M, Duchmann R, Pauschinger M: Pulmonary fibrosis after chemotherapy with oxaliplatin and 5-fluorouracil for colorectal cancer. Oncology; 2007;73(3-4):270-2
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  • [Title] Pulmonary fibrosis after chemotherapy with oxaliplatin and 5-fluorouracil for colorectal cancer.
  • BACKGROUND: Chemotherapy with oxaliplatin and 5-fluorouracil (5-FU)/folinic acid is the standard first-line therapy of metastatic colorectal carcinoma and has shown activity in several other malignancies.
  • CASE REPORT: A 66-year-old male with metastatic rectal adenocarcinoma treated with 12 cycles of oxaliplatin and 5-FU/folinic acid developed bilateral pulmonary infiltrates and respiratory failure.
  • [MeSH-major] Adenocarcinoma / drug therapy. Antineoplastic Combined Chemotherapy Protocols / adverse effects. Pulmonary Fibrosis / chemically induced. Rectal Neoplasms / drug therapy
  • [MeSH-minor] Adrenal Cortex Hormones / therapeutic use. Aged. Fluorouracil / administration & dosage. Humans. Male. Organoplatinum Compounds / administration & dosage. Treatment Outcome

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  • [Copyright] (c) 2008 S. Karger AG, Basel
  • (PMID = 18424892.001).
  • [ISSN] 1423-0232
  • [Journal-full-title] Oncology
  • [ISO-abbreviation] Oncology
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / Adrenal Cortex Hormones; 0 / Organoplatinum Compounds; 04ZR38536J / oxaliplatin; U3P01618RT / Fluorouracil
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9. Chou SH, Tseleni-Balafouta S, Moon HS, Chamberland JP, Liu X, Kavantzas N, Mantzoros CS: Adiponectin receptor expression in human malignant tissues. Horm Cancer; 2010 Jun;1(3):136-45
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  • [Title] Adiponectin receptor expression in human malignant tissues.
  • We used immunohistochemistry to assess expression of adiponectin receptors in archival specimens of renal cell carcinoma (n = 64), hepatocellular carcinoma (n = 123), melanoma (n = 20), cholangiocarcinoma (n = 20), transitional cell carcinoma of the bladder (n = 24), ovarian epithelial carcinoma (n = 63), cervical squamous cell carcinoma (n = 49), and adrenocortical carcinoma (n = 48).
  • To compare expression in malignant versus nonmalignant tissues, we also studied AdipoR1 and AdipoR2 expression in pairs of renal cell carcinoma and adjacent healthy kidney tissue specimens by immunohistochemistry.
  • We also studied mRNA expression in 45 specimens of renal cell carcinoma by real-time polymerase chain reaction.
  • Finally, we utilized Western blotting to confirm the presence of adiponectin receptors and subsequently studied cell signaling pathways of adiponectin in the renal cancer cell line 786-O.
  • Of the specimens of renal cell carcinoma, which is strongly associated with obesity, 93.8% expressed AdipoR1 compared to 44.9% of the specimens of cervical cell carcinoma, which is not associated with obesity (p < 0.001).
  • There was no difference in the expression of adiponectin receptors or their mRNA between malignant and benign kidney tissue specimens.
  • Finally, Western blotting confirmed the presence of AdipoR1 in the renal cancer cell line 786-O, and adiponectin activates in vitro several signaling pathways in this cell line.
  • [MeSH-minor] Adult. Aged. Blotting, Western. Carcinoma, Renal Cell / metabolism. Female. Humans. Immunohistochemistry. Kidney Neoplasms / metabolism. Male. Middle Aged. Obesity / complications. Obesity / metabolism. RNA, Messenger / analysis. Real-Time Polymerase Chain Reaction

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  • (PMID = 21761356.001).
  • [ISSN] 1868-8500
  • [Journal-full-title] Hormones & cancer
  • [ISO-abbreviation] Horm Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / RNA, Messenger; 0 / Receptors, Adiponectin
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10. Cheng MF, Wu YW, Tzen KY, Yen RF: F-18 FDG PET/CT illustrating tumor invasion in the IVC from adrenocortical carcinoma. Clin Nucl Med; 2007 Nov;32(11):891-2
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  • [Title] F-18 FDG PET/CT illustrating tumor invasion in the IVC from adrenocortical carcinoma.
  • [MeSH-major] Adrenal Cortex Neoplasms / pathology. Adrenocortical Carcinoma / pathology. Fluorodeoxyglucose F18. Positron-Emission Tomography. Tomography, X-Ray Computed. Vena Cava, Inferior / pathology. Vena Cava, Inferior / radionuclide imaging

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  • (PMID = 18075433.001).
  • [ISSN] 0363-9762
  • [Journal-full-title] Clinical nuclear medicine
  • [ISO-abbreviation] Clin Nucl Med
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0Z5B2CJX4D / Fluorodeoxyglucose F18
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11. Meyer A, Behrend M: State of the art management of adrenal masses--"how to do it?". Eur J Med Res; 2006 Sep 29;11(9):397-404
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  • [Title] State of the art management of adrenal masses--"how to do it?".
  • By the frequent use of computed tomography or ultrasound the detection of incidentally found adrenal tumours has become a common problem.
  • Most incidentally-found adrenal tumours are benign non-functioning cortical adenomas.
  • But benign functioning tumours producing aldosterone, cortisol or catecholamines, adrenocortical carcinoma or adrenal gland metastasis can also be found.
  • Surgical therapy is always indicated in case of hormonal overproduction or in case of suspected adrenocortical carcinoma; in all other cases the correct and adequate therapeutic approach is still under debate and a controversial topic of discussion.
  • This review deals with the different forms of adrenal tumours regarding the optimal diagnostic and therapeutic approach to give physicians an easy-to-follow guideline.
  • [MeSH-major] Adrenal Gland Neoplasms / therapy

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  • (PMID = 17101464.001).
  • [ISSN] 0949-2321
  • [Journal-full-title] European journal of medical research
  • [ISO-abbreviation] Eur. J. Med. Res.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Germany
  • [Number-of-references] 50
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12. Bielinska M, Parviainen H, Kiiveri S, Heikinheimo M, Wilson DB: Review paper: origin and molecular pathology of adrenocortical neoplasms. Vet Pathol; 2009 Mar;46(2):194-210
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  • [Title] Review paper: origin and molecular pathology of adrenocortical neoplasms.
  • Neoplastic adrenocortical lesions are common in humans and several species of domestic animals.
  • Although there are unanswered questions about the origin and evolution of adrenocortical neoplasms, analysis of human tumor specimens and animal models indicates that adrenocortical tumorigenesis involves both genetic and epigenetic alterations.
  • Epigenetic changes serve to expand the size of the uncommitted adrenal progenitor population, modulate their phenotypic plasticity (i.e., responsiveness to extracellular signals), and increase the likelihood of subsequent genetic alterations.
  • Analyses of heritable and spontaneous types of human adrenocortical tumors documented alterations in either cell surface receptors or their downstream effectors that impact neoplastic transformation.
  • Many of the mutations associated with benign human adrenocortical tumors result in dysregulated cyclic adenosine monophosphate signaling, whereas key factors and/or signaling pathways associated with adrenocortical carcinomas include dysregulated expression of the IGF2 gene cluster, activation of the Wnt/beta-catenin pathway, and inactivation of the p53 tumor suppressor.
  • A better understanding of the factors and signaling pathways involved in adrenal tumorigenesis is necessary to develop targeted pharmacologic and genetic therapies.

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  • (PMID = 19261630.001).
  • [ISSN] 0300-9858
  • [Journal-full-title] Veterinary pathology
  • [ISO-abbreviation] Vet. Pathol.
  • [Language] ENG
  • [Grant] United States / NIDDK NIH HHS / DK / DK075618-02; United States / NIDDK NIH HHS / DK / R01 DK075618-02; United States / NIDDK NIH HHS / DK / DK52574; United States / NIDDK NIH HHS / DK / DK075618; United States / NIDDK NIH HHS / DK / P30 DK052574; United States / NIDDK NIH HHS / DK / R01 DK075618; United States / NIDDK NIH HHS / DK / P30 DK052574-10
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Number-of-references] 165
  • [Other-IDs] NLM/ NIHMS85359; NLM/ PMC2811968
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13. Meyer A, Behrend M: 32-year survival with metastatic adrenal cortical carcinoma--update of a case report. Anticancer Res; 2007 Mar-Apr;27(2):1045-6
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  • [Title] 32-year survival with metastatic adrenal cortical carcinoma--update of a case report.
  • Adrenal cortical carcinoma (ACC) is a rare and highly malignant tumour with up to 70% of the patients diagnosed at an advanced clinical stage and up to 40% presenting with metastasis.
  • In 2004, we reported a case of a 62-year-old woman with a non-functional ACC of the left adrenal gland (T2 N0 M0, classified as stage II) who survived the disease for 28 years with 3 operations for metastases of the contralateral adrenal gland and 4 operations for metastases of the lung.
  • We can now give an update of a further four years of survival and one additional operation.
  • The case again emphasises the need for thorough radiographic examination and follow-up for the possibility of a repeated re-operation whenever possible to provide long-term survival over decades.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Lung Neoplasms / secondary

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  • (PMID = 17465241.001).
  • [ISSN] 0250-7005
  • [Journal-full-title] Anticancer research
  • [ISO-abbreviation] Anticancer Res.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Greece
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14. Kaneko T, Kojima Y, Umemoto Y, Sasaki S, Hayashi Y, Kohri K: Usefulness of transcription factors Ad4BP/SF-1 and DAX-1 as immunohistologic markers for diagnosis of advanced adrenocortical carcinoma. Horm Res; 2008;70(5):294-9
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  • [Title] Usefulness of transcription factors Ad4BP/SF-1 and DAX-1 as immunohistologic markers for diagnosis of advanced adrenocortical carcinoma.
  • BACKGROUND: We examined the expressions of Ad4BP/SF-1 and DAX-1 in primary and metastatic lesions of advanced adrenocortical carcinoma with normal hormonal findings by immunohistochemistry and discussed their usefulness as immunohistochemical markers for diagnosis.
  • Ad4BP/SF-1 and DAX-1 underwent immunohistochemical study using needle biopsy specimens from the retroperitoneal tumor or metastatic tumor of these patients to diagnose primary adrenocortical carcinoma.
  • Ad4BP/SF-1 immunoreactivity was observed in the carcinoma cells of 2 patients, but not in the others, whereas DAX-1 immunoreactivity was observed in the carcinoma cells of all 4 patients.
  • CONCLUSION: The expressions of Ad4BP/SF-1 and DAX-1 are considered essential to maintain the biological characteristics of adrenocortical cells even without abnormal hormonal findings or even after malignant transformation and metastasis.
  • These markers are useful to distinguish other retroperitoneal tumors, especially in patients with bulky tumors, and to diagnose the metastatic site of origin as adrenocortical carcinoma in patients clinically presenting with widespread metastasis.
  • [MeSH-major] Adrenal Cortex Neoplasms / diagnosis. Adrenocortical Carcinoma / diagnosis. Biomarkers, Tumor / analysis. DNA-Binding Proteins / analysis. Receptors, Retinoic Acid / analysis. Repressor Proteins / analysis. Steroidogenic Factor 1 / analysis
  • [MeSH-minor] DAX-1 Orphan Nuclear Receptor. Humans. Immunohistochemistry. Middle Aged. Retroperitoneal Neoplasms / diagnosis

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  • [Copyright] Copyright 2008 S. Karger AG, Basel.
  • (PMID = 18824868.001).
  • [ISSN] 1423-0046
  • [Journal-full-title] Hormone research
  • [ISO-abbreviation] Horm. Res.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / DAX-1 Orphan Nuclear Receptor; 0 / DNA-Binding Proteins; 0 / NR0B1 protein, human; 0 / NR5A1 protein, human; 0 / Receptors, Retinoic Acid; 0 / Repressor Proteins; 0 / Steroidogenic Factor 1
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15. Kinoshita Y, Kuratsukuri K, Landas S, Imaida K, Rovito PM Jr, Wang CY, Haas GP: Expression of prostate-specific membrane antigen in normal and malignant human tissues. World J Surg; 2006 Apr;30(4):628-36
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  • [Title] Expression of prostate-specific membrane antigen in normal and malignant human tissues.
  • BACKGROUND: Prostate-specific membrane antigen (PSMA) is upregulated in androgen-dependent prostate carcinoma and it has been targeted for immunotherapy and diagnosis of this cancer.
  • The objective of this study is to investigate its expression in normal and malignant human tissues.
  • RESULTS: Prostate-specific membrane antigen was detected in the epithelium of prostate, urinary bladder, proximal tubules of kidney, liver, esophagus, stomach, small intestine, colon, breast, fallopian tubes and testicular seminiferous tubules, hippocampal neurons and astrocytes, ependyma, cortex and medulla of the adrenal gland, and ovary stroma.
  • It was also detected in neoplasms of the prostate, kidney, urinary bladder, stomach, small intestine, colon, lung, adrenal gland, and testis.
  • The broad distribution of PSMA may make it suitable for the diagnosis and therapy of a wide variety of tumors.

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  • (PMID = 16555021.001).
  • [ISSN] 0364-2313
  • [Journal-full-title] World journal of surgery
  • [ISO-abbreviation] World J Surg
  • [Language] eng
  • [Grant] United States / NIA NIH HHS / AG / 1R01AG21389-01
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, U.S. Gov't, Non-P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, Surface; 0 / Biomarkers, Tumor; EC 3.4.17.21 / Glutamate Carboxypeptidase II; EC 3.4.17.21 / glutamate carboxypeptidase II, human
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16. 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
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  • [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.
  • 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.
  • [MeSH-major] Adrenal Cortex Neoplasms / therapy. Carcinoma / therapy

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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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17. 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
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  • [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.
  • Over a 17 months time course, transgenic adrenals developed malignant characteristics such as uncontrolled neovascularization and loco-regional metastatic invasion.
  • 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


18. Terzolo M, Berruti A: Adjunctive treatment of adrenocortical carcinoma. Curr Opin Endocrinol Diabetes Obes; 2008 Jun;15(3):221-6
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  • [Title] Adjunctive treatment of adrenocortical carcinoma.
  • PURPOSE OF REVIEW: Description of the adjunctive treatment strategies in patients with adrenocortical carcinoma after complete surgical resection.
  • RECENT FINDINGS: A retrospective analysis showing that adjuvant mitotane may prolong recurrence-free survival in a large cohort of patients with radically resected adrenocortical carcinoma has recently been published.
  • SUMMARY: Radical surgical resection of adrenocortical carcinoma offers the best chance for prolonged recurrence-free survival; however, a significant number of patients without objective and biochemical evidence of residual tumor after surgery are destined to relapse.
  • Mitotane, an analogue of the insecticide dichlorodiphenyltrichloroethane, has been used for treatment of advanced adrenocortical carcinoma since the 1960s, but its use as an adjunctive postoperative measure has remained controversial.
  • The recent demonstration that mitotane treatment following macroscopically complete removal of adrenocortical carcinoma was associated with beneficial effects on outcome in a well designed, multicenter, international study should renew interest in adjuvant mitotane therapy.
  • [MeSH-major] Adrenal Cortex Neoplasms / drug therapy. Adrenocortical Carcinoma / drug therapy. Mitotane / adverse effects. Neoplasm Recurrence, Local / prevention & control
  • [MeSH-minor] Adrenalectomy. Antineoplastic Combined Chemotherapy Protocols / adverse effects. Chemotherapy, Adjuvant / adverse effects. Chemotherapy, Adjuvant / methods. Disease-Free Survival. Humans


19. Lindhe O, Skogseid B: Mitotane effects in a H295R xenograft model of adjuvant treatment of adrenocortical cancer. Horm Metab Res; 2010 Sep;42(10):725-30
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  • [Title] Mitotane effects in a H295R xenograft model of adjuvant treatment of adrenocortical cancer.
  • Adrenocortical cancer is one of the most aggressive endocrine malignancies.
  • Growth through the capsule or accidental release of cancer cells during surgery frequently results in metastatic disease.
  • We investigated the antitumoral effect of 2 adrenocorticolytic compounds, O, P'-DDD and MeSO2-DDE, in the adrenocortical cell line H295R both in vitro and as a xenograft model in vivo.
  • O, P'-DDD, MeSO2-DDE, or oil (control) was administered i. p., either simultaneously with cell injection at day 0 (mimicking adjuvant treatment), or at day 48 (established tumors).
  • We suggest that FLT may be a potential PET biomarker when assessing adrenocortical cancer treatment with O,P'-DDD.
  • [MeSH-major] Adjuvants, Pharmaceutic / therapeutic use. Adrenal Cortex Neoplasms / drug therapy. Mitotane / therapeutic use. Xenograft Model Antitumor Assays
  • [MeSH-minor] Animals. Cell Aggregation / drug effects. Cell Count. Cell Proliferation / drug effects. Female. Humans. Mice. Positron-Emission Tomography. Radioactive Tracers. Spheroids, Cellular / drug effects. Spheroids, Cellular / pathology. Time Factors. Tumor Burden / drug effects. Tumor Cells, Cultured

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  • (PMID = 20665429.001).
  • [ISSN] 1439-4286
  • [Journal-full-title] Hormone and metabolic research = Hormon- und Stoffwechselforschung = Hormones et métabolisme
  • [ISO-abbreviation] Horm. Metab. Res.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Adjuvants, Pharmaceutic; 0 / Radioactive Tracers; 78E4J5IB5J / Mitotane
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20. Haleblian GE, Wilson C, Haddad D, Albala DM: Adrenocortical carcinoma: role of laparoscopic surgery in treatment. Expert Rev Anticancer Ther; 2007 Sep;7(9):1295-300
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  • [Title] Adrenocortical carcinoma: role of laparoscopic surgery in treatment.
  • Adrenocortical carcinoma is a rare disorder with a prevalence of one case per 1.7 million people and a generally poor prognosis.
  • It accounts for 0.02% of all cancer cases and 0.2% of cancer deaths.
  • Within the past three decades, accurate diagnosis, precise radiologic localization, satisfactory preoperative medical management, appropriate anesthesia and refined surgical techniques have come together to render the surgical management of adrenal abnormalities a safe endeavor with predictable outcomes.
  • While there is a general agreement on the suitability of the laparoscopic approach for benign adrenal lesions, controversy remains regarding the use of laparoscopy for suspected adrenal malignancies.
  • This paper provides an overview of adrenal cancer and reviews the literature on laparoscopic adrenalectomy for cancer, including the operative techniques, indications and contraindications.
  • [MeSH-major] Adrenal Cortex Neoplasms / surgery. Adrenocortical Carcinoma / surgery. Laparoscopy / methods

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  • (PMID = 17892430.001).
  • [ISSN] 1744-8328
  • [Journal-full-title] Expert review of anticancer therapy
  • [ISO-abbreviation] Expert Rev Anticancer Ther
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Number-of-references] 28
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21. Shi Z, Henwood MJ, Bannerman P, Batista D, Horvath A, Guttenberg M, Stratakis CA, Grimberg A: Primary pigmented nodular adrenocortical disease reveals insulin-like growth factor binding protein-2 regulation by protein kinase A. Growth Horm IGF Res; 2007 Apr;17(2):113-21
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  • [Title] Primary pigmented nodular adrenocortical disease reveals insulin-like growth factor binding protein-2 regulation by protein kinase A.
  • OBJECTIVE: Primary pigmented nodular adrenocortical disease (PPNAD) can occur as an isolated trait or part of Carney complex, a familial lentiginosis-multiple endocrine neoplasia syndrome frequently caused by mutations in PRKAR1A, which encodes the 1alpha regulatory subunit of protein kinase A (PKA).
  • Because alterations in the insulin-like growth factor (IGF) axis, particularly IGF-II and IGF binding protein (IGFBP)-2 overexpression, have been implicated in sporadic adrenocortical tumors, we sought to examine the IGF axis in PPNAD.
  • NCI-H295R cells were used to study PKA and IGF axis signaling in adrenocortical cells in vitro.
  • RESULTS: IGFBP-2 mRNA level distinguished between the two genetic subtypes of this disease; increased IGFBP-2 expression in PRKAR1A mutation-positive PPNAD tissues was also confirmed by immunohistochemistry.
  • Moreover, PKA inhibitors increased IGFBP-2 expression in NCI-H295R adrenocortical cells, and anti-IGFBP-2 antibody reduced their proliferation.
  • CONCLUSIONS: IGFBP-2 expression is increased in PPNAD caused by PRKAR1A mutations, and in adrenocortical cancer cells.
  • This is the first evidence for PKA-dependent regulation of IGFBP-2 expression in adrenocortical cells.

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  • (PMID = 17280861.001).
  • [ISSN] 1096-6374
  • [Journal-full-title] Growth hormone & IGF research : official journal of the Growth Hormone Research Society and the International IGF Research Society
  • [ISO-abbreviation] Growth Horm. IGF Res.
  • [Language] ENG
  • [Databank-accession-numbers] OMIM/ 160980
  • [Grant] United States / NIDDK NIH HHS / DK / T32 DK63688; United States / NIDDK NIH HHS / DK / T32 DK063688; United States / NICHD NIH HHS / HD / Z01 HD000642-04; United States / NIDDK NIH HHS / DK / 5 K08 DK64352; United States / NICHD NIH HHS / HD / Z01 HD000642; United States / NIDDK NIH HHS / DK / K08 DK064352
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] Scotland
  • [Chemical-registry-number] 0 / Cyclic AMP-Dependent Protein Kinase RIalpha Subunit; 0 / Insulin-Like Growth Factor Binding Protein 2; 0 / PRKAR1A protein, human; 0 / Protein Kinase Inhibitors; 0 / RNA, Messenger; 67763-97-7 / Insulin-Like Growth Factor II; EC 2.7.11.11 / Cyclic AMP-Dependent Protein Kinases
  • [Other-IDs] NLM/ NIHMS22924; NLM/ PMC2577759
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22. Waldmann J, Bartsch DK, Kann PH, Fendrich V, Rothmund M, Langer P: Adrenal involvement in multiple endocrine neoplasia type 1: results of 7 years prospective screening. Langenbecks Arch Surg; 2007 Jul;392(4):437-43
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  • [Title] Adrenal involvement in multiple endocrine neoplasia type 1: results of 7 years prospective screening.
  • BACKGROUND: Adrenal tumors are a common manifestation of the multiple endocrine neoplasia type 1 (MEN-1) syndrome.
  • A genotype-phenotype correlation has been described as well as the development of adrenocortical carcinomas.
  • Adrenal glands have been screened by biochemical analysis and either by endoscopic ultrasound (EUS) or computed tomography (CT) or both.
  • Age at diagnosis of MEN-1, type of adrenal tumor, genotype, therapy, and clinical characteristics have been analyzed.
  • RESULTS: In 21 (55%) patients, adrenal involvement of the disease was detected.
  • Adrenal lesions were detected in average 6.9 years after the initial diagnosis of MEN-1.
  • Twelve patients had unilateral while nine had bilateral adrenal lesions.
  • EUS detected all adrenal tumors, whereas CT failed in seven cases.
  • In three patients, functioning tumors (one pheochromocytoma, one bilateral Cushing adenoma, and one adrenocortical carcinoma) and one nonfunctioning adenoma were diagnosed by histology and biochemical assessment.
  • Nonfunctioning adrenal lesions, not characterized by histology yet, were found in 18 patients.
  • There was no statistical difference with regard to adrenal involvement between patients with germline mutations in exons 2 and 10 (12/21) and those with mutations in exons 3-9 (6/11).
  • CONCLUSION: MEN-1-associated adrenal tumors are mostly small, benign, and nonfunctioning and much more common than previously reported.
  • [MeSH-major] Adrenal Gland Neoplasms. Multiple Endocrine Neoplasia Type 1

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  • (PMID = 17235589.001).
  • [ISSN] 1435-2443
  • [Journal-full-title] Langenbeck's archives of surgery
  • [ISO-abbreviation] Langenbecks Arch Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
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23. Mumford S: Reviewing the evidence for prescribing steroids for non-specific symptoms in patients with advanced cancer. Int J Palliat Nurs; 2010 Aug;16(8):406-10
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  • [Title] Reviewing the evidence for prescribing steroids for non-specific symptoms in patients with advanced cancer.
  • his article sets out to review the evidence for prescribing steroids for non-specific symptoms experienced by patients with advanced carcinoma.
  • [MeSH-major] Adrenal Cortex Hormones / therapeutic use. Evidence-Based Medicine. Neoplasms / drug therapy

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  • (PMID = 20852517.001).
  • [ISSN] 1357-6321
  • [Journal-full-title] International journal of palliative nursing
  • [ISO-abbreviation] Int J Palliat Nurs
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Adrenal Cortex Hormones
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24. Hancock S, Still RA, Fielding AM, Doran JF, Stephens JW: A rare cause of Cushing's syndrome demonstrates analytical discrepancies between the Roche E170 and Bayer Centaur testosterone assays. Ann Clin Biochem; 2008 May;45(Pt 3):328-30
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  • The initial biochemical investigations of a female patient with suspected Cushing's syndrome revealed abnormal endocrine results, including a marked elevation of serum testosterone.
  • Overnight and low-dose dexamethasone suppression tests confirmed the diagnosis of Cushing's syndrome.
  • Imaging investigations revealed an appearance compatible with adrenocortical carcinoma with metastases in the lungs and liver.
  • [MeSH-major] Cushing Syndrome / diagnosis. Testosterone / blood
  • [MeSH-minor] Adrenocortical Carcinoma / secondary. Adult. Female. Humans. Liver Neoplasms / secondary. Lung Neoplasms / secondary

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  • (PMID = 18482927.001).
  • [ISSN] 0004-5632
  • [Journal-full-title] Annals of clinical biochemistry
  • [ISO-abbreviation] Ann. Clin. Biochem.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 3XMK78S47O / Testosterone
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25. 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
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  • [Title] [Assessment of pro- and antiangiogenic factors blood serum concentrations in patients with hormonal inactive adrenal tumors].
  • [Transliterated title] Ocena stezeń wybranych czynników pro i antyangiogennych we krwi u chorych z nieczynnymi hormonalnie guzami nadnerczy.
  • 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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26. Suzuki H: Laparoscopic adrenalectomy for adrenal carcinoma and metastases. Curr Opin Urol; 2006 Mar;16(2):47-53
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  • [Title] Laparoscopic adrenalectomy for adrenal carcinoma and metastases.
  • PURPOSE OF REVIEW: This review article discusses topics concerned with laparoscopic adrenalectomy for adrenal pathologies.
  • RECENT FINDINGS: Over the past decade, laparoscopic adrenalectomy has become the operation of choice for the resection of adrenal tumors, and provides dramatically reduced morbidity associated with the operation.
  • Although current laparoscopic adrenalectomy for metastatic or primary adrenal malignancy is a feasible procedure, great care is required.
  • Laparoscopic adrenalectomy for these adrenal pathologies should be converted to open adrenalectomy or hand-assisted laparoscopic adrenalectomy for difficult dissection, invasion, adhesions or surgeon inexperience.
  • In general, laparoscopic adrenalectomy can be performed with acceptable outcomes in carefully selected patients with small, organ-confined, solitary adrenal metastasis or primary adrenal carcinoma.
  • SUMMARY: As surgical technique and complete resection are crucial to the success of the procedure, appropriate and careful judgment on the part of the surgeon is the most important factor in the care of a patient with such adrenal malignancies.
  • [MeSH-major] Adrenal Gland Neoplasms / surgery. Adrenalectomy / methods. Laparoscopy

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  • (PMID = 16479203.001).
  • [ISSN] 0963-0643
  • [Journal-full-title] Current opinion in urology
  • [ISO-abbreviation] Curr Opin Urol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 26
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27. Mantovani G, Lania AG, Bondioni S, Peverelli E, Pedroni C, Ferrero S, Pellegrini C, Vicentini L, Arnaldi G, Bosari S, Beck-Peccoz P, Spada A: Different expression of protein kinase A (PKA) regulatory subunits in cortisol-secreting adrenocortical tumors: relationship with cell proliferation. Exp Cell Res; 2008 Jan 1;314(1):123-30
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  • [Title] Different expression of protein kinase A (PKA) regulatory subunits in cortisol-secreting adrenocortical tumors: relationship with cell proliferation.
  • The four regulatory subunits (R1A, R1B, R2A, R2B) of protein kinase A (PKA) are differentially expressed in several cancer cell lines and exert distinct roles in growth control.
  • Mutations of the R1A gene have been found in patients with Carney complex and in a minority of sporadic primary pigmented nodular adrenocortical disease (PPNAD).
  • The aim of this study was to evaluate the expression of PKA regulatory subunits in non-PPNAD adrenocortical tumors causing ACTH-independent Cushing's syndrome and to test the impact of differential expression of these subunits on cell growth.
  • Conversely, carcinomas (n=5) showed high levels of all subunits.
  • The effect of R1/R2 ratio on proliferation was assessed in mouse adrenocortical Y-1 cells.
  • The R2-selective cAMP analogue 8-Cl-cAMP dose-dependently inhibited Y-1 cell proliferation and induced apoptosis, while the R1-selective cAMP analogue 8-HA-cAMP stimulated cell proliferation.
  • Finally, R2B gene silencing induced up-regulation of R1A protein, associated with an increase in cell proliferation.
  • In conclusion, we propose that a high R1/R2 ratio favors the proliferation of well differentiated and hormone producing adrenocortical cells, while unbalanced expression of these subunits is not required for malignant transformation.
  • [MeSH-major] Adrenal Cortex Neoplasms / enzymology. Adrenal Cortex Neoplasms / genetics. Adrenocortical Adenoma / enzymology. Adrenocortical Adenoma / genetics. Cell Proliferation / drug effects. Cyclic AMP-Dependent Protein Kinases / genetics. Hydrocortisone / secretion
  • [MeSH-minor] 8-Bromo Cyclic Adenosine Monophosphate / analogs & derivatives. 8-Bromo Cyclic Adenosine Monophosphate / pharmacology. Base Sequence / genetics. Carcinoma / enzymology. Carcinoma / genetics. Carcinoma / secretion. Cell Line, Tumor. Cell Transformation, Neoplastic / genetics. Cyclic AMP-Dependent Protein Kinase RIIbeta Subunit / genetics. Cyclic AMP-Dependent Protein Kinase RIIbeta Subunit / metabolism. Cyclic AMP-Dependent Protein Kinase RIalpha Subunit / genetics. Cyclic AMP-Dependent Protein Kinase RIalpha Subunit / metabolism. Dose-Response Relationship, Drug. Gene Expression Regulation, Enzymologic / genetics. Gene Expression Regulation, Neoplastic / genetics. Gene Silencing / physiology. Humans. Up-Regulation / genetics

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  • (PMID = 17904549.001).
  • [ISSN] 0014-4827
  • [Journal-full-title] Experimental cell research
  • [ISO-abbreviation] Exp. Cell Res.
  • [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 RIIbeta Subunit; 0 / Cyclic AMP-Dependent Protein Kinase RIalpha Subunit; 23583-48-4 / 8-Bromo Cyclic Adenosine Monophosphate; 41941-56-4 / 8-chloro-cyclic adenosine monophosphate; EC 2.7.11.11 / Cyclic AMP-Dependent Protein Kinases; WI4X0X7BPJ / Hydrocortisone
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28. Cantón RF, Scholten DE, Marsh G, de Jong PC, van den Berg M: Inhibition of human placental aromatase activity by hydroxylated polybrominated diphenyl ethers (OH-PBDEs). Toxicol Appl Pharmacol; 2008 Feb 15;227(1):68-75
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  • Recently, our laboratory focused on the possible effects on steroidogenesis of PBDEs and OH-PBDEs, e.g. in the human adrenocortical carcinoma (H295R) cell line indicating that some OH-PBDEs can significantly influence steroidogenic enzymes like CYP19 (aromatase) and CYP17.

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  • (PMID = 18022659.001).
  • [ISSN] 0041-008X
  • [Journal-full-title] Toxicology and applied pharmacology
  • [ISO-abbreviation] Toxicol. Appl. Pharmacol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Aromatase Inhibitors; 0 / Polybrominated Biphenyls; EC 1.14.14.1 / Aromatase
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29. Sencan M, Dokmetas HS: A case of postpartum hypopituitarism accompanied by Cushing's syndrome as a result of an adrenocortical carcinoma. Endocr J; 2005 Apr;52(2):219-22
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  • [Title] A case of postpartum hypopituitarism accompanied by Cushing's syndrome as a result of an adrenocortical carcinoma.
  • We found an adrenal mass with distant metastases to the liver and lung while investigating the origin of the hypercortisolism.
  • Accordingly, the patient was diagnosed as hypopituitarism due to Sheehan's syndrome accompanied by Cushing's syndrome as a result of an adrenocortical carcinoma.
  • [MeSH-major] Adrenal Cortex Neoplasms / complications. Carcinoma / complications. Cushing Syndrome / etiology. Hypopituitarism / etiology. Puerperal Disorders / etiology


30. Igaz P, Tömböl Z, Szabó PM, Likó I, Rácz K: Steroid biosynthesis inhibitors in the therapy of hypercortisolism: theory and practice. Curr Med Chem; 2008;15(26):2734-47
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  • Cushing's syndrome is a rare disease with significant morbidity and mortality.
  • It is not uncommon, however, that surgery fails to cure or control the disease.
  • A few drugs inhibiting single or multiple steps in adrenal steroid biosynthesis can be used in clinical practice.
  • Besides blocking multiple steps in adrenal steroid biosynthesis, the DDT (insecticide) analogue mitotane also has adrenolytic properties by inducing mitochondrial degeneration that renders it superior to other drugs in the treatment of adrenocortical cancer.

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  • (PMID = 18991633.001).
  • [ISSN] 0929-8673
  • [Journal-full-title] Current medicinal chemistry
  • [ISO-abbreviation] Curr. Med. Chem.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / Enzyme Inhibitors; 0 / Steroids
  • [Number-of-references] 153
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31. Moid F, DePalma L: Comparison of relative value of bone marrow aspirates and bone marrow trephine biopsies in the diagnosis of solid tumor metastasis and Hodgkin lymphoma: institutional experience and literature review. Arch Pathol Lab Med; 2005 Apr;129(4):497-501
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  • [Title] Comparison of relative value of bone marrow aspirates and bone marrow trephine biopsies in the diagnosis of solid tumor metastasis and Hodgkin lymphoma: institutional experience and literature review.
  • OBJECTIVE: To compare the relative value of aspirates and trephine biopsies in the diagnosis of solid tumor metastasis and Hodgkin lymphoma.
  • The diagnosis and findings made on aspirates were compared with those made on trephine biopsies in each case.
  • The correlation between aspirates and trephine biopsies was highest in cases of small cell carcinoma of the lung (3/11, or 36.3%) followed by breast carcinoma (7/20, or 35%), prostate carcinoma (1/9, or 11.1%), and Hodgkin lymphoma (1/20, or 5%).
  • Two of 5 cases from the miscellaneous category demonstrated simultaneous involvement of aspirate and trephine biopsy by a gastric carcinoma as well as an adrenal gland carcinoma.
  • In cases of breast carcinoma, small cell carcinoma of lung, and prostate carcinoma, aspirate evaluation may confirm trephine biopsy results or, more rarely, provide the sole confirmation of the malignancy.
  • [MeSH-major] Bone Marrow / pathology. Bone Marrow Examination / methods. Bone Marrow Neoplasms / pathology. Bone Marrow Neoplasms / secondary. Hodgkin Disease / pathology

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  • (PMID = 15794673.001).
  • [ISSN] 1543-2165
  • [Journal-full-title] Archives of pathology & laboratory medicine
  • [ISO-abbreviation] Arch. Pathol. Lab. Med.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] United States
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32. Zhu Y, Zhang HB, Lu L, Li NS, Fu Y, Wang O, Xu LL, Jiang Y, Tong AL, Zhu HJ, Li W, Hu MM, Yu M, Mao JF, Yuan T, Li M, Xia WB, Xing XP: [Adrenal lesions in patients of multiple endocrine neoplasia type 1]. Zhonghua Yi Xue Za Zhi; 2010 Oct 19;90(38):2689-92
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  • [Title] [Adrenal lesions in patients of multiple endocrine neoplasia type 1].
  • OBJECTIVE: To investigate the prevalence and characteristics of adrenal lesions in Chinese multiple endocrine neoplasia type 1 (MEN-1) patients.
  • METHODS: Adrenal CT scan and clinical manifestations were retrospectively reviewed in 32 consecutive MEN-1 patients who were evaluated at our hospital during January 1986 to December 2009.
  • RESULTS: Adrenal lesions were identified in 16 of 32 (50%) MEN-1 patients.
  • Five (31.3%) patents with adrenal involvement showed bilateral lesions, including bilateral adenoma (n=1), bilateral hyperplasia (n=2) and adenoma and hyperplasia on each side (n=2).
  • Unilateral adrenal lesion was presented in 11 (68.7%) patients.
  • In two patients, functioning adrenal abnormalities were detected including Cushing adenoma (n=1) and aldosterone-secreting adenoma (n=1).
  • CONCLUSIONS: The prevalence of adrenal lesion in MEN-1 patient is similar between China and western countries.
  • Taking into account a high incidence of adrenal carcinoma in previous foreign studies, routine screening and close surveillance are still recommended for adrenal lesions in MEN-1 patients.
  • [MeSH-major] Adrenal Glands / pathology. Multiple Endocrine Neoplasia Type 1 / pathology


33. Kirschner LS: Emerging treatment strategies for adrenocortical carcinoma: a new hope. J Clin Endocrinol Metab; 2006 Jan;91(1):14-21
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  • [Title] Emerging treatment strategies for adrenocortical carcinoma: a new hope.
  • CONTEXT: Adrenocortical carcinoma (ACC) is a rare cancer but one that has devastating consequences for affected patients.
  • Surgery is the mainstay of therapy, although the high frequency of metastatic disease implies that it is frequently noncurative.
  • Traditional cytotoxic chemotherapy for ACC has generally produced disappointing responses, implying the need for the new therapies for this disease.
  • EVIDENCE ACQUISITION: Review articles and primary literature were identified by extensive PubMed searching to obtain papers evaluating the current state of knowledge regarding ACC, as well as assessing the development of new therapeutic modalities for the treatment of cancer.
  • This review provides a brief summary of the progress and prospects of each of these modalities and focuses on emerging data and treatments that may alter the course of this disease within the next few years.
  • CONCLUSIONS: Despite the current grim outlook, the recent applications of emerging technology to the study of ACC and the development of newer, "targeted" therapies for cancer suggest the possibility of a new hope for patients with this disease, although these therapies will need to be evaluated by rigorous clinical trials to verify their effectiveness.
  • [MeSH-major] Adrenal Cortex Neoplasms / therapy. Carcinoma / therapy

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  • (PMID = 16234301.001).
  • [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; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Angiogenesis Inhibitors; 0 / Antineoplastic Agents; 0 / Enzyme Inhibitors; EC 2.7.10.1 / Protein-Tyrosine Kinases
  • [Number-of-references] 127
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34. Bertherat J, Groussin L, Bertagna X: Mechanisms of disease: adrenocortical tumors--molecular advances and clinical perspectives. Nat Clin Pract Endocrinol Metab; 2006 Nov;2(11):632-41
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  • [Title] Mechanisms of disease: adrenocortical tumors--molecular advances and clinical perspectives.
  • Most adrenocortical tumors are benign, unilateral, adrenocortical adenomas that are often discovered incidentally.
  • Adrenocortical cancer is rare.
  • Exceptionally, adrenocortical tumors can be bilateral.
  • Although most adrenocortical tumors occur sporadically, they may also feature in congenital and/or familial disease.
  • The identification of germline genetic defects in familial diseases associated with adrenocortical tumors helped to define the somatic alterations in sporadic disease: for example, overexpression of insulin-like growth factor 2 and alterations at the 11p15 locus (observed in Beckwith-Wiedemann syndrome) are also found in most adrenocortical cancers.
  • Similarly, inactivating mutations of the TP53 gene, located at 17p13 (observed in Li-Fraumeni syndrome), can also be found at the somatic level in sporadic adrenocortical cancers, as can 17p13 allelic losses.
  • Components of the cyclic AMP signaling pathway--for example, adrenocorticotropic hormone receptors and other membrane receptors, Gs proteins and protein kinase A--can be altered to various degrees in adrenocortical tumors.
  • More recently, gene profiling and genetic studies have shown that the Wnt-beta-catenin signaling pathway is frequently activated in adrenocortical tumors.
  • These research findings already have profound implications for clinical management of patients with adrenocortical tumors, for example in unraveling the genetic origin of the disease in some patients, and in the development of molecular markers for diagnosis and prognosis.
  • [MeSH-major] Adrenal Cortex Neoplasms / genetics. Adrenocortical Adenoma / genetics

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  • (PMID = 17082810.001).
  • [ISSN] 1745-8366
  • [Journal-full-title] Nature clinical practice. Endocrinology & metabolism
  • [ISO-abbreviation] Nat Clin Pract Endocrinol Metab
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents
  • [Number-of-references] 54
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35. Vranic S, Kapur L, Foco F, Bilalovic N, Hainaut P: The first case of Li-Fraumeni syndrome in Bosnia and Herzegovina: case report. Pathologica; 2006 Apr;98(2):156-9
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  • Li-Fraumeni syndrome (LFS) is a very rare autosomal dominant and highly penetrant cancer syndrome characterized by early-onset primary tumours, including soft tissue and bone sarcoma, breast cancer, leukemia, brain tumours and adrenocortical carcinoma.
  • [MeSH-minor] Bosnia and Herzegovina / epidemiology. Breast Neoplasms / genetics. Breast Neoplasms / pathology. Breast Neoplasms / surgery. Child. DNA, Neoplasm / genetics. Exons / genetics. Facial Neoplasms / diagnosis. Facial Neoplasms / genetics. Facial Neoplasms / pathology. Facial Neoplasms / surgery. Female. Fibroadenoma / genetics. Fibroadenoma / pathology. Fibroadenoma / surgery. Genes, p53. Germ-Line Mutation. Haplotypes / genetics. Humans. Introns / genetics. Lip Neoplasms / genetics. Lip Neoplasms / pathology. Lip Neoplasms / surgery. Neoplasm Recurrence, Local. Orbit Evisceration. Pedigree. Rhabdomyosarcoma / diagnosis. Rhabdomyosarcoma / genetics. Rhabdomyosarcoma / pathology. Rhabdomyosarcoma / surgery

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  • (PMID = 16929790.001).
  • [ISSN] 0031-2983
  • [Journal-full-title] Pathologica
  • [ISO-abbreviation] Pathologica
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / DNA, Neoplasm
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36. Doghman M, Cazareth J, Lalli E: The T cell factor/beta-catenin antagonist PKF115-584 inhibits proliferation of adrenocortical carcinoma cells. J Clin Endocrinol Metab; 2008 Aug;93(8):3222-5
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  • [Title] The T cell factor/beta-catenin antagonist PKF115-584 inhibits proliferation of adrenocortical carcinoma cells.
  • CONTEXT: Mutations of the beta-catenin (CTNNB1) gene are frequently found in adrenocortical tumors.
  • OBJECTIVE: The objective of the study was to investigate the effect of the small-molecule inhibitor of the T cell factor (Tcf)/beta-catenin complex PKF115-584 on beta-catenin-dependent transcription and proliferation of H295R adrenocortical tumor cells, which harbor mutations in CTNNB1 as well as the TP53 tumor suppressor gene.
  • PKF115-584 dose-dependently inhibited beta-catenin-dependent transcription and H295R proliferation, even in the presence of increased steroidogenic factor-1 levels, which augment proliferation in this cell line.
  • The drug had no effect on HeLa cells, a cell line in which the beta-catenin pathway is not activated.
  • CONCLUSIONS: Inhibitors of the Tcf/beta-catenin complex may prove useful in the treatment of adrenocortical tumors in which multiple genetic alterations have accumulated.
  • [MeSH-major] Adrenal Cortex Neoplasms / drug therapy. TCF Transcription Factors / antagonists & inhibitors. beta Catenin / antagonists & inhibitors
  • [MeSH-minor] Cell Proliferation / drug effects. Genes, p53. Humans. Mutation. Tumor Cells, Cultured

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  • (PMID = 18544621.001).
  • [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 / TCF Transcription Factors; 0 / beta Catenin
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37. Wagnerova H, Lazúrová I, Habalová V, Dudásová D, Vrzgula A: The prevalence of 21-hydroxylase deficiency in adrenal incidentalomas - hormonal and mutation screening. Exp Clin Endocrinol Diabetes; 2008 May;116(5):272-5
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  • [Title] The prevalence of 21-hydroxylase deficiency in adrenal incidentalomas - hormonal and mutation screening.
  • The aim of the present study was to evaluate and compare the response of 17 OHP to ACTH stimulation in patients with various types of adrenal incidentalomas and to examine the occurence of germline CYP21 mutation in these patients.
  • 21 of them had unilateral adrenal adenoma, 13 patients had adrenal hyperplasia (six of them unilateral) and 6 patients had CT characteristics of other tumors (myelolipomas, cysts, adrenocortical carcinoma).
  • SUMMARY: Although 12 % of patients with adrenal incidentalomas had an exaggerated response of 17 OHP after ACTH administration indicating a possible 21-hydroxylase deficiency, these findings are not associated with CYP21 mutation estimated in peripheral blood samples.
  • There was found no germline CYP21 mutation in all patients with various adrenal incidentalomas.
  • [MeSH-major] Adenoma / epidemiology. Adrenal Gland Neoplasms / epidemiology. Adrenal Hyperplasia, Congenital / genetics. Hormones / secretion. Incidental Findings. Steroid 21-Hydroxylase / genetics
  • [MeSH-minor] 17-alpha-Hydroxyprogesterone / blood. Adrenal Cortex Function Tests. Adrenocorticotropic Hormone. Adult. Aged. DNA Mutational Analysis. Female. Germ-Line Mutation. Humans. Hyperplasia / blood. Hyperplasia / epidemiology. Hyperplasia / genetics. Male. Middle Aged. Prevalence

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  • (PMID = 18589890.001).
  • [ISSN] 0947-7349
  • [Journal-full-title] Experimental and clinical endocrinology & diabetes : official journal, German Society of Endocrinology [and] German Diabetes Association
  • [ISO-abbreviation] Exp. Clin. Endocrinol. Diabetes
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Hormones; 68-96-2 / 17-alpha-Hydroxyprogesterone; 9002-60-2 / Adrenocorticotropic Hormone; EC 1.14.99.10 / Steroid 21-Hydroxylase
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38. Tomkova K, Tomka M, Zajac V: Contribution of p53, p63, and p73 to the developmental diseases and cancer. Neoplasma; 2008;55(3):177-81
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  • [Title] Contribution of p53, p63, and p73 to the developmental diseases and cancer.
  • This syndrome is characterized by the early onset of different types of cancers including soft-tissue sarcomas, breast and brain cancers, leukemias, lung, laryngeal cancers, and adrenocortical carcinomas.

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  • (PMID = 18348649.001).
  • [ISSN] 0028-2685
  • [Journal-full-title] Neoplasma
  • [ISO-abbreviation] Neoplasma
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Slovakia
  • [Chemical-registry-number] 0 / CKAP4 protein, human; 0 / DNA-Binding Proteins; 0 / Membrane Proteins; 0 / Nuclear Proteins; 0 / Tumor Suppressor Proteins
  • [Number-of-references] 63
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39. Caso J, Seigne J, Back M, Spiess PE, Pow-Sang J, Sexton WJ: Circumferential resection of the inferior vena cava for primary and recurrent malignant tumors. J Urol; 2009 Sep;182(3):887-93
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  • [Title] Circumferential resection of the inferior vena cava for primary and recurrent malignant tumors.
  • We explored the oncological effectiveness of inferior vena caval resection, as determined by margin status, cancer recurrence and survival.
  • Primary tumor type was renal cell carcinoma in 7 patients, metastatic testicular cancer in 5, leiomyosarcoma in 3, and adrenal cortical carcinoma, primary retroperitoneal germ cell tumor and upper tract transitional cell carcinoma in 1 each.
  • Data reviewed included preoperative and postoperative sequelae of inferior vena caval obstruction, postoperative complications, pathological results, cancer recurrence, graft requirements and functional outcomes.
  • A total of 12 patients underwent simultaneous nephrectomy and/or left renal vein ligation in the same setting with acceptable alterations in postoperative renal function and no need for permanent dialysis.
  • Cancer recurred locally in 4 of 15 patients who underwent resection.
  • Five of 15 patients in the resection group died of disease or were lost to followup compared to all 3 in whom resection was aborted or macroscopically incomplete (mean followup 19.2 vs 4.3 months).
  • CONCLUSIONS: Local cancer control and potentially increased cancer specific survival can be achieved with successful complete circumferential resection of the inferior vena cava as a component of multimodality care in select patients with locally advanced malignancy.
  • The most common postoperative complications are renal insufficiency and lower extremity edema, which are generally transient.

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  • (PMID = 19616230.001).
  • [ISSN] 1527-3792
  • [Journal-full-title] The Journal of urology
  • [ISO-abbreviation] J. Urol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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40. Malandrino P, Al Ghuzlan A, Castaing M, Young J, Caillou B, Travagli JP, Elias D, de Baere T, Dromain C, Paci A, Chanson P, Schlumberger M, Leboulleux S, Baudin E: Prognostic markers of survival after combined mitotane- and platinum-based chemotherapy in metastatic adrenocortical carcinoma. Endocr Relat Cancer; 2010 Sep;17(3):797-807
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  • [Title] Prognostic markers of survival after combined mitotane- and platinum-based chemotherapy in metastatic adrenocortical carcinoma.
  • To progress in the stratification of the first-line therapeutic management of metastatic adrenocortical carcinoma (ACC), we searched for prognostic parameters of survival in patients treated with combined mitotane- and cisplatinum-based chemotherapy as first-line.
  • In the univariate analysis, resection of the primary, time since diagnosis, mitotane monotherapy as single first-line treatment, number of affected organs, plasma mitotane above 14 mg/l, and objective response were predictors of survival.
  • [MeSH-major] Adrenal Cortex Neoplasms / drug therapy. Adrenal Cortex Neoplasms / metabolism. Adrenocortical Carcinoma / drug therapy. Adrenocortical Carcinoma / metabolism. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Biomarkers, Tumor / metabolism

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  • (PMID = 20592067.001).
  • [ISSN] 1479-6821
  • [Journal-full-title] Endocrine-related cancer
  • [ISO-abbreviation] Endocr. Relat. Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / DNA-Binding Proteins; 78E4J5IB5J / Mitotane; EC 3.1.- / ERCC1 protein, human; EC 3.1.- / Endonucleases; Q20Q21Q62J / Cisplatin
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41. Trahair T, Andrews L, Cohn RJ: Recognition of Li Fraumeni syndrome at diagnosis of a locally advanced extremity rhabdomyosarcoma. Pediatr Blood Cancer; 2007 Mar;48(3):345-8
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  • [Title] Recognition of Li Fraumeni syndrome at diagnosis of a locally advanced extremity rhabdomyosarcoma.
  • A contemporaneous presentation of a second breast cancer in a mother and an extremity rhabdomyosarcoma (RMS) in her daughter led to the diagnosis of the Li Fraumeni syndrome (LFS).
  • [MeSH-major] Genes, p53. Li-Fraumeni Syndrome / diagnosis. Muscle Neoplasms / genetics. Rhabdomyosarcoma, Embryonal / genetics
  • [MeSH-minor] Adrenal Cortex Neoplasms / genetics. Adrenocortical Carcinoma / genetics. Adult. Antineoplastic Agents / therapeutic use. Astrocytoma / genetics. Astrocytoma / surgery. Breast Neoplasms / genetics. Carcinoma / genetics. Case Management. Child, Preschool. Codon, Nonsense. Esophageal Neoplasms / genetics. Female. Humans. Leg. Liver Neoplasms / genetics. Male. Neoplasms, Radiation-Induced / prevention & control. Neoplasms, Second Primary / prevention & control. Pedigree. Radiotherapy, Adjuvant / contraindications. Retroperitoneal Neoplasms / genetics. Retroperitoneal Neoplasms / surgery. Sarcoma / genetics. Sarcoma / surgery

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  • [Copyright] (c) 2006 Wiley-Liss, Inc.
  • (PMID = 16534790.001).
  • [ISSN] 1545-5009
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Codon, Nonsense
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42. Schteingart DE: Adjuvant mitotane therapy of adrenal cancer - use and controversy. N Engl J Med; 2007 Jun 7;356(23):2415-8
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  • [Title] Adjuvant mitotane therapy of adrenal cancer - use and controversy.
  • [MeSH-major] Adrenal Cortex Neoplasms / drug therapy. Adrenocortical Carcinoma / drug therapy. Antineoplastic Agents, Hormonal / therapeutic use. Mitotane / therapeutic use

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  • [CommentIn] N Engl J Med. 2007 Sep 20;357(12):1257-8; author reply 1259 [17891838.001]
  • [CommentOn] N Engl J Med. 2007 Jun 7;356(23):2372-80 [17554118.001]
  • (PMID = 17554125.001).
  • [ISSN] 1533-4406
  • [Journal-full-title] The New England journal of medicine
  • [ISO-abbreviation] N. Engl. J. Med.
  • [Language] eng
  • [Publication-type] Comment; Editorial
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Hormonal; 78E4J5IB5J / Mitotane
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43. Muro Toledo GE, Losada Guerra JL, Martín Pérez A, Pérez Marín IR: [Giant adrenal carcinoma. Case report]. Arch Esp Urol; 2009 Mar;62(2):134-6
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  • [Title] [Giant adrenal carcinoma. Case report].
  • [Transliterated title] Carcinoma suprarenal gigante. Presentación de un caso.
  • BACKGROUND: To present a case of giant suprarenal carcinoma assisted by the Urology Service of the "Camilo Cienfuegos Gorriarán" General University Hospital of Sancti Spíritus, Cuba.
  • Pathological study confirmed the diagnosis of suprarenal carcinoma.
  • CONCLUSIONS: The big dimensions of the tumor determined a wide excision surgery which didn't stop the progression of the disease and the death of the patient 6 months after being operated.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Carcinoma / pathology

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  • (PMID = 19448281.001).
  • [ISSN] 0004-0614
  • [Journal-full-title] Archivos españoles de urología
  • [ISO-abbreviation] Arch. Esp. Urol.
  • [Language] spa
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Spain
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44. Hiroi N, Yanagisawa R, Yoshida-Hiroi M, Endo T, Kawase T, Tsuchida Y, Toyama K, Shibuya K, Nakata K, Yoshino G: Retroperitoneal hemorrhage due to bilateral adrenal metastases from lung adenocarcinoma. J Endocrinol Invest; 2006 Jun;29(6):551-4
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  • [Title] Retroperitoneal hemorrhage due to bilateral adrenal metastases from lung adenocarcinoma.
  • A 56-yr-old man was admitted to our university hospital for severe back pain one month after a resection for lung adenocarcinoma (stage IIIA) without evidence of the adrenal mass.
  • Computed tomography (CT) of the abdomen showed bilateral bleeding of adrenal tumors.
  • Core-needle biopsy was performed on the right adrenal tumor and revealed adenocarcinoma cells mimicking a primary lung tumor previously examined.
  • We diagnosed retroperitoneal hemorrhage due to bilateral adrenal gland metastasis from lung adenocarcinoma with adrenal insufficiency.
  • Adrenal metastases most commonly originate from a primary lung tumor, followed by stomach, esophagus and liver/bile ducts.
  • Bilateral adrenal metastases were noted in approximately half of all adrenal metastases patients.
  • Clinically significant adrenal hemorrhage by metastasis is exceedingly rare and non-specific symptoms, such as abdominal, chest or back pain, nausea and vomiting, confusion, weakness, hypotension, shock and high fever, are often observed in these patients.
  • We present a case of massive retroperitoneal hemorrhage and adrenal insufficiency due to adrenal gland metastasis from adenocarcinoma of lung.
  • [MeSH-major] Adenocarcinoma / secondary. Adrenal Gland Neoplasms / secondary. Adrenal Insufficiency / etiology. Hemorrhage / etiology. Lung Neoplasms / pathology

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  • (PMID = 16840834.001).
  • [ISSN] 0391-4097
  • [Journal-full-title] Journal of endocrinological investigation
  • [ISO-abbreviation] J. Endocrinol. Invest.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Italy
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45. Abiven G, Coste J, Groussin L, Anract P, Tissier F, Legmann P, Dousset B, Bertagna X, Bertherat J: Clinical and biological features in the prognosis of adrenocortical cancer: poor outcome of cortisol-secreting tumors in a series of 202 consecutive patients. J Clin Endocrinol Metab; 2006 Jul;91(7):2650-5
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  • [Title] Clinical and biological features in the prognosis of adrenocortical cancer: poor outcome of cortisol-secreting tumors in a series of 202 consecutive patients.
  • CONTEXT: Adrenocortical carcinomas (ACC) are rare tumors with a poor prognosis.
  • DESIGN AND SETTING: This study is a descriptive and multivariate analysis of a cohort from a single endocrinology center.
  • Mean age at diagnosis was 44 +/- 16 yr (range, 11-88 yr).
  • We found that 154 patients (76%) had hypersecreting tumors [mostly cortisol and androgens (47%), cortisol alone (27%), or androgens alone (6%)] and 43 patients (21%) had metastases at diagnosis.
  • Multivariate analysis identified the following independent prognostic factors associated with shorter survival: older age at diagnosis [hazard ratio (HR), 1.03; P < 0.0001], initial MacFarlane extension stages 3 (HR, 4.42; P = 0.005) and 4 (HR, 7.93; P < 0.0001), and cortisol hypersecretion (HR, 3.90; P < 0.0001).
  • [MeSH-major] Adrenal Cortex Neoplasms / secretion. Hydrocortisone / secretion

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  • (PMID = 16670169.001).
  • [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 / Androgens; 0 / Antineoplastic Agents, Hormonal; 78E4J5IB5J / Mitotane; WI4X0X7BPJ / Hydrocortisone
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46. Babinska A, Sworczak K, Wisniewski P, Nałecz A, Jaskiewicz K: The role of immunohistochemistry in histopathological diagnostics of clinically "silent" incidentally detected adrenal masses. Exp Clin Endocrinol Diabetes; 2008 Apr;116(4):246-51
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  • [Title] The role of immunohistochemistry in histopathological diagnostics of clinically "silent" incidentally detected adrenal masses.
  • BACKGROUND: The detectability of adrenal incidentalomas (incidentally found adrenal tumours) in the whole population is estimated at 0.1%; 0.42% in non-endocrine patients and at 4.3% in oncologically diagnosed ones.
  • Even up to 16% of incidentalomas of adrenal glands can be malignant lesions.
  • The issue of crucial importance is the histopathological differentiation between benign lesions and malignant tumours of the adrenal cortex and medulla.
  • OBJECTIVES: To evaluate whether the immunohistochemical analysis of the expression of p53, p21, PCNA and Ki67 in the tumour's tissue can be useful in the histopathological diagnostics of adrenal incidentalomas and whether it is important for prognosis.
  • There were 43 cortical adenomas, 11 cortical adrenocarcinomas and 20 PHEOs (including 5 malignant lesions).
  • RESULTS: We found a statistically significant correlation between the expression of p53, p21, Ki67 and the differential diagnosis of adrenal cortical adenoma and adrenocortical carcinoma (for proteins: p53 p=0.010, for p21 p=0.010, for Ki67 p<0.001).
  • The statistical significant correlation between PCNA protein and diagnosis of adrenal cortical adenoma and adrenocortical carcinoma was not found.
  • The statistically significant correlation between p21, PCNA proteins and the diagnosis of benign and malignant PHEOs was not estimated.
  • There was no expression of Ki67 or p53 protein above the assumed level in benign and malignant pheochromocytomas.
  • The statistically significant correlation between p53, p21, PCNA or Ki67 and the occurrence of metastases in adrenocarcinoma and malignant PHEOs was not found.
  • [MeSH-major] Adenoma / pathology. Adrenal Gland Neoplasms / pathology. Pheochromocytoma / pathology
  • [MeSH-minor] Gene Expression Regulation, Neoplastic. Humans. Immunohistochemistry. Ki-67 Antigen / genetics. Proliferating Cell Nuclear Antigen / genetics. Proto-Oncogene Proteins c-bcl-2 / genetics. Tumor Suppressor Protein p53 / genetics. p21-Activated Kinases / genetics

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  • (PMID = 18393131.001).
  • [ISSN] 0947-7349
  • [Journal-full-title] Experimental and clinical endocrinology & diabetes : official journal, German Society of Endocrinology [and] German Diabetes Association
  • [ISO-abbreviation] Exp. Clin. Endocrinol. Diabetes
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Ki-67 Antigen; 0 / Proliferating Cell Nuclear Antigen; 0 / Proto-Oncogene Proteins c-bcl-2; 0 / Tumor Suppressor Protein p53; EC 2.7.11.1 / p21-Activated Kinases
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47. Giordano TJ: Adrenocortical tumors: an integrated clinical, pathologic, and molecular approach at the University of Michigan. Arch Pathol Lab Med; 2010 Oct;134(10):1440-3
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  • [Title] Adrenocortical tumors: an integrated clinical, pathologic, and molecular approach at the University of Michigan.
  • Recently, an integrated clinical and research program focused on primary cancer of the adrenal gland has been developed.
  • OBJECTIVE: To discuss the foundation of the University of Michigan Adrenal Cancer Program that consists of 3 components:.
  • DATA SOURCES: Recent programmatic activity includes genome-wide transcriptomic evaluation of human adrenocortical tumors for diagnostic and prognostic evaluation; interrogation of the Wnt signaling pathway in adrenocortical carcinoma, using mouse models and transcriptome profiling; and clinical trials with targeted therapy focused on inhibition of insulin-like growth factor signaling pathway.
  • [MeSH-major] Adrenal Cortex Neoplasms / genetics. Adrenal Cortex Neoplasms / pathology

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  • (PMID = 20923297.001).
  • [ISSN] 1543-2165
  • [Journal-full-title] Archives of pathology & laboratory medicine
  • [ISO-abbreviation] Arch. Pathol. Lab. Med.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] EC 2.7.10.1 / Receptor, IGF Type 1
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48. Advani A, Vaikkakara S, Gill MS, Arun CS, Pearce SH, Ball SG, James RA, Lennard TW, Bliss RD, Quinton R, Johnson SJ: Impact of standardised reporting in adrenocortical carcinoma: a single centre clinicopathological review. J Clin Pathol; 2008 Aug;61(8):939-44
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  • [Title] Impact of standardised reporting in adrenocortical carcinoma: a single centre clinicopathological review.
  • AIMS: Structured multicentre efforts are needed if the prognosis of adrenocortical carcinoma (ACC) is to be improved.
  • CONCLUSIONS: Standardised criteria for histopathological reporting of ACC will improve the accuracy of data for cancer registration and may also assist in individual patient stratification.
  • [MeSH-major] Adrenal Cortex Neoplasms / pathology. Adrenocortical Carcinoma / pathology. Medical Records / standards

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  • (PMID = 18515402.001).
  • [ISSN] 1472-4146
  • [Journal-full-title] Journal of clinical pathology
  • [ISO-abbreviation] J. Clin. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Hormonal; 0 / Ki-67 Antigen; 78E4J5IB5J / Mitotane
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49. Dichtchekenian V, de Bragança Pereira CA, Kuperman H, Della Manna T, Damiani D, Ferreira Alves VA, Filho AL, Setian N: Adrenocortical carcinoma: prognostic indices based on clinical and immunohistochemical markers. J Pediatr Endocrinol Metab; 2005 Apr;18(4):347-53
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  • [Title] Adrenocortical carcinoma: prognostic indices based on clinical and immunohistochemical markers.
  • Adrenocortical carcinoma is a rare condition with an unpredictable prognosis as a rule.
  • Y = 1 when chronological age (CA) >33 mo, Y = 0 when CA < or =33 mo; L = 1 for right sided tumor and L = 0 for left sided tumor; H = 1 in presence of hypertension and H = 0 for normal blood pressure; T = length of disease in months; W = weight of tumor (g); O = 1 in the absence of p53 protein and O = 0 in the presence of p53.
  • [MeSH-major] Adrenal Cortex Neoplasms / physiopathology. Adrenocortical Carcinoma / physiopathology. Biomarkers, Tumor / metabolism. Models, Biological

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  • (PMID = 15844468.001).
  • [ISSN] 0334-018X
  • [Journal-full-title] Journal of pediatric endocrinology & metabolism : JPEM
  • [ISO-abbreviation] J. Pediatr. Endocrinol. Metab.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
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50. Cantini G, Lombardi A, Piscitelli E, Poli G, Ceni E, Marchiani S, Ercolino T, Galli A, Serio M, Mannelli M, Luconi M: Rosiglitazone inhibits adrenocortical cancer cell proliferation by interfering with the IGF-IR intracellular signaling. PPAR Res; 2008;2008:904041
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  • [Title] Rosiglitazone inhibits adrenocortical cancer cell proliferation by interfering with the IGF-IR intracellular signaling.
  • We investigated RGZ effect on cell proliferation in two cell line models (SW13 and H295R) of human adrenocortical carcinoma (ACC) and its interaction with the signaling pathways of the activated IGF-I receptor (IGF-IR).
  • We demonstrate a high expression of IGF-IR in the two cell lines and in ACC.
  • Cell proliferation is stimulated by IGF-I in a dose- and time-dependent manner and is inhibited by RGZ.
  • In conclusion, our results suggest that RGZ exerts an inhibitory effect on human ACC cell proliferation by interfering with the PI3K/Akt and ERK1/2 signaling pathways downstream of the activated IGF-IR.

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  • (PMID = 18670617.001).
  • [ISSN] 1687-4757
  • [Journal-full-title] PPAR research
  • [ISO-abbreviation] PPAR Res
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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51. Hahner S, Stürmer A, Fassnacht M, Hartmann RW, Schewe K, Cochran S, Zink M, Schirbel A, Allolio B: Etomidate unmasks intraadrenal regulation of steroidogenesis and proliferation in adrenal cortical cell lines. Horm Metab Res; 2010 Jun;42(7):528-34
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  • [Title] Etomidate unmasks intraadrenal regulation of steroidogenesis and proliferation in adrenal cortical cell lines.
  • To characterize intraadrenal adaptations for inhibition of cortisol synthesis, we analyzed the effects of etomidate (ETO) on steroid hormone secretion and expression of key regulators of steroidogenesis and proliferation in human NCI-h295 adrenocortical cancer cells.
  • In summary, ETO exhibits pleiotropic effects on adrenal function in vitro.
  • These changes reflect adaptations to maintain steroidogenesis at the cost of adrenal proliferation.
  • [MeSH-major] Adrenal Cortex / cytology. Adrenal Cortex / metabolism. Cell Proliferation / drug effects. Etomidate / pharmacology. Steroids / biosynthesis
  • [MeSH-minor] Cell Line. Cholesterol Side-Chain Cleavage Enzyme / genetics. Cholesterol Side-Chain Cleavage Enzyme / metabolism. Cytochrome P-450 CYP11B2 / genetics. Cytochrome P-450 CYP11B2 / metabolism. Gene Expression Regulation / drug effects. Humans. Steroid 11-beta-Hydroxylase / genetics. Steroid 11-beta-Hydroxylase / metabolism

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  • [Copyright] Georg Thieme Verlag KG Stuttgart * New York.
  • (PMID = 20352599.001).
  • [ISSN] 1439-4286
  • [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 / Steroids; EC 1.14.15.4 / Cytochrome P-450 CYP11B2; EC 1.14.15.4 / Steroid 11-beta-Hydroxylase; EC 1.14.15.6 / Cholesterol Side-Chain Cleavage Enzyme; Z22628B598 / Etomidate
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52. Ji ZG, Xue C, Li HZ, Wang HJ, Xie Y, Liu GH: Piggyback liver transplant techniques in the surgical management of urological tumors with inferior vena cava tumor thrombus. Chin Med J (Engl); 2009 Sep 20;122(18):2155-8
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  • BACKGROUND: An important characteristic of renal cell carcinomas and adrenal tumors is that these tumors may expand into the renal vein and inferior vena cava, and transform into tumor thrombi.
  • METHODS: Nineteen patients with renal cell carcinomas or adrenal tumors with inferior vena cava tumor thrombus were treated from November 1995 to April 2008.
  • One patient with adrenal cortical carcinoma and level IV thrombus died in the immediate postoperative period.
  • [MeSH-major] Adrenal Gland Neoplasms / surgery. Carcinoma, Renal Cell / surgery. Liver Transplantation / methods. Vena Cava, Inferior / pathology


53. Gamliel-Lazarovich A, Gantman A, Shiner M, Coleman R, Aviram M, Keidar S: Paraoxonase 1 deficiency in mice is associated with reduced steroid biosynthesis: effects on HDL binding, cholesteryl ester accumulation and scavenger receptor type BI expression. Atherosclerosis; 2010 Jul;211(1):130-5
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  • OBJECTIVE: Selective uptake of high density lipoprotein (HDL) cholesteryl ester (CE) is considered as the major source of cholesterol for production of steroids in the adrenal gland in rodents.
  • Adrenal glands obtained from PON1KO mice had significantly lower CE content compared to adrenals from C57Bl6 control mice.
  • Binding of HDL obtained from PON1KO mice to human adrenocortical carcinoma cell line was found to be significantly lower than that of control HDL, and was associated with decreased CS biosynthesis.
  • When challenged with low salt diet, PON1KO mice demonstrated an increase in adrenal SR-BI gene expression and in serum corticosterone which reached levels similar to those obtained in control mice.
  • CONCLUSION: PON1 regulates adrenal CS biosynthesis at two levels: (a) via an accessory role in HDL binding properties, and (b) a supportive role in SR-BI expression and CE supply to the cells.
  • [MeSH-minor] Adrenal Glands / metabolism. Animals. Diet, Sodium-Restricted. Humans. Mice. Mice, Knockout. Tumor Cells, Cultured

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  • [Copyright] Copyright (c) 2010 Elsevier Ireland Ltd. All rights reserved.
  • (PMID = 20189567.001).
  • [ISSN] 1879-1484
  • [Journal-full-title] Atherosclerosis
  • [ISO-abbreviation] Atherosclerosis
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Ireland
  • [Chemical-registry-number] 0 / Cholesterol Esters; 0 / Lipoproteins, HDL; 0 / Scarb1 protein, mouse; 0 / Scavenger Receptors, Class B; EC 3.1.8.1 / Aryldialkylphosphatase; W980KJ009P / Corticosterone
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54. Kuwada M, Hosokawa Y, Takada S, Kumamoto H, Hayashi Y, Fujimoto K, Hirao Y: [Adrenocortical carcinoma with intratumoral hemorrhage detected from chest and back pain: a case report]. Hinyokika Kiyo; 2009 Oct;55(10):599-602
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  • [Title] [Adrenocortical carcinoma with intratumoral hemorrhage detected from chest and back pain: a case report].
  • Based on abdominal CT, magnetic resonance imaging and blood tests, preoperative diagnosis was adrenocortical carcinoma.
  • The histological diagnosis was adrenocortical carcinoma.
  • The patient died three months after operation because of disease progression.
  • [MeSH-major] Adrenal Cortex Neoplasms / diagnosis. Back Pain / etiology. Carcinoma / diagnosis. Chest Pain / etiology. Hemorrhage / etiology


55. Dural C, Bilge O, Toker A, Erbil Y, Salmaslioglu A, Ozbey N, Aral F: Malignant phaeochromocytoma with cavoatrial extension: transcaval removal of tumor without cardiopulmonary bypass A case report. Minerva Chir; 2010 Aug;65(4):485-8
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  • [Title] Malignant phaeochromocytoma with cavoatrial extension: transcaval removal of tumor without cardiopulmonary bypass A case report.
  • Adrenal carcinoma is a rare tumor and with metastasis usually in lungs, lymph nodes, liver, and bones.
  • The surgical approach to adrenal tumor extending into the vena cava is challenging.
  • This article reports a case of malignant pheochromocytoma extending into the cavoatrial junction in a young man.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Heart Atria. Heart Neoplasms / secondary. Pheochromocytoma / pathology. Vena Cava, Inferior

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  • (PMID = 20802436.001).
  • [ISSN] 0026-4733
  • [Journal-full-title] Minerva chirurgica
  • [ISO-abbreviation] Minerva Chir
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Italy
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56. Lau SK, Weiss LM: The Weiss system for evaluating adrenocortical neoplasms: 25 years later. Hum Pathol; 2009 Jun;40(6):757-68
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  • [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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57. Karakas Z, Tugcu D, Unuvar A, Atay D, Akcay A, Gedik H, Kayserili H, Dogan O, Anak S, Devecioglu O: Li-Fraumeni syndrome in a Turkish family. Pediatr Hematol Oncol; 2010 May;27(4):297-305
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • The adrenocortical carcinoma (ACC) association with acute leukemia is unusual in childhood, even in LFS.
  • The authors here present a family with pR337P mutation in TP53 gene who had a child with acute lymphoblastic leukemia (ALL) and associated adrenocortical carcinoma as a case 1 and his cousin with brain tumor as a case 2.
  • A hereditary TP53 mutation supported the diagnosis of LFS in this family.
  • The availability of a reliable molecular marker to detect the R337P TP53 mutation allows the rapid identification of carriers in families that have a child with ACC.
  • [MeSH-major] Adrenocortical Carcinoma / genetics. Brain Neoplasms / genetics. Li-Fraumeni Syndrome / genetics. Mutation, Missense. Neoplasms, Second Primary / genetics. Precursor Cell Lymphoblastic Leukemia-Lymphoma / genetics. Tumor Suppressor Protein p53 / genetics

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  • (PMID = 20426520.001).
  • [ISSN] 1521-0669
  • [Journal-full-title] Pediatric hematology and oncology
  • [ISO-abbreviation] Pediatr Hematol Oncol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / TP53 protein, human; 0 / Tumor Suppressor Protein p53
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58. Muttarak M, Chotirosniramit A, Unsrisong K, Na Chiangmai W: Adrenal carcinoma. Biomed Imaging Interv J; 2006 Jan;2(1):e9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Adrenal carcinoma.

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  • (PMID = 21614222.001).
  • [ISSN] 1823-5530
  • [Journal-full-title] Biomedical imaging and intervention journal
  • [ISO-abbreviation] Biomed Imaging Interv J
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Malaysia
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59. Mermejo LM, Elias Junior J, Saggioro FP, Tucci Junior S, Castro Md, Moreira AC, Elias PC: Giant adrenal myelolipoma associated with 21-hydroxylase deficiency: unusual association mimicking an androgen-secreting adrenocortical carcinoma. Arq Bras Endocrinol Metabol; 2010 Jun;54(4):419-24
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Giant adrenal myelolipoma associated with 21-hydroxylase deficiency: unusual association mimicking an androgen-secreting adrenocortical carcinoma.
  • The objective of this study was to describe a case of giant myelolipoma associated with undiagnosed congenital adrenal hyperplasia (CAH) due to 21-hydroxylase (21OH) deficiency.
  • Five seven year-old male patient referred with abdominal ultrasound revealing a left adrenal mass.
  • Biochemical investigation revealed hyperandrogenism and imaging exams characterized a large heterogeneous left adrenal mass with interweaving free fat tissue, compatible with the diagnosis of myelolipoma, and a 1.5 cm nodule in the right adrenal gland.
  • Biochemical correlation has brought concerns about differential diagnosis with adrenocortical carcinoma, and surgical excision of the left adrenal mass was indicated.
  • Further investigation resulted in the diagnosis of CAH due to 21OH deficiency.
  • Concluded that CAH has been shown to be associated with adrenocortical tumors.
  • Although rare, myelolipoma associated with CAH should be included in the differential diagnosis of adrenal gland masses.
  • Moreover, CAH should always be ruled out in incidentally detected adrenal masses to avoid unnecessary surgical procedures.
  • [MeSH-major] Adrenal Cortex Neoplasms / diagnosis. Adrenal Hyperplasia, Congenital / diagnosis. Adrenocortical Carcinoma / diagnosis. Myelolipoma / diagnosis
  • [MeSH-minor] Diagnosis, Differential. Humans. Male. Middle Aged. Steroid 21-Hydroxylase / genetics

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  • (PMID = 20625655.001).
  • [ISSN] 1677-9487
  • [Journal-full-title] Arquivos brasileiros de endocrinologia e metabologia
  • [ISO-abbreviation] Arq Bras Endocrinol Metabol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Brazil
  • [Chemical-registry-number] EC 1.14.99.10 / Steroid 21-Hydroxylase
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60. Mitra S, Roy SG, Sur PK: Adrenocortical carcinoma with skeletal metastases in a postmenopausal woman. Indian J Med Paediatr Oncol; 2009 Jan;30(1):24-7
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  • [Title] Adrenocortical carcinoma with skeletal metastases in a postmenopausal woman.
  • Adrenocortical cancer is a very rare tumor with a poor prognosis.
  • CT-guided fine-needle aspiration cytology of an abdominal mass revealed the presence of a carcinoma of the left adrenal cortex.

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  • (PMID = 20668603.001).
  • [ISSN] 0975-2129
  • [Journal-full-title] Indian journal of medical and paediatric oncology : official journal of Indian Society of Medical & Paediatric Oncology
  • [ISO-abbreviation] Indian J Med Paediatr Oncol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] India
  • [Other-IDs] NLM/ PMC2902211
  • [Keywords] NOTNLM ; Adrenocortical carcinoma / androgen secreting tumors / mitotane
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61. Shackelford RE, Veillon DM, Heldmann M, Elmajian DA, Gonzalez E, Cotelingam JD: Pathology case of the month. Thirty-year-old man with a right adrenal mass. Adrenocortical carcinoma with lung and right renal hilar metastases. J La State Med Soc; 2006 Jul-Aug;158(4):172-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Pathology case of the month. Thirty-year-old man with a right adrenal mass. Adrenocortical carcinoma with lung and right renal hilar metastases.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Adrenocortical Carcinoma / pathology. Kidney Neoplasms / secondary. Lung Neoplasms / secondary


62. Else T, Trovato A, Kim AC, Wu Y, Ferguson DO, Kuick RD, Lucas PC, Hammer GD: Genetic p53 deficiency partially rescues the adrenocortical dysplasia phenotype at the expense of increased tumorigenesis. Cancer Cell; 2009 Jun 2;15(6):465-76
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  • [Title] Genetic p53 deficiency partially rescues the adrenocortical dysplasia phenotype at the expense of increased tumorigenesis.
  • In this study, we analyze the adrenocortical dysplasia (acd) mouse, harboring a mutation in Tpp1/Acd.
  • Additional loss of p53 dramatically rescues the acd phenotype in an organ-specific manner, including skin hyperpigmentation and adrenal morphology, but not germ cell atrophy.
  • Tumors from Acd(acd/acd) p53(+/-) mice show a striking switch from the classic spectrum of p53(-/-) mice toward carcinomas.

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  • (PMID = 19477426.001).
  • [ISSN] 1878-3686
  • [Journal-full-title] Cancer cell
  • [ISO-abbreviation] Cancer Cell
  • [Language] ENG
  • [Grant] United States / NIDDK NIH HHS / DK / DK062027-05; United States / NIDDK NIH HHS / DK / R01 DK062027; United States / NIDDK NIH HHS / DK / R01 DK062027-05
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Acd protein, mouse; 0 / Telomere-Binding Proteins; 0 / Tumor Suppressor Protein p53
  • [Other-IDs] NLM/ NIHMS115389; NLM/ PMC2703790
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63. Leboulleux S, Dromain C, Bonniaud G, Aupérin A, Caillou B, Lumbroso J, Sigal R, Baudin E, Schlumberger M: Diagnostic and prognostic value of 18-fluorodeoxyglucose positron emission tomography in adrenocortical carcinoma: a prospective comparison with computed tomography. J Clin Endocrinol Metab; 2006 Mar;91(3):920-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Diagnostic and prognostic value of 18-fluorodeoxyglucose positron emission tomography in adrenocortical carcinoma: a prospective comparison with computed tomography.
  • OBJECTIVE: Patients with adrenocortical cancer are submitted to multiple imaging procedures for diagnosis of recurrence and staging.
  • METHODS: Twenty-eight consecutive patients with adrenocortical cancer referred from November 2003 to December 2004 to the Institut Gustave Roussy were included.
  • The sensitivities for the detection of distinct lesions and the diagnosis of metastatic organs were 90 and 93% for PET/CT and 88 and 82% for TAP-CT, respectively.
  • CONCLUSIONS: We show that FDG-PET/CT is complementary to TAP-CT and of special interest in the diagnosis of local relapses.
  • [MeSH-major] Adrenal Cortex Neoplasms / radiography. Adrenal Cortex Neoplasms / radionuclide imaging. Fluorodeoxyglucose F18

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  • (PMID = 16368753.001).
  • [ISSN] 0021-972X
  • [Journal-full-title] The Journal of clinical endocrinology and metabolism
  • [ISO-abbreviation] J. Clin. Endocrinol. Metab.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; 0Z5B2CJX4D / Fluorodeoxyglucose F18
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64. Palmero EI, Schüler-Faccini L, Caleffi M, Achatz MI, Olivier M, Martel-Planche G, Marcel V, Aguiar E, Giacomazzi J, Ewald IP, Giugliani R, Hainaut P, Ashton-Prolla P: Detection of R337H, a germline TP53 mutation predisposing to multiple cancers, in asymptomatic women participating in a breast cancer screening program in Southern Brazil. Cancer Lett; 2008 Mar 8;261(1):21-5
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  • [Title] Detection of R337H, a germline TP53 mutation predisposing to multiple cancers, in asymptomatic women participating in a breast cancer screening program in Southern Brazil.
  • Germline TP53 mutations predispose to a rare familial cancer syndrome, the Li-Fraumeni Syndrome (LFS), characterized by the early onset of multiple cancers including childhood adrenocortical carcinomas, sarcomas and brain tumors, and breast and colon cancer in young adults.
  • An identical germline mutation at codon 337 in TP53 (R337H) has been shown to be causally related to an increased risk of multiple cancers in unrelated subjects with familial cancer risk in Southern Brazil.
  • Here we have assessed the prevalence of R337H in 750 healthy women participating in a community-based breast cancer screening program in the area of Porto Alegre.
  • The mutant was detected in two participants (0.3%) who were fourth-degree relatives and reported a familial history of cancer at multiple sites that did not match classical criteria for LFS and its variants.
  • Testing in additional family members detected the mutation in three subjects, one of whom developed breast cancer at the age of 36.
  • [MeSH-major] Genes, p53. Genetic Predisposition to Disease. Germ-Line Mutation. Neoplasms / genetics
  • [MeSH-minor] Adult. Aged. Brazil. Breast Neoplasms / diagnosis. Female. Genetic Testing. Humans. Li-Fraumeni Syndrome / genetics. Middle Aged. Pedigree

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  • (PMID = 18248785.001).
  • [ISSN] 0304-3835
  • [Journal-full-title] Cancer letters
  • [ISO-abbreviation] Cancer Lett.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Ireland
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65. Noda M, Ohno S, Nakajin S: Mono-(2-ethylhexyl) phthalate (MEHP) induces nuclear receptor 4A subfamily in NCI-H295R cells: a possible mechanism of aromatase suppression by MEHP. Mol Cell Endocrinol; 2007 Aug 15;274(1-2):8-18
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  • In the present study, we investigated the effects of phthalate esters on aromatase (CYP19) activity and on its gene expression in a human adrenocortical carcinoma cell line, NCI-H295R.
  • [MeSH-minor] Animals. Cell Line, Tumor. Humans. Nuclear Receptor Subfamily 4, Group A, Member 1. Phosphatidylinositol 3-Kinases / metabolism. Promoter Regions, Genetic. Protein Kinase C / metabolism. Signal Transduction / physiology. Transcription, Genetic

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  • (PMID = 17574328.001).
  • [ISSN] 0303-7207
  • [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 / DNA-Binding Proteins; 0 / Endocrine Disruptors; 0 / NR4A1 protein, human; 0 / Nuclear Receptor Subfamily 4, Group A, Member 1; 0 / Plasticizers; 0 / Receptors, Cytoplasmic and Nuclear; 0 / Receptors, Steroid; 0 / Transcription Factors; C42K0PH13C / Diethylhexyl Phthalate; EC 1.14.14.1 / Aromatase; EC 2.7.1.- / Phosphatidylinositol 3-Kinases; EC 2.7.11.13 / Protein Kinase C
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66. Chen CH, Wu HC, Chang CH: An accessory spleen mimics a left adrenal carcinoma. MedGenMed; 2005;7(2):9
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  • [Title] An accessory spleen mimics a left adrenal carcinoma.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Spleen / abnormalities. Spleen / pathology
  • [MeSH-minor] Adult. Diagnosis, Differential. Humans. Male

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  • (PMID = 16369388.001).
  • [ISSN] 1531-0132
  • [Journal-full-title] MedGenMed : Medscape general medicine
  • [ISO-abbreviation] MedGenMed
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Other-IDs] NLM/ PMC1681608
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67. Gross BA, Mindea SA, Pick AJ, Chandler JP, Batjer HH: Medical management of Cushing disease. Neurosurg Focus; 2007;23(3):E10
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Medical management of Cushing disease.
  • Although transsphenoidal excision of the adrenocorticotropic hormone (ACTH)-producing neoplasm is often the treatment of choice in patients with Cushing disease, medical management is itself a useful preoperative temporizing measure, an option for long-term management in nonsurgical candidates, and an option for patients in whom surgery and/or radiotherapy have failed.
  • Three pathophysiologically based approaches exist in the research literature--neuro-modulation to limit ACTH levels, adrenal enzyme inhibition, and glucocorticoid receptor antagonism.
  • Adrenal enzyme inhibitors, however, offer substantial future promise in the management of Cushing disease but are limited by the potential need to use them indefinitely and by dose-tolerance effects.
  • Although etomidate is a potential intravenous alternative for acute cortisol level control, ketoconazole has shown efficacy in the long-term treatment of patients with the disease.
  • If success is still not achieved, the potent adrenolytic agent often used for adrenocortical carcinomas, mitotane, is another alternative.

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  • (PMID = 17961023.001).
  • [ISSN] 1092-0684
  • [Journal-full-title] Neurosurgical focus
  • [ISO-abbreviation] Neurosurg Focus
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Hormonal; 0 / Hormone Antagonists; 0 / Neurotransmitter Agents; 0 / Receptors, Glucocorticoid
  • [Number-of-references] 32
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68. Jiménez Peláez M, Bouvy BM, Dupré GP: Laparoscopic adrenalectomy for treatment of unilateral adrenocortical carcinomas: technique, complications, and results in seven dogs. Vet Surg; 2008 Jul;37(5):444-53
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  • [Title] Laparoscopic adrenalectomy for treatment of unilateral adrenocortical carcinomas: technique, complications, and results in seven dogs.
  • OBJECTIVE: To investigate the feasibility of, and outcome after, laparoscopic adrenalectomy in dogs with unilateral adrenocortical carcinoma.
  • ANIMALS: Dogs (n=7) with Cushing's syndrome caused by unilateral adrenocortical carcinoma.
  • After dissection and hemostatic control of the phrenicoabdominal vein, the adrenal gland was carefully dissected or when there was capsule fragility, necrotic content was partially aspirated.
  • RESULTS: Dogs with unilateral adrenocortical carcinoma (3 right-sided, 4 left-sided) without invasion of the caudal vena cava were successfully operated by laparoscopic approach.
  • CONCLUSIONS: Laparoscopic adrenalectomy is feasible in dogs with either right- or left-sided adrenocortical carcinoma not involving the caudal vena cava.
  • CLINICAL RELEVANCE: When performed by experienced surgeons, laparoscopic adrenalectomy offers a minimally invasive alternative to open laparotomy or retroperitoneal surgery for the treatment of unilateral adrenocortical carcinoma in dogs.
  • [MeSH-major] Adrenal Cortex Neoplasms / veterinary. Adrenalectomy / veterinary. Adrenocortical Carcinoma / veterinary. Adrenocortical Hyperfunction / veterinary. Dog Diseases / surgery

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  • (PMID = 18986311.001).
  • [ISSN] 1532-950X
  • [Journal-full-title] Veterinary surgery : VS
  • [ISO-abbreviation] Vet Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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69. Patel VV, Shah DS, Raychaudhari CR, Patel KB: Giant non-functioning adrenocortical carcinoma: A rare childhood tumor. Indian J Med Paediatr Oncol; 2010 Apr;31(2):65-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Giant non-functioning adrenocortical carcinoma: A rare childhood tumor.
  • Adrenocortical carcinoma (ACC) is a rare malignancy, especially in children.
  • Empty left renal fossa and compensatory enlarged right kidney were seen.
  • With a stage IV disease, the patient died after 2 months from diagnosis.

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  • (PMID = 21209768.001).
  • [ISSN] 0975-2129
  • [Journal-full-title] Indian journal of medical and paediatric oncology : official journal of Indian Society of Medical & Paediatric Oncology
  • [ISO-abbreviation] Indian J Med Paediatr Oncol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] India
  • [Other-IDs] NLM/ PMC2970938
  • [Keywords] NOTNLM ; Adrenocortical carcinoma / adrenocortical tumor / nonfunctioning
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70. Vega Vega A, Canga Presa JM, Sanz de la Morena P, de la Cruz Vigo JL: [Laparoscopic adrenalectomy in adrenal carcinoma]. Actas Urol Esp; 2005 Mar;29(3):277-80
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Laparoscopic adrenalectomy in adrenal carcinoma].
  • There is general agreement on the suitability of the laparoscopic approach for benign adrenal lesions against open procedures because of the efficacy and less morbidity.
  • For suspected adrenal malignancies laparoscopic use is controversial.
  • We report our experience in 6 cases of laparoscopic adrenalectomy in patients with the suspicion of adrenal malignancy confined in the gland.
  • We concluded that in a suspected adrenal malignancy organ confined laparoscopic adrenalectomy presents the advantage compared with open surgery of reduced morbidity and similar results in the follow up of the patient.
  • [MeSH-major] Adrenal Gland Neoplasms / surgery. Adrenalectomy / methods. Laparoscopy

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  • (PMID = 15945253.001).
  • [ISSN] 0210-4806
  • [Journal-full-title] Actas urologicas espanolas
  • [ISO-abbreviation] Actas Urol Esp
  • [Language] spa
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Spain
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71. Kanczkowski W, Zacharowski K, Wirth MP, Ehrhart-Bornstein M, Bornstein SR: Differential expression and action of Toll-like receptors in human adrenocortical cells. Mol Cell Endocrinol; 2009 Mar 5;300(1-2):57-65
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Differential expression and action of Toll-like receptors in human adrenocortical cells.
  • During sepsis, an intact adrenal gland glucocorticoid stress response is critical for survival.
  • However, the exact role which TLRs play in adrenal homeostasis and malfunction is not yet sufficiently known.
  • Using quantitative real-time PCR, confocal microscopy and the NF-kappaB reporter gene assay, we aimed to analyse both, expression and function of all relevant TLRs in the human adrenocortical cell line-NCI-H295R and adrenal cells in primary culture.
  • Our results demonstrate a differential expression pattern of TLR1-9 in human adrenocortical cells as compared to immune cells and adrenocortical cancer cells.
  • Therefore, Toll-like receptors expression and function is a novel feature of the adrenal stress system contributing to adrenal tissue homeostasis, regeneration and tumorigenesis.
  • [MeSH-major] Adrenal Cortex. Gene Expression Regulation. Protein Isoforms / metabolism. Toll-Like Receptors / metabolism

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  • (PMID = 19022344.001).
  • [ISSN] 0303-7207
  • [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 / Interleukin-8; 0 / NF-kappa B; 0 / Protein Isoforms; 0 / Toll-Like Receptors; 0 / Tumor Necrosis Factor-alpha
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72. Erbil Y, Salmaslioğlu A, Barbaros U, Bozbora A, Mete O, Aral F, Ozarmağan S: Clinical and radiological features of adrenal cysts. Urol Int; 2008;80(1):31-6
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  • [Title] Clinical and radiological features of adrenal cysts.
  • Adrenal cysts are very rare lesions, usually asymptomatic or without characteristic symptoms.
  • Although pseudocysts are reported to be the most common clinically recognized adrenal cysts in surgical series, endothelial cysts are more common in autopsy series.
  • We studied 15 consecutive patients with adrenal cysts who underwent surgical resection at our institution from 1990 to 2005.
  • Of 15 patients with adrenal cysts, 10 had pseudocysts, 3 epithelial cysts, 1 an endothelial cyst and 1 a parasitic cyst.
  • In conclusion, a better understanding of cystic adrenal masses is necessary to recognize true adrenal cysts and differentiating them from adrenal carcinoma or adenoma by demonstrating the foci of cystic or degenerative changes.
  • [MeSH-major] Adrenal Gland Diseases / radiography. Adrenal Gland Neoplasms / radiography. Endothelium / pathology. Epithelium / pathology
  • [MeSH-minor] Adult. Aged. Carcinoma / diagnosis. Carcinoma / radiography. Female. Humans. Male. Middle Aged. Retrospective Studies. Tomography, X-Ray Computed

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  • [Copyright] (c) 2008 S. Karger AG, Basel.
  • (PMID = 18204230.001).
  • [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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73. Bakthavathsalam G, Shanmugasundaram VP, Prabakaran J, Venkatesh SP, Sowndaravalli DV, Jain CB: Nonfunctioning adrenocorticalcarcinoma. Int Surg; 2008 Mar-Apr;93(2):81-7
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  • Clinically inapparent adrenal masses detected through imaging for nonadrenal disease, often referred to as adrenal incidentalomas, were first described approximately 20 years ago.
  • Despite the rarity of primary endocrine cancers of the adrenal, adrenal masses are one of the most prevalent of all human tumors.
  • The prevalence of adrenal incidentaloma approaches 3% in middle age and increases to as much as 10% in the elderly.
  • This report describes the case of a 30-year-old man who presented primarily with complaints of deep vein thrombosis of the left leg secondary to a nonfunctioning adrenocortical carcinoma.
  • [MeSH-major] Adrenal Cortex Neoplasms / diagnosis
  • [MeSH-minor] Adult. Humans. Incidental Findings. Male. Venous Thrombosis / diagnosis

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  • (PMID = 18998286.001).
  • [ISSN] 0020-8868
  • [Journal-full-title] International surgery
  • [ISO-abbreviation] Int Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Italy
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74. Delaney HM, Prauner RD, Person DA: Germline p53 mutation in a Micronesian child with adrenocortical carcinoma and subsequent osteosarcoma. J Pediatr Hematol Oncol; 2008 Nov;30(11):803-6
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  • [Title] Germline p53 mutation in a Micronesian child with adrenocortical carcinoma and subsequent osteosarcoma.
  • In 1990, an 18-month-old Micronesian girl was initially diagnosed with a right adrenocortical carcinoma.
  • Given this child's remarkable history of malignancy, she underwent testing for a genetic mutation that is associated with increased cancer formation.
  • One such cancer syndrome is called Li-Fraumeni syndrome where approximately 70% of patients carry a genetic mutation in the p53 tumor suppressor gene.
  • Patients with LFS are at risk for developing cancers of the breast, soft tissues, brain, bone, adrenal gland, and blood cells.
  • Mutational analysis of our patient did reveal the presence of a germline mutation of the p53 tumor suppressor gene.
  • She was found to have a base pair change (A-->C) at nucleotide 394 resulting in a lysine to glutamine amino acid change at codon 132 (K132Q), which remarkably has never been described in association with either adrenocortical carcinoma or osteosarcoma.
  • [MeSH-major] Adrenal Cortex Neoplasms / genetics. Adrenocortical Carcinoma / genetics. Bone Neoplasms / genetics. Germ-Line Mutation / genetics. Neoplasms, Second Primary / genetics. Osteosarcoma / genetics. Tumor Suppressor Protein p53 / genetics


75. Else T, Giordano TJ, Hammer GD: Evaluation of telomere length maintenance mechanisms in adrenocortical carcinoma. J Clin Endocrinol Metab; 2008 Apr;93(4):1442-9
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  • [Title] Evaluation of telomere length maintenance mechanisms in adrenocortical carcinoma.
  • CONTEXT: Adrenocortical cancer (ACC) is a rare disease with an often fatal outcome.
  • The clinical and pathological diagnosis of a malignant vs. benign adrenocortical tumor is sometimes challenging.
  • Telomere maintenance mechanisms (TMMs) are critical for the persistence of the malignant phenotype, but little is known about these mechanisms or their diagnostic value in adrenocortical lesions.
  • OBJECTIVE: Tissue samples of diagnostically known adrenocortical neoplasms were evaluated for parameters of known TMMs, telomerase activity (TA), and alternative telomere lengthening (ALT).
  • DESIGN: The study analyzed retrospectively collected frozen adrenocortical tissue samples from the University of Michigan Health System.
  • PATIENT SAMPLES: Samples included 24 ACCs, 11 adrenocortical adenomas (ACAs), and three normal adrenal tissues.
  • None of the normal adrenal tissues (none of three) or ACA (none of 11) samples had signs of an active TMM.
  • Determination of telomere maintenance mechanisms in diagnostically challenging adrenocortical tumors might be of additional diagnostic value in the pathological diagnosis of malignant vs. benign lesions.
  • [MeSH-major] Adrenal Cortex Neoplasms / genetics. Telomere

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  • (PMID = 18198226.001).
  • [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] EC 2.7.7.49 / Telomerase
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76. West AN, Neale GA, Pounds S, Figueredo BC, Rodriguez Galindo C, Pianovski MA, Oliveira Filho AG, Malkin D, Lalli E, Ribeiro R, Zambetti GP: Gene expression profiling of childhood adrenocortical tumors. Cancer Res; 2007 Jan 15;67(2):600-8
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  • [Title] Gene expression profiling of childhood adrenocortical tumors.
  • Pediatric adrenocortical tumors (ACT) are rare and often fatal malignancies; little is known regarding their etiology and biology.
  • To provide additional insight into the nature of ACT, we determined the gene expression profiles of 24 pediatric tumors (five adenomas, 18 carcinomas, and one undetermined) and seven normal adrenal glands.
  • Distinct patterns of gene expression, validated by quantitative real-time PCR and Western blot analysis, were identified that distinguish normal adrenal cortex from tumor.
  • Differences in gene expression were also identified between adrenocortical adenomas and carcinomas.
  • In addition, pediatric adrenocortical carcinomas were found to share similar patterns of gene expression when compared with those published for adult ACT.
  • Our findings lay the groundwork for establishing gene expression profiles that may aid in the diagnosis and prognosis of pediatric ACT, and in the identification of signaling pathways that contribute to this disease.
  • [MeSH-major] Adrenal Cortex Neoplasms / genetics. Adrenocortical Adenoma / genetics. Adrenocortical Carcinoma / genetics

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  • (PMID = 17234769.001).
  • [ISSN] 0008-5472
  • [Journal-full-title] Cancer research
  • [ISO-abbreviation] Cancer Res.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA21765; United States / NCI NIH HHS / CA / CA63230; United States / NCI NIH HHS / CA / CA71907
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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77. Heye S, Woestenborghs H, Van Kerkhove F, Oyen R: Adrenocortical carcinoma with fat inclusion: case report. Abdom Imaging; 2005 Sep-Oct;30(5):641-3
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  • [Title] Adrenocortical carcinoma with fat inclusion: case report.
  • Adrenocortical carcinoma is a rare tumor that arises from the adrenal cortex, with an estimated incidence of 0.5% to 2% per 1 million patients yearly.
  • Although some fat content can be expected in hormonally active adrenocortical carcinomas, areas of 100% fat are extremely rare.
  • We present a case of an adrenocortical carcinoma with a small focus of pure fat depicted on magnetic resonance imaging.
  • [MeSH-major] Adipose Tissue / pathology. Adrenal Cortex Neoplasms / diagnosis. Adrenocortical Carcinoma / diagnosis. Magnetic Resonance Imaging

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  • (PMID = 15688105.001).
  • [ISSN] 0942-8925
  • [Journal-full-title] Abdominal imaging
  • [ISO-abbreviation] Abdom Imaging
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Contrast Media
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78. Owecki M, Baszko-Błaszyk D, Waśko R, Sowiński J: [Non-small cell lung cancer presenting under the mask of a primary adrenal cancer--case report]. Pol Merkur Lekarski; 2005 Feb;18(104):216-8
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  • [Title] [Non-small cell lung cancer presenting under the mask of a primary adrenal cancer--case report].
  • [Transliterated title] Niedrobnokomórkowy rak płuca przebiegajacy pod maska pierwotnego raka nadnercza--opis przypadku.
  • Adrenal carcinoma is a malignant disease that often results in distant metastases to different organs, including the lungs.
  • While diagnosing patients with suspected adrenal carcinoma, metastases to the lungs should always be considered.
  • The opposite clinical situation also should be considered, i.e. lung cancer metastases to the adrenal gland.
  • Both conditions may have a very similar course and their differential diagnosis may be sometimes very difficult.
  • However, proper diagnosis is of great importance because both diseases are treated by different means.
  • We present a case of a 50-year-old female patient with a small primary focus of non-small cell lung carcinoma and its large metastasis to the left adrenal gland, accompanied by SIADH and paraneoplastic hypercalcemia.
  • In the presented case adrenal carcinoma and its lung metastasis were primarily misdiagnosed which led to unnecessary laparotomy.
  • The proper diagnosis was enabled by CT guided biopsy of the lung tumor.
  • [MeSH-major] Adrenal Gland Neoplasms / radiography. Adrenal Gland Neoplasms / secondary. Carcinoma, Non-Small-Cell Lung / radiography. Carcinoma, Non-Small-Cell Lung / secondary. Diagnostic Errors. Lung Neoplasms / radiography
  • [MeSH-minor] Adrenalectomy. Biopsy, Needle / methods. Combined Modality Therapy. Diagnosis, Differential. Female. Humans. Laparoscopy. Middle Aged. Palliative Care. Tomography, X-Ray Computed. Unnecessary Procedures


79. Brix D, Allolio B, Fenske W, Agha A, Dralle H, Jurowich C, Langer P, Mussack T, Nies C, Riedmiller H, Spahn M, Weismann D, Hahner S, Fassnacht M, German Adrenocortical Carcinoma Registry Group: Laparoscopic versus open adrenalectomy for adrenocortical carcinoma: surgical and oncologic outcome in 152 patients. Eur Urol; 2010 Oct;58(4):609-15
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  • [Title] Laparoscopic versus open adrenalectomy for adrenocortical carcinoma: surgical and oncologic outcome in 152 patients.
  • BACKGROUND: The role of laparoscopic adrenalectomy in the treatment of patients with adrenocortical carcinoma (ACC) is controversial.
  • OBJECTIVE: Our aim was to compare oncologic outcome in patients with ACC who underwent either open adrenalectomy (OA) or laparoscopic adrenalectomy (LA) for localised disease.
  • INTERVENTION: Patients were stratified into two groups according to the surgical procedure (LA or OA).
  • For comparison, we used both a matched pairs approach by selecting for each patient from the LA group (n=35) one corresponding patient from the OA group (n=117) and multivariate analysis in all 152 patients.
  • MEASUREMENTS: Disease-specific survival was chosen as the predefined primary end point.
  • Secondary end points were recurrence-free survival, frequency of tumour capsule violation and postoperative peritoneal carcinomatosis, and incidence and reasons for conversion from LA to OA.
  • RESULTS AND LIMITATIONS: LA and OA did not differ with regard to the primary end point using either the matched pairs approach (hazard ratio [HR] for death: 0.79; 95% confidence interval [CI], 0.36-1.72; p=0.55) or multivariate analysis (HR for death: 0.98; 95% CI, 0.51-1.92; p=0.92).
  • Similarly, adjusted recurrence-free survival was not different between LA and OA (HR: 0.91; 95% CI, 0.56-1.47; p=0.69).
  • In 12 of 35 patients of the LA group, surgery was converted to open surgery with no impact on the clinical outcome.
  • CONCLUSIONS: For localised ACC with a diameter of < or =10 cm, LA by an experienced surgeon is not inferior to OA with regard to oncologic outcome.
  • [MeSH-major] Adrenal Cortex Neoplasms / surgery. Adrenalectomy / methods. Adrenocortical Carcinoma / surgery. Laparoscopy

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  • [Copyright] Copyright 2010 European Association of Urology. Published by Elsevier B.V. All rights reserved.
  • [CommentIn] Eur Urol. 2010 Dec;58(6):e53; author reply e54 [20864252.001]
  • (PMID = 20580485.001).
  • [ISSN] 1873-7560
  • [Journal-full-title] European urology
  • [ISO-abbreviation] Eur. Urol.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Switzerland
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80. Daffara F, De Francia S, Reimondo G, Zaggia B, Aroasio E, Porpiglia F, Volante M, Termine A, Di Carlo F, Dogliotti L, Angeli A, Berruti A, Terzolo M: Prospective evaluation of mitotane toxicity in adrenocortical cancer patients treated adjuvantly. Endocr Relat Cancer; 2008 Dec;15(4):1043-53
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  • [Title] Prospective evaluation of mitotane toxicity in adrenocortical cancer patients treated adjuvantly.
  • Seventeen consecutive patients who were treated with mitotane after radical resection of adrenocortical cancer (ACC) from 1999 to 2005 underwent physical examination, routine laboratory evaluation, monitoring of mitotane concentrations, and a hormonal work-up at baseline and every 3 months till ACC relapse or study end (December 2007).
  • Mitotane affected adrenal steroidogenesis with a more remarkable inhibition of cortisol and DHEAS than aldosterone.
  • [MeSH-major] Adrenal Cortex Neoplasms / drug therapy. Antineoplastic Agents, Hormonal / therapeutic use. Mitotane / therapeutic use

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  • (PMID = 18824557.001).
  • [ISSN] 1351-0088
  • [Journal-full-title] Endocrine-related cancer
  • [ISO-abbreviation] Endocr. Relat. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Hormonal; 3XMK78S47O / Testosterone; 78E4J5IB5J / Mitotane; WI4X0X7BPJ / Hydrocortisone
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81. Polat B, Fassnacht M, Pfreundner L, Guckenberger M, Bratengeier K, Johanssen S, Kenn W, Hahner S, Allolio B, Flentje M: Radiotherapy in adrenocortical carcinoma. Cancer; 2009 Jul 1;115(13):2816-23
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Radiotherapy in adrenocortical carcinoma.
  • Adrenocortical carcinoma (ACC) is a rare malignancy, and patients with ACC have a poor prognosis.
  • Even after radical surgery, up to 85% of patients develop recurrent disease.
  • Ten articles were identified that covered radiotherapy in a total of 129 patients with ACC (64 patients received postoperative irradiation, and 65 patients received palliative therapy for advanced disease).
  • In those with advanced disease, a response to radiotherapy was observed in 57% of patients who received palliative radiotherapy.
  • [MeSH-major] Adrenal Cortex Neoplasms / radiotherapy. Adrenocortical Carcinoma / radiotherapy

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  • (PMID = 19402169.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Evaluation Studies; Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Number-of-references] 42
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82. Abiven-Lepage G, Coste J, Tissier F, Groussin L, Billaud L, Dousset B, Goffinet F, Bertagna X, Bertherat J, Raffin-Sanson ML: Adrenocortical carcinoma and pregnancy: clinical and biological features and prognosis. Eur J Endocrinol; 2010 Nov;163(5):793-800
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  • [Title] Adrenocortical carcinoma and pregnancy: clinical and biological features and prognosis.
  • OBJECTIVE: Adrenocortical carcinoma (ACC) is a rare, severe disease.
  • For the survival analysis, pregnant patients were compared with a subgroup of nonpregnant women matched for age, stage, and year of diagnosis (1 pregnant patient/2 controls).
  • RESULTS: Adrenocortical tumors diagnosed during pregnancy or in the postpartum period tend to be more often cortisol-secreting tumors (P=0.06) and to be discovered at a more advanced stage than those in nonpregnant women, although the differences were not significant.

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  • (PMID = 20699382.001).
  • [ISSN] 1479-683X
  • [Journal-full-title] European journal of endocrinology
  • [ISO-abbreviation] Eur. J. Endocrinol.
  • [Language] ENG
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
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83. Louiset E, Isvi K, Gasc JM, Duparc C, Cauliez B, Laquerrière A, Kuhn JM, Lefebvre H: Ectopic expression of serotonin7 receptors in an adrenocortical carcinoma co-secreting renin and cortisol. Endocr Relat Cancer; 2008 Dec;15(4):1025-34
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  • [Title] Ectopic expression of serotonin7 receptors in an adrenocortical carcinoma co-secreting renin and cortisol.
  • Abnormal expression of membrane receptors has been previously described in benign adrenocortical neoplasms causing Cushing's syndrome.
  • In particular, we have observed that, in some adreno corticotropic hormone (ACTH)-independent macronodular adrenal hyperplasia tissues, cortisol secretion is controlled by ectopic serotonin(7) (5-HT(7)) receptors.
  • The objective of the present study was to investigate in vitro the effect of serotonin (5-hydroxy tryptamine; 5-HT) on cortisol and renin production by a left adrenocortical carcinoma removed from a 48-year-old female patient with severe Cushing's syndrome and elevated plasma renin levels.
  • Tumor explants were obtained at surgery and processed for immunohistochemistry, in situ hybridization and cell culture studies.
  • In addition, immunohistochemistry showed the occurrence of 5-HT(7) receptor-like immunoreactivity in carcinoma cells. mRNAs encoding renin as well as renin-like immunoreactivity were detected in endothelial and tumor cells.
  • Cell incubation studies revealed that the adrenocortical tissue also released renin.
  • 5-HT(7) receptors, in an adrenocortical carcinoma.
  • Our results also indicate that 5-HT can influence the secretory activity of malignant adrenocortical tumors in an autocrine/paracrine manner.
  • The effects of 5-HT on adrenocortical tumor cells included a paradoxical inhibitory action on renin production and a stimulatory action on cortisol secretion involving 5-HT(7) receptors.
  • [MeSH-major] Adrenal Cortex Neoplasms / metabolism. Adrenocortical Carcinoma / metabolism. Hydrocortisone / metabolism. Receptors, Serotonin / metabolism. Renin / metabolism

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  • (PMID = 18708508.001).
  • [ISSN] 1351-0088
  • [Journal-full-title] Endocrine-related cancer
  • [ISO-abbreviation] Endocr. Relat. Cancer
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Hormones; 0 / Phenols; 0 / Receptors, Serotonin; 0 / SB 269970; 0 / Sulfonamides; 0 / Vasoconstrictor Agents; 0 / serotonin 7 receptor; 11128-99-7 / Angiotensin II; 333DO1RDJY / Serotonin; 9002-60-2 / Adrenocorticotropic Hormone; EC 3.4.23.15 / Renin; WI4X0X7BPJ / Hydrocortisone
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84. Shimony A, Bereza S, Shalev A, Gilutz H, Ilia R, Zahger D: Ventricular fibrillation as the presenting manifestation of adrenocortical carcinoma. Am Heart Hosp J; 2009;7(1):65-6
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  • [Title] Ventricular fibrillation as the presenting manifestation of adrenocortical carcinoma.
  • We describe a case of a young adult in whom sudden cardiac death due to ventricular fibrillation was the presenting manifestation of an adrenocortical carcinoma.
  • Sudden death has not been previously described as a manifestation of this adrenal neoplasm.
  • Unexplained persistent hypokalemia after resuscitated sudden death (especially when combined with hypertension( should prompt investigation for an underlying secondary hypertension, particularly adrenal pathology.
  • Adrenocortical carcinoma should be considered in the differential diagnosis of unexplained sudden death associated with unexplained hypokalemia.
  • [MeSH-major] Adrenal Cortex Neoplasms / complications. Adrenocortical Carcinoma / complications. Ventricular Fibrillation / etiology

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  • (PMID = 19742438.001).
  • [ISSN] 1751-7168
  • [Journal-full-title] The American heart hospital journal
  • [ISO-abbreviation] Am Heart Hosp J
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
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85. Park BK, Kim CK, Jung BC, Suh YL: Cortical adenoma in adrenohepatic fusion tissue: clue to making a correct diagnosis at preoperative computed tomography examination. Eur Urol; 2009 Dec;56(6):1082-5
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  • [Title] Cortical adenoma in adrenohepatic fusion tissue: clue to making a correct diagnosis at preoperative computed tomography examination.
  • Preoperative differential diagnoses included primary or secondary malignant hepatic tumors or adrenal cortical carcinoma due to aggressive imaging features.
  • The tumor proved to be an adrenal cortical adenoma arising from the adrenohepatic fusion tissue and consisted of adenoma cells with lipid-rich cytoplasm.
  • Retrospective review of preoperative computed tomography (CT) images demonstrated that the tumor measured 6 Hounsfield units in mean CT number and was continuous with a medial limb of the right adrenal gland.
  • [MeSH-major] Adrenal Cortex Neoplasms / radiography. Adrenocortical Adenoma / radiography. Liver Neoplasms / radiography. Tomography, X-Ray Computed
  • [MeSH-minor] Diagnosis, Differential. Female. Humans. Middle Aged. Preoperative Care. Ultrasonography

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  • (PMID = 19447543.001).
  • [ISSN] 1873-7560
  • [Journal-full-title] European urology
  • [ISO-abbreviation] Eur. Urol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Switzerland
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86. Ochi T, Tanji N, Shimamoto K, Ikeda T, Toshino A, Yokoyama M: Application of cardiopulmonary bypass for resection of renal cell carcinoma and adrenocortical carcinoma extending into the right atrium. Int J Urol; 2006 Mar;13(3):202-5
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  • [Title] Application of cardiopulmonary bypass for resection of renal cell carcinoma and adrenocortical carcinoma extending into the right atrium.
  • AIM: The application of cardiopulmonary bypass to atrial involvement represents an important advance that has improved the safety and technical efficacy of a difficult surgical undertaking.
  • Tumors originated in the right kidney in four patients, and in left adrenal gland in one patient.
  • Of the five patients, three died of metastatic diseases, one died of liver dysfunction and one remains disease free as of 18 months postoperatively.
  • [MeSH-major] Adrenal Cortex Neoplasms / surgery. Adrenocortical Carcinoma / surgery. Carcinoma, Renal Cell / surgery. Cardiac Surgical Procedures / methods. Cardiopulmonary Bypass / methods. Heart Neoplasms / surgery. Kidney Neoplasms / surgery


87. Soon PS, McDonald KL, Robinson BG, Sidhu SB: Molecular markers and the pathogenesis of adrenocortical cancer. Oncologist; 2008 May;13(5):548-61
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  • [Title] Molecular markers and the pathogenesis of adrenocortical cancer.
  • Adrenal tumors are common, with an estimated incidence of 7.3% in autopsy cases, while adrenocortical carcinomas (ACCs) are rare, with an estimated prevalence of 4-12 per million population.
  • Because the prognoses for adrenocortical adenomas (ACAs) and ACCs are vastly different, it is important to be able to accurately differentiate the two tumor types.
  • Advancement in the understanding of the pathophysiology of ACCs is essential for the development of more sensitive means of diagnosis and treatment, resulting in better clinical outcome.
  • Adrenocortical tumors (ACTs) occur as a component of several hereditary tumor syndromes, which include the Li-Fraumeni syndrome, Beckwith-Wiedemann syndrome, multiple endocrine neoplasia 1, Carney complex, and congenital adrenal hyperplasia.
  • The adrenocorticotropic hormone-cAMP-protein kinase A and Wnt pathways are also implicated in adrenocortical tumorigenesis.
  • The aim of this review is to summarize the current knowledge on the molecular mechanisms involved in adrenocortical tumorigenesis, including results of comparative genomic hybridization, loss of heterozygosity, and microarray gene-expression profiling studies.
  • [MeSH-major] Adrenal Cortex Neoplasms / genetics. Adrenocortical Adenoma / genetics. Adrenocortical Carcinoma / genetics. Neoplastic Syndromes, Hereditary / genetics

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  • (PMID = 18515740.001).
  • [ISSN] 1083-7159
  • [Journal-full-title] The oncologist
  • [ISO-abbreviation] Oncologist
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 9002-60-2 / Adrenocorticotropic Hormone
  • [Number-of-references] 135
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88. Crand A, Borson-Chazot F, Brue T: [Recent data in adrenocortical tumorigenesis]. Ann Endocrinol (Paris); 2009 Sep;70 Suppl 1:S20-5
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  • [Title] [Recent data in adrenocortical tumorigenesis].
  • [Transliterated title] Actualités dans la tumorigenèse surrénalienne.
  • Adrenocortical carcinomas are rare tumors characterized by an aggressive behaviour with a 5-year survival rate below 30%.
  • Until now, surgery is the only curative treatment for tumors confined to the adrenal gland and there is a lack of an effective medical treatment for invasive or metastatic tumors due to the poor knowledge of the mechanisms underlying adrenocortical malignancy.
  • Moreover, histopathology is sometimes insufficient to establish an accurate diagnosis between a benign and a malignant adrenal tumor and a poor indicator of prognosis.
  • In the last decade, the study of rare genetic syndromes associated with adrenocortical carcinomas and the identification of genetic alterations in adrenal tumors has improved our understanding of the pathogenesis of adrenal tumors.
  • The development of molecular predictors of malignancy and of survival could help for histological diagnosis and determination of prognosis.
  • These significant advances are essential to improve adrenocortical carcinoma management.
  • This review summarizes recent advances in the understanding and management of adrenocortical tumors.
  • [MeSH-major] Adrenal Cortex Neoplasms / etiology

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  • (PMID = 19878765.001).
  • [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 / Biomarkers, Tumor
  • [Number-of-references] 34
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89. Li XB, Schluesener HJ: Therapeutic effects of cisplatin on rat experimental autoimmune encephalomyelitis. Arch Immunol Ther Exp (Warsz); 2006 Jan-Feb;54(1):51-3
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  • INTRODUCTION: Experimental autoimmune encephalomyelitis (EAE) is a prototypic Th1-mediated autoimmune inflammatory disease of the central nervous system (CNS), and serves as a model for the human demyelinating disease, multiple sclerosis.
  • Cisplatin is a drug widely used in the treatment of a variety of human neoplasias, such as advanced bladder carcinoma, adrenal cortex carcinoma, breast cancer, head and neck or lung carcinoma.
  • Cisplatin binds to DNA and interferes with cellular repair and other mechanism, which eventually result into cell death.
  • It is known that cisplatin can induce immunosuppressive effects through inhibition of T cell activity.
  • Immunohistochemistry was applied to show immune cell infiltration into the CNS.
  • RESULTS: Early treatment of EAE rats with cisplatin effectively ameliorated the development of disease and provided a significant protective effect compared to control rats.
  • Further, histological analysis demonstrated that the formation of the typical perivascular cuffs and brain infiltration of monocytes and lymphocytes were complete absent in cisplatin treated rats, while abundant T cell infiltration was seen in the CNS of EAE rats.

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  • (PMID = 16642257.001).
  • [ISSN] 0004-069X
  • [Journal-full-title] Archivum immunologiae et therapiae experimentalis
  • [ISO-abbreviation] Arch. Immunol. Ther. Exp. (Warsz.)
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / Immunosuppressive Agents; 0 / Myelin Basic Protein; Q20Q21Q62J / Cisplatin
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90. Cazejust J, De Baère T, Auperin A, Deschamps F, Hechelhammer L, Abdel-Rehim M, Schlumberger M, Leboulleux S, Baudin E: Transcatheter arterial chemoembolization for liver metastases in patients with adrenocortical carcinoma. J Vasc Interv Radiol; 2010 Oct;21(10):1527-32
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  • [Title] Transcatheter arterial chemoembolization for liver metastases in patients with adrenocortical carcinoma.
  • PURPOSE: To retrospectively evaluate the effectiveness, tolerance, and predictors of response to transcatheter arterial chemoembolization for treatment of liver metastases from adrenocortical carcinoma.
  • MATERIALS AND METHODS: Twenty-nine patients with progressive liver metastases from adrenocortical carcinoma were treated with transcatheter arterial chemoembolization.
  • RESULTS: Three months after transcatheter arterial chemoembolization, a liver morphologic response was observed in six of 29 patients (21%), stabilization in 18 (62%), and progression in five (17%).
  • According to per-lesion analysis (n = 103), a morphologic response was observed in 23 lesions (22%), stabilization in 67 (65%), and progression in 13 (13%).
  • CONCLUSIONS: Transcatheter arterial chemoembolization should be considered as part of the therapeutic arsenal to treat liver metastases from adrenocortical carcinoma.
  • [MeSH-major] Adrenal Cortex Neoplasms / drug therapy. Adrenocortical Carcinoma / drug therapy. Adrenocortical Carcinoma / secondary. Hemostatics / therapeutic use. Liver Neoplasms / drug therapy. Liver Neoplasms / secondary

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  • [Copyright] Copyright © 2010 SIR. Published by Elsevier Inc. All rights reserved.
  • (PMID = 20801688.001).
  • [ISSN] 1535-7732
  • [Journal-full-title] Journal of vascular and interventional radiology : JVIR
  • [ISO-abbreviation] J Vasc Interv Radiol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Hemostatics
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91. Oller AR, Kirkpatrick DT, Radovsky A, Bates HK: Inhalation carcinogenicity study with nickel metal powder in Wistar rats. Toxicol Appl Pharmacol; 2008 Dec 1;233(2):262-75
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  • Epidemiological studies of nickel refinery workers have demonstrated an association between increased respiratory cancer risk and exposure to certain nickel compounds (later confirmed in animal studies).
  • Adrenal gland pheochromocytomas (benign and malignant) in males and combined cortical adenomas/carcinomas in females were induced in a dose-dependent manner by the nickel metal exposure.
  • Pheochromocytomas appear to be secondary to the lung toxicity associated with the exposure rather than being related to a direct nickel effect on the adrenal glands.
  • The incidence of cortical tumors among 0.4 mg Ni/m(3) females, although statistically higher compared to the concurrent controls, falls within the historical control range; therefore, in the present study, this tumor is of uncertain relationship to nickel metal exposure.
  • [MeSH-minor] Adrenal Cortex Neoplasms / chemically induced. Adrenal Gland Neoplasms / chemically induced. Adrenocortical Adenoma / chemically induced. Adrenocortical Carcinoma / chemically induced. Animals. Dose-Response Relationship, Drug. Female. Male. Models, Animal. Occupational Exposure / adverse effects. Pheochromocytoma / chemically induced. Powders. Rats. Rats, Wistar. Respiratory Tract Neoplasms / epidemiology. Respiratory Tract Neoplasms / etiology. Sex Factors

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  • (PMID = 18822311.001).
  • [ISSN] 1096-0333
  • [Journal-full-title] Toxicology and applied pharmacology
  • [ISO-abbreviation] Toxicol. Appl. Pharmacol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Carcinogens; 0 / Powders; 7OV03QG267 / Nickel
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92. Higley EB, Newsted JL, Zhang X, Giesy JP, Hecker M: Assessment of chemical effects on aromatase activity using the H295R cell line. Environ Sci Pollut Res Int; 2010 Jun;17(5):1137-48
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  • [Title] Assessment of chemical effects on aromatase activity using the H295R cell line.
  • Here we compare direct, indirect, and combined measurements of AA using the H295R cell line after exposure to seven model chemicals.
  • RECOMMENDATIONS AND PERSPECTIVES: For future work with the H295R, it is recommended that a combination of direct and indirect aromatase measurements is used because it was best in predicting the effects of a chemical on E2 production and its mechanism of action.
  • [MeSH-minor] Adrenal Cortex / enzymology. Adrenocortical Carcinoma / enzymology. Cell Line, Tumor. Ecotoxicology / methods. Enzyme-Linked Immunosorbent Assay. Estradiol / metabolism. Fungicides, Industrial / chemistry. Fungicides, Industrial / toxicity. Herbicides / chemistry. Herbicides / toxicity. Humans. Risk Assessment. Testosterone / metabolism. Time Factors

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  • (PMID = 20087668.001).
  • [ISSN] 1614-7499
  • [Journal-full-title] Environmental science and pollution research international
  • [ISO-abbreviation] Environ Sci Pollut Res Int
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, Non-P.H.S.
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Aromatase Inhibitors; 0 / Endocrine Disruptors; 0 / Environmental Pollutants; 0 / Fungicides, Industrial; 0 / Herbicides; 3XMK78S47O / Testosterone; 4TI98Z838E / Estradiol; EC 1.14.14.1 / Aromatase
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93. Ribeiro RC, Pinto EM, Zambetti GP: Familial predisposition to adrenocortical tumors: clinical and biological features and management strategies. Best Pract Res Clin Endocrinol Metab; 2010 Jun;24(3):477-90
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  • [Title] Familial predisposition to adrenocortical tumors: clinical and biological features and management strategies.
  • The incidence of adrenocortical tumors (ACTs) is increased in several familial cancer syndromes resulting from abnormalities in genes that encode transcription factors implicated in cell proliferation, differentiation, senescence, apoptosis, and genomic instability.
  • Adenomas are the most common ACTs, but adrenocortical carcinomas occur rarely as well.
  • The clinical manifestations of ACTs, which result from increased secretion of adrenocortical hormones, are similar in the familial and sporadic forms of the disease.
  • The analysis of gene expression profiles of ACTs in these constitutional syndromes have contributed to our understanding of adrenal tumorigenesis and revealed new molecular diagnostic and prognostic markers and candidate genes for targeted therapies.
  • This chapter summarizes the clinical and biological features, pathogenesis, and management strategies for ACTs that develop in patients with familial cancer syndrome.
  • [MeSH-major] Adrenal Cortex Neoplasms / genetics. Adrenocortical Carcinoma / genetics. Li-Fraumeni Syndrome / genetics
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Female. Genes, p53. Genetic Predisposition to Disease. Humans. Male

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  • [Copyright] Copyright 2010. Published by Elsevier Ltd.
  • (PMID = 20833338.001).
  • [ISSN] 1878-1594
  • [Journal-full-title] Best practice & research. Clinical endocrinology & metabolism
  • [ISO-abbreviation] Best Pract. Res. Clin. Endocrinol. Metab.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA-21765
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] Netherlands
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94. Attivi D, Ajana I, Astier A, Demoré B, Gibaud S: Development of microemulsion of mitotane for improvement of oral bioavailability. Drug Dev Ind Pharm; 2010 Apr;36(4):421-7
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  • BACKGROUND: Mitotane (o,p'-DDD) is considered to be the drug of choice in the treatment of nonresectable and metastasized adrenocortical carcinoma.
  • RESULTS: The optimum formulation consisted of a mixture of Capryol, Tween, and Cremophor EL (33:33:33).
  • [MeSH-minor] Administration, Oral. Adrenocortical Carcinoma / drug therapy. Animals. Antineoplastic Agents, Hormonal / administration & dosage. Antineoplastic Agents, Hormonal / pharmacokinetics. Biological Availability. Drug Compounding / methods. Emulsions. Humans. Intestinal Absorption. Jejunum / metabolism. Male. Particle Size. Rabbits. Rats. Rats, Wistar. Solubility. Surface-Active Agents / chemistry

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  • (PMID = 19778161.001).
  • [ISSN] 1520-5762
  • [Journal-full-title] Drug development and industrial pharmacy
  • [ISO-abbreviation] Drug Dev Ind Pharm
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Hormonal; 0 / Emulsions; 0 / Surface-Active Agents; 78E4J5IB5J / Mitotane
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95. Schlamp A, Hallfeldt K, Mueller-Lisse U, Pfluger T, Reincke M: Recurrent adrenocortical carcinoma after laparoscopic resection. Nat Clin Pract Endocrinol Metab; 2007 Feb;3(2):191-5; quiz 1 p following 195
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  • [Title] Recurrent adrenocortical carcinoma after laparoscopic resection.
  • A left adrenal mass of 6.5 cm by 7.5 cm was detected by a CT scan.
  • The patient underwent laparoscopic surgery to remove the tumor mass; histologic work-up revealed an adrenocortical carcinoma.
  • INVESTIGATIONS: In our department, laboratory work-up for endocrine activity was performed, as well as CT scans of the adrenal region, and FDG-PET scans in order to determine the extension of disease.
  • DIAGNOSIS: Recurrent adrenocortical carcinoma after laparoscopic adrenalectomy.
  • The patient is now scheduled for polychemotherapy because of progressive metastatic disease revealed by follow-up CT and FDG-PET scanning in June 2006.
  • [MeSH-major] Adrenal Cortex Neoplasms / radionuclide imaging. Adrenocortical Carcinoma / radionuclide imaging. Neoplasm Recurrence, Local / radionuclide imaging

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  • (PMID = 17237845.001).
  • [ISSN] 1745-8366
  • [Journal-full-title] Nature clinical practice. Endocrinology & metabolism
  • [ISO-abbreviation] Nat Clin Pract Endocrinol Metab
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; 0Z5B2CJX4D / Fluorodeoxyglucose F18
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96. Castillo O, Sánchez-Salas R, Vidal I: Laparoscopic adrenalectomy. Minerva Urol Nefrol; 2008 Sep;60(3):177-84
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  • Laparoscopic adrenalectomy (LA) is the gold standard for the surgical management of small and medium adrenal masses.
  • Nevertheless, there is still controversy for the laparoscopic treatment of adrenal carcinoma.
  • The aim of this article was to report current standards on LA.
  • Even when available evidence in the literature is low for LA, it has become the standard of treatment for adrenal masses especially in benign lesions.
  • The most employed surgical technique for LA is the lateral transabdominal, but novel approaches have been developed to treat surgically adrenal diseases and an objective evaluation of outcomes is awaited.
  • Laparoscopic treatment of adrenal primary malignancy and metastases is still controversial although clear indications for laparoscopy in these cases are bounded to surgical experience.
  • LA has definitively replaced open surgery in the surgical management of adrenal tumors < or = 12 cm, because of its advantages in terms of morbidity and recovery.
  • Large and malignant tumors should be carefully approached by experienced laparoscopic surgeons.
  • [MeSH-minor] Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / surgery. Humans. Postoperative Complications / etiology

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  • (PMID = 18787512.001).
  • [ISSN] 0393-2249
  • [Journal-full-title] Minerva urologica e nefrologica = The Italian journal of urology and nephrology
  • [ISO-abbreviation] Minerva Urol Nefrol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Italy
  • [Number-of-references] 51
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97. Morimoto R, Satoh F, Murakami O, Suzuki T, Abe T, Tanemoto M, Abe M, Uruno A, Ishidoya S, Arai Y, Takahashi K, Sasano H, Ito S: Immunohistochemistry of a proliferation marker Ki67/MIB1 in adrenocortical carcinomas: Ki67/MIB1 labeling index is a predictor for recurrence of adrenocortical carcinomas. Endocr J; 2008 Mar;55(1):49-55
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  • [Title] Immunohistochemistry of a proliferation marker Ki67/MIB1 in adrenocortical carcinomas: Ki67/MIB1 labeling index is a predictor for recurrence of adrenocortical carcinomas.
  • Adrenocortical carcinoma (ACC) is a rare, highly malignant tumor.
  • The aim of the present study is to evaluate the prognostic relevance of a proliferation marker Ki67/MIB1 by immunohistochemistry in 17 cases who underwent resections of the primary tumors and diagnosed to have ACC at Tohoku University Hospital based on the criteria of Weiss during the period from 1976 to 2005.
  • The median age at diagnosis was 46 years old, and the mean size of the primary tumors was 7.1 cm with the minimal of 3.5 cm.
  • Kaplan-Meier analysis revealed that patients with Ki67/MIB1LI of 7% or more were associated with significantly shortened disease-free survival (P = 0.0037).
  • The survival analysis with Weiss score showed that patients with the scores of 6 or more had both significantly shortened disease-free survival (P = 0.0001) and overall survival (P = 0.0063).
  • [MeSH-major] Adrenal Cortex Neoplasms / diagnosis. Adrenocortical Carcinoma / diagnosis. Biomarkers, Tumor / metabolism. Cell Proliferation. Ki-67 Antigen / metabolism. Ubiquitin-Protein Ligases / metabolism

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  • (PMID = 18187873.001).
  • [ISSN] 1348-4540
  • [Journal-full-title] Endocrine journal
  • [ISO-abbreviation] Endocr. J.
  • [Language] eng
  • [Publication-type] Evaluation Studies; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Ki-67 Antigen; EC 6.3.2.19 / MIB1 ligase, human; EC 6.3.2.19 / Ubiquitin-Protein Ligases
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98. Xiao ZJ, Li CL: [Combined therapy of advanced adrenal cortical adenocarcinoma]. Zhonghua Yi Xue Za Zhi; 2010 Aug 10;90(30):2123-5
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  • [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

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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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99. Ozimek A, Diebold J, Linke R, Heyn J, Hallfeldt K, Mussack T: Bilateral primary adrenal non-Hodgkin's lymphoma and primary adrenocortical carcinoma--review of the literature preoperative differentiation of adrenal tumors. Endocr J; 2008 Aug;55(4):625-38
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Bilateral primary adrenal non-Hodgkin's lymphoma and primary adrenocortical carcinoma--review of the literature preoperative differentiation of adrenal tumors.
  • Most of the adrenal tumors that are incidentally detected are benign adenomas.
  • The incidence of malignant adrenal tumors including adrenocortical carcinoma (ACC) and primary adrenal lymphoma (PAL) is rather low.
  • As many patients with ACC and PAL are diagnosed at an advanced stage of disease, the overall survival time of both entities remains poor.
  • Unfortunately hitherto preoperative diagnosis of potentially malignant adrenal masses is still a main problem in the treatment of adrenal tumors.
  • We present the case of a 57-year-old male patient with ACC and the case of an 87-year-old male patient with PAL and provide a systematic comparison of the clinical and pathological features of both entities.
  • In both cases clinical and radiological features resulted in an initially false diagnosis.
  • We propose some guidelines for diagnosis and surgical management of adrenal tumors.
  • [MeSH-major] Adrenal Cortex Neoplasms / diagnosis. Adrenal Gland Neoplasms / diagnosis. Adrenocortical Carcinoma / diagnosis. Lymphoma, Non-Hodgkin / diagnosis

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  • (PMID = 18490838.001).
  • [ISSN] 1348-4540
  • [Journal-full-title] Endocrine journal
  • [ISO-abbreviation] Endocr. J.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Japan
  • [Chemical-registry-number] 78E4J5IB5J / Mitotane
  • [Number-of-references] 55
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100. Rescorla FJ: Malignant adrenal tumors. Semin Pediatr Surg; 2006 Feb;15(1):48-56
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Malignant adrenal tumors.
  • Adrenal tumors, apart from neuroblastoma, are relatively rare in infancy and childhood.
  • Most adrenal lesions are benign, and both benign and malignant tumors may be hormonally active thus, making accurate preoperative diagnosis difficult.
  • The two main malignant tumors are adrenocortical carcinoma and pheochromocytoma.
  • In both tumors, it may be difficult to determine benign from malignant and the biologic behavior and degree of invasion may portend a more malignant course.
  • An open procedure should be considered for invasive adrenocortical carcinoma and in pheochromocytomas in which preoperative imaging demonstrates metastatic nodal disease.
  • Chemotherapy, although without proven efficacy, is utilized in some children with metastatic or unresectable disease.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / therapy. Adrenocortical Carcinoma / diagnosis. Adrenocortical Carcinoma / therapy. Pheochromocytoma / diagnosis. Pheochromocytoma / therapy

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  • (PMID = 16458846.001).
  • [ISSN] 1055-8586
  • [Journal-full-title] Seminars in pediatric surgery
  • [ISO-abbreviation] Semin. Pediatr. Surg.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 98
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