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6. Fulmer BR: Diagnosis and management of adrenal cortical carcinoma. Curr Urol Rep; 2007 Jan;8(1):77-82
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  • [Title] Diagnosis and management of adrenal cortical carcinoma.
  • Adrenal cortical carcinoma is a relatively uncommon malignancy that represents a significant clinical challenge for the development of optimal treatment strategies.
  • Although the framework of a successful treatment paradigm still relies on these steps, advances in diagnostic imaging have led to increased accuracy in diagnosis, and advances in laparoscopic surgical technique have served to reduce morbidity for patients facing treatment.
  • This review focuses on a discussion of advances in modalities for the diagnosis and treatment of adrenal cortical carcinoma amenable to curative therapy.
  • Patients that present with metastatic or locally advanced disease generally are treated with mitotane-based chemotherapy with or without the addition of cytotoxic drugs.
  • Contemporary results of this treatment approach are presented in this review as well as a discussion of further directions for the treatment of patients with advanced disease.
  • [MeSH-major] Adrenal Cortex Neoplasms / diagnosis. Adrenal Cortex Neoplasms / therapy. Adrenocortical Carcinoma / diagnosis. Adrenocortical Carcinoma / therapy

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  • (PMID = 17239320.001).
  • [ISSN] 1534-6285
  • [Journal-full-title] Current urology reports
  • [ISO-abbreviation] Curr Urol Rep
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 50
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7. Karasavvidou F, Potamianos SP, Barbanis S, Stathakis E, Psychos A, Kapsoritakis AN, Koukoulis G: Malakoplakia of the colon associated with colonic adenocarcinoma diagnosed in colonic biopsies. World J Gastroenterol; 2007 Dec 7;13(45):6109-11
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  • [Title] Malakoplakia of the colon associated with colonic adenocarcinoma diagnosed in colonic biopsies.
  • It has also been found in many other sites such as the gastrointestinal tract, pancreas, liver, lymph nodes, skin, respiratory tract, adrenal gland, vagina and brain.
  • Colonoscopy revealed a large malignant - appearing polypoid mass of the ascending colon and multiple distinct polyps throughout the rest of the colon.
  • Biopsies of the ascending colon mass confirmed the diagnosis of adenocarcinoma.
  • [MeSH-major] Adenocarcinoma / complications. Colonic Neoplasms / complications. Malacoplakia / complications

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  • (PMID = 18023111.001).
  • [ISSN] 1007-9327
  • [Journal-full-title] World journal of gastroenterology
  • [ISO-abbreviation] World J. Gastroenterol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] China
  • [Other-IDs] NLM/ PMC4250902
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8. Ignaszak-Szczepaniak M, Horst-Sikorska W, Sawicka J, Kaczmarek M, Slomski R: The TP53 codon 72 polymorphism and predisposition to adrenocortical cancer in Polish patients. Oncol Rep; 2006 Jul;16(1):65-71
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  • [Title] The TP53 codon 72 polymorphism and predisposition to adrenocortical cancer in Polish patients.
  • We have analyzed the 72Pro polymorphic variant in patients with adrenocortical tumors to evaluate whether 72G--> C substitution at codon 72 of TP53 gene may be associated with increased risk for malignancy in adrenal cortex in comparison to the control group.
  • DNA extracted from peripheral leucocytes of 46 Polish patients with adrenocortical tumors (17 malignant and 29 benign) and 50 controls was examined by PCR-HD method followed by direct sequencing.
  • High frequency of 72Pro allele in patients with carcinoma (29%) in comparison to the benign tumors (14%) and controls (12%) was statistically analyzed.
  • Our results suggest that the TP53 codon 72 polymorphism could be associated with susceptibility for adrenocortical cancer in the examined Polish patients.
  • [MeSH-major] Adrenal Cortex Neoplasms / genetics. Genetic Predisposition to Disease. Polymorphism, Genetic. Tumor Suppressor Protein p53 / genetics

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  • (PMID = 16786124.001).
  • [ISSN] 1021-335X
  • [Journal-full-title] Oncology reports
  • [ISO-abbreviation] Oncol. Rep.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Greece
  • [Chemical-registry-number] 0 / Codon; 0 / TP53 protein, human; 0 / Tumor Suppressor Protein p53
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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.
  • [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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1
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4. 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. Strosberg JR, Hammer GD, Doherty GM: Management of adrenocortical carcinoma. J Natl Compr Canc Netw; 2009 Jul;7(7):752-8; quiz 759
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  • [Title] Management of adrenocortical carcinoma.
  • Adrenocortical carcinomas (ACCs) are rare tumors that arise from the cortex of the adrenal gland with an incidence 1 to 2 per million.
  • This article describes the basic diagnostic and prognostic issues in adrenal cancer management, and presents detailed rationales for therapeutic management.
  • [MeSH-major] Adrenal Cortex Neoplasms / therapy. Adrenocortical Carcinoma / therapy

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  • (PMID = 19635227.001).
  • [ISSN] 1540-1405
  • [Journal-full-title] Journal of the National Comprehensive Cancer Network : JNCCN
  • [ISO-abbreviation] J Natl Compr Canc Netw
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Hormonal; 0 / Catecholamines; 5001-33-2 / Metanephrine; 78E4J5IB5J / Mitotane; WI4X0X7BPJ / Hydrocortisone
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16. Barzon L, Masi G, Pacenti M, Trevisan M, Fallo F, Remo A, Martignoni G, Montanaro D, Pezzi V, Palù G: Expression of aromatase and estrogen receptors in human adrenocortical tumors. Virchows Arch; 2008 Feb;452(2):181-91
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  • [Title] Expression of aromatase and estrogen receptors in human adrenocortical tumors.
  • We recently demonstrated that adrenocortical carcinoma cells express aromatase and estrogen receptors (ERs) and that 17beta-estradiol enhances adrenocortical cell proliferation.
  • To provide a clue to the role of estrogens in adrenal tumorigenesis, we investigated the expression profile of genes involved in sex steroid hormone production and activity in a large series of normal and neoplastic human adrenocortical tissues.
  • Quantitative reverse transcriptase-polymerase chain reaction, Western blotting, and immunohistochemistry showed that ERalpha and ERbeta, androgen receptor (AR), and aromatase were expressed in the adrenal cortex and in adrenocortical tumors.
  • Western blot analysis revealed the presence of a truncated form of AR in adrenocortical tissues.
  • With respect to the normal adrenal cortex and adrenocortical adenomas, carcinomas were characterized by significantly lower ERbeta levels, ERalpha upregulation, and aromatase overexpression.
  • In agreement with our in vitro findings, the results of this study suggest that estrogens, locally produced by aromatase, could enhance adrenocortical cell proliferation though autocrine/paracrine mechanisms.
  • [MeSH-major] Adrenal Cortex Neoplasms / metabolism. Adrenocortical Adenoma / metabolism. Adrenocortical Carcinoma / metabolism. Aromatase / metabolism. Receptors, Estrogen / metabolism
  • [MeSH-minor] Adrenal Cortex / embryology. Adrenal Cortex / metabolism. Adult. Aged. Biomarkers, Tumor / metabolism. Blotting, Western. Female. Gene Expression. Humans. Male. Middle Aged. RNA, Messenger / metabolism. RNA, Neoplasm / analysis

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  • [CommentIn] Virchows Arch. 2008 Aug;453(2):221-2 [18553103.001]
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  • (PMID = 18157729.001).
  • [ISSN] 0945-6317
  • [Journal-full-title] Virchows Archiv : an international journal of pathology
  • [ISO-abbreviation] Virchows Arch.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / RNA, Messenger; 0 / RNA, Neoplasm; 0 / Receptors, Estrogen; EC 1.14.14.1 / Aromatase
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17. 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


18. 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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19. 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.
  • 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


20. 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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21. 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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22. Gil J, Kalembkiewicz M, Polak E, Kostecka-Matyja M: [Disseminated adrenocortical carcinoma: case report]. Pol Arch Med Wewn; 2007 Jul;117(7):317-21
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  • [Title] [Disseminated adrenocortical carcinoma: case report].
  • [Transliterated title] Rozsiany rak kory nadnercza.
  • Adrenocortical carcinoma is a rare neoplasm occurring with a frequency of 1-2 cases per million.
  • Etiology of adrenocortical carcinoma is still unclear, but a role of genetic and environmental factors has been largely considered.
  • Most of the carcinomas (60%) are functional and usually the first manifestation is Cushing's syndrome with virilization.
  • Histopathology specimen from biopsy or obtained during operation should be stained for Melan A, which can confirm the adrenal origin of the tumor.
  • The only method of treatment is a complete surgical excision of the carcinoma.
  • We presented the case of functioning adrenocortical cancer in 37-year-old patient who at time of diagnosis had 12 cm in diameter tumor of the left adrenal gland and metastases to the liver and lung.
  • In the article the symptoms associated with hormones produced by the carcinoma, diagnostics and treatment with regard to the progression of the disease have also been discussed.
  • [MeSH-major] Adrenal Cortex Neoplasms / pathology. Adrenocortical Carcinoma / pathology. Liver Neoplasms / secondary. Lung Neoplasms / secondary

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  • (PMID = 17966598.001).
  • [Journal-full-title] Polskie Archiwum Medycyny Wewnetrznej
  • [ISO-abbreviation] Pol. Arch. Med. Wewn.
  • [Language] pol
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Poland
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23. Carmona-Bayonas A, Soler IO, Gómez FI, Billalabeitia EG, Saura HP, Tafalla MS, Díaz MP: Tailored hormonal therapy in secretory adrenocortical cancer. Ann Oncol; 2007 Jul;18(7):1281
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  • [Title] Tailored hormonal therapy in secretory adrenocortical cancer.

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  • (PMID = 17675396.001).
  • [ISSN] 0923-7534
  • [Journal-full-title] Annals of oncology : official journal of the European Society for Medical Oncology
  • [ISO-abbreviation] Ann. Oncol.
  • [Language] ENG
  • [Publication-type] Case Reports; Letter
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Angiotensin-Converting Enzyme Inhibitors; 0 / Anti-Inflammatory Agents; 0 / Antifungal Agents; 0 / Antineoplastic Agents, Hormonal; 0 / Mineralocorticoid Receptor Antagonists; 27O7W4T232 / Spironolactone; 4964P6T9RB / Aldosterone; 6PLQ3CP4P3 / Etoposide; 78E4J5IB5J / Mitotane; 80168379AG / Doxorubicin; 9G64RSX1XD / Captopril; Q20Q21Q62J / Cisplatin; R9400W927I / Ketoconazole; RWP5GA015D / Potassium; WI4X0X7BPJ / Hydrocortisone
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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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  • 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. 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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28. 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


29. West AN, Ribeiro RC, Jenkins J, Rodriguez-Galindo C, Figueiredo BC, Kriwacki R, Zambetti GP: Identification of a novel germ line variant hotspot mutant p53-R175L in pediatric adrenal cortical carcinoma. Cancer Res; 2006 May 15;66(10):5056-62
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  • [Title] Identification of a novel germ line variant hotspot mutant p53-R175L in pediatric adrenal cortical carcinoma.
  • When inherited, hotspot mutants are associated with Li-Fraumeni syndrome (LFS), a familial cancer predisposition.
  • We have identified a novel germ line variant of the 175 mutant (Arg to Leu; R175L) in a pediatric patient who developed adrenal cortical carcinoma.
  • These findings suggest that p53-R175L retains sufficient activity to suppress LFS, but not adrenal cortical carcinoma.
  • [MeSH-major] Adrenal Cortex Neoplasms / genetics. Genes, p53 / genetics. Germ-Line Mutation
  • [MeSH-minor] Adult. Apoptosis / genetics. Cell Growth Processes / genetics. Child. Child, Preschool. Female. Humans. Male. Models, Molecular. Pedigree

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  • (PMID = 16707427.001).
  • [ISSN] 0008-5472
  • [Journal-full-title] Cancer research
  • [ISO-abbreviation] Cancer Res.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA104568; 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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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. Aiba M, Fujibayashi M: Histopathological diagnosis and prognostic factors in adrenocortical carcinoma. Endocr Pathol; 2005;16(1):13-22
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  • [Title] Histopathological diagnosis and prognostic factors in adrenocortical carcinoma.
  • A great majority of adrenocortical tumors are benign, and many adrenocortical carcinomas (ACC) are obviously malignant at presentation.
  • The histopathological diagnosis of ACC is occasionally difficult, particularly with stage I and stage II disease.
  • Histopathological prognostic factors of ACC have not been fully established because of the rarity of the disease.
  • These special type tumors include pediatric adrenocortical tumors, oncocytomas, and aldosterone-producing tumors of pure zona glomerulosa type.
  • Then we present three cases with unusual small adrenocortical tumors.
  • One patient had an unequivocal ACC showing metastatic disease.
  • [MeSH-major] Adrenal Cortex Neoplasms / diagnosis. Adrenocortical Carcinoma / diagnosis
  • [MeSH-minor] Adenoma, Oxyphilic. Adult. Aged. Aged, 80 and over. Aldosterone / metabolism. Biomarkers, Tumor / metabolism. Cell Nucleus / pathology. Female. Humans. Immunohistochemistry. Infant. Insulin-Like Growth Factor II / metabolism. Male. Neoplasm Staging. Prognosis

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  • (PMID = 16000842.001).
  • [ISSN] 1046-3976
  • [Journal-full-title] Endocrine pathology
  • [ISO-abbreviation] Endocr. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 4964P6T9RB / Aldosterone; 67763-97-7 / Insulin-Like Growth Factor II
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32. 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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33. 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


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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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. 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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41. 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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42. Opocher G, Schiavi F, Cicala MV, Patalano A, Mariniello B, Boaretto F, Zovato S, Pignataro V, Macino B, Negro I, Mantero F: Genetics of adrenal tumors. Minerva Endocrinol; 2009 Jun;34(2):107-21
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  • [Title] Genetics of adrenal tumors.
  • Endocrinology pioneered the development of molecular medicine, but also the study of adrenal tumors had a great impact in this field.
  • Particularly important was the detection of genetics of tumors derived from the adrenal medulla, as well as that of those derived from the sympathetic and parasympathetic paraganglia.
  • Less well understood is the genetics of adrenal cortex tumors, in particular adrenocortical carcinoma, a rare and particularly aggressive disease.
  • There are only a few examples of hereditary transmission of adrenocortical carcinoma, but the analysis of low penetrance genes by genome wide association study may enable us to discover new genetic mechanisms responsible for adrenocortical-derived tumors.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Biomarkers, Tumor / genetics. Mutation. Pheochromocytoma / genetics
  • [MeSH-minor] Adrenal Cortex Neoplasms / genetics. Adrenocortical Carcinoma / genetics. Genetic Predisposition to Disease. Genomics. Humans. Neoplasm Proteins / genetics. Paraganglioma / genetics

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  • (PMID = 19471236.001).
  • [ISSN] 0391-1977
  • [Journal-full-title] Minerva endocrinologica
  • [ISO-abbreviation] Minerva Endocrinol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Neoplasm Proteins
  • [Number-of-references] 81
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43. Welsh SJ, Khan S: Radiological localizing techniques in adrenal tumors. Minerva Endocrinol; 2009 Jun;34(2):161-9
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  • [Title] Radiological localizing techniques in adrenal tumors.
  • The characterisation of adrenal lesions is a common radiological dilemma.
  • Incidental adrenal lesions are commonly detected with computed tomography (CT), and lesion characterisation is critical.
  • The prevalence of incidental adrenal lesions has been reported to be 2.3% at autopsy and 0.5-2% with abdominal CT.
  • Although the majority of adrenal lesions are benign, in patients with an extra-adrenal primary cancer the probability of an adrenal mass being a metastasis is 52%.
  • Unfortunately, there may be significant overlap between the imaging appearances of benign lesions such as lipid-poor adenomas and malignant lesions, particularly metastases and small adrenal carcinomas.
  • This review highlights recent advances in radiological imaging of adrenal lesions and we discuss the relative merits of CT and magnetic resonance imaging to aid the identification of benign and malignant adrenal lesions and their roles, in combination with biochemical and clinical data, in recognizing common pathologies such as adrenal adenoma, phaeochromocytoma, carcinoma and metastases.
  • We also discuss the radiological characteristics of rarer adrenal lesions including lymphoma, neuroblastic tumours (neuroblastoma, ganglioneuroblastoma, and ganglioneuroma), lipomatous tumours (myelolipoma, angiolipoma, teratoma, lipoma and liposarcoma), in addition to hemangioma, hemangiosarcoma and leiomyosarcoma.
  • [MeSH-major] Adrenal Gland Neoplasms / radiography. Incidental Findings. Tomography, X-Ray Computed
  • [MeSH-minor] Adrenal Cortex Neoplasms / radiography. Adrenocortical Adenoma / radiography. Adrenocortical Carcinoma / radiography. Diagnosis, Differential. Ganglioneuroblastoma / radiography. Ganglioneuroma / radiography. Humans. Lymphoma / radiography. Magnetic Resonance Imaging. Neoplasm Metastasis. Neoplasms, Adipose Tissue / radiography. Neoplasms, Vascular Tissue / radiography. Neuroblastoma / radiography. Pheochromocytoma / radiography. Predictive Value of Tests. Sensitivity and Specificity. Teratoma / radiography

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  • (PMID = 19471240.001).
  • [ISSN] 0391-1977
  • [Journal-full-title] Minerva endocrinologica
  • [ISO-abbreviation] Minerva Endocrinol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Italy
  • [Number-of-references] 42
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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
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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.
  • 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. Schmidt A, Schwella N, Helmchen U, von Renteln D, Caca K: [A 42 year old patient with bilateral loss of sight and hypertension. Gemcitabine-associated thrombotic microangiopathy (TMA)]. Internist (Berl); 2008 Aug;49(8):989-90, 992-4
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  • We report a case of a 42 year old male patient with a history of adrenocortical carcinoma, who was admitted with bilateral loss of sight and hypertension.
  • Laboratory tests and further clinical evaluation showed hemolytic anemia, thrombocytopenia and acute renal failure.
  • [MeSH-major] Adrenal Cortex Neoplasms / drug therapy. Anemia, Hemolytic / chemically induced. Antimetabolites, Antineoplastic / toxicity. Blindness / etiology. Deoxycytidine / analogs & derivatives. Hemolytic-Uremic Syndrome / chemically induced. Hypertension, Malignant / etiology. Purpura, Thrombotic Thrombocytopenic / chemically induced
  • [MeSH-minor] Adult. Biopsy. Diagnosis, Differential. Humans. Kidney / pathology. Male

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  • (PMID = 18427761.001).
  • [ISSN] 0020-9554
  • [Journal-full-title] Der Internist
  • [ISO-abbreviation] Internist (Berl)
  • [Language] ger
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Germany
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48. 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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49. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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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50. 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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51. Fernandez-Ranvier GG, Weng J, Yeh RF, Shibru D, Khafnashar E, Chung KW, Hwang J, Duh QY, Clark OH, Kebebew E: Candidate diagnostic markers and tumor suppressor genes for adrenocortical carcinoma by expression profile of genes on chromosome 11q13. World J Surg; 2008 May;32(5):873-81
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  • [Title] Candidate diagnostic markers and tumor suppressor genes for adrenocortical carcinoma by expression profile of genes on chromosome 11q13.
  • BACKGROUND: The most common genetic change observed in adrenocortical carcinoma is loss of heterozygozity on chromosome 11q13.
  • As genes on this chromosome may be important in the pathogenesis of adrenocortical carcinoma, we compared their expression profile between benign and malignant adrenocortical tissue.
  • METHODS: We used the Affymetrix GeneChip (U133 plus 2.0) array in 54 adrenocortical tumors (11 carcinoma and 43 benign).
  • The area under the receiver operating characteristic (ROC) curve (AUC) was used to determined the diagnostic accuracy of the differently expressed genes for distinguishing benign from malignant tumors.
  • RESULTS: We found 25 of the 314 genes on chromosome 11q13 to be differentially expressed between adrenocortical carcinoma and benign adrenocortical tumor.
  • All 25 were downregulated in adrenocortical carcinoma by 2-fold to 4.8-fold; 21 were validated to be differentially expressed by RT-PCR (Pearson's coefficient>0.5).
  • Six genes (SERPING1, MRPL48, TM7SF2, DDB1, NDUSF8, PRDX5) validated by RT-PCR were significantly differentially expressed between benign and malignant adrenocortical tumors (p<0.05) with an overall accuracy of 89% for SERPING1, 91% for MRPL48, 87% for TM7SF2, 88% for DDB1, 91% for NDUFS8, and 89% for PRDX5.
  • CONCLUSIONS: We have identified 25 genes located on chromosome 11q13 that are downregulated in adrenocortical carcinoma and may be candidate tumor suppressor genes.
  • Six of these genes were good diagnostic markers for distinguishing adrenocortical carcinoma from adenoma.
  • [MeSH-major] Adrenal Cortex Neoplasms / genetics. Adrenocortical Carcinoma / genetics. Chromosomes, Human, Pair 11 / genetics. Genes, Tumor Suppressor / physiology. Loss of Heterozygosity / genetics

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  • (PMID = 18324346.001).
  • [ISSN] 0364-2313
  • [Journal-full-title] World journal of surgery
  • [ISO-abbreviation] World J Surg
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Genetic Markers
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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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  • [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. Lou E, Goodwin J, Howell DN, Hicks J, Caram LB: A G-CSF-secreting adrenal carcinoma with rhabdoid-like differentiation causing leukocytosis. Nat Rev Urol; 2009 Jul;6(7):392-7
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  • [Title] A G-CSF-secreting adrenal carcinoma with rhabdoid-like differentiation causing leukocytosis.
  • He had chronic renal insufficiency, sleep apnea, hypertension, and peripheral vascular disease.
  • DIAGNOSIS: G-CSF-secreting adrenal carcinoma with rhabdoid-like differentiation.
  • [MeSH-major] Adrenal Cortex Neoplasms / secretion. Adrenocortical Carcinoma / secretion. Biomarkers, Tumor / secretion. Cell Transformation, Neoplastic / pathology. Cell Transformation, Neoplastic / secretion. Granulocyte Colony-Stimulating Factor / secretion. Leukocytosis / diagnosis. Rhabdoid Tumor / secretion

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  • (PMID = 19578356.001).
  • [ISSN] 1759-4820
  • [Journal-full-title] Nature reviews. Urology
  • [ISO-abbreviation] Nat Rev Urol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 143011-72-7 / Granulocyte Colony-Stimulating Factor
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58. 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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  • 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.
  • [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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59. 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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  • [Cites] World J Urol. 1999 Feb;17(1):26-34 [10096148.001]
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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
  • [Other-IDs] NLM/ PMC3097607
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60. 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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  • [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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61. Soon PS, Sidhu SB: Adrenocortical carcinoma. Cancer Treat Res; 2010;153:187-210
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Adrenocortical carcinoma.
  • [MeSH-major] Adrenal Cortex Neoplasms / therapy. Adrenocortical Carcinoma / therapy

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  • (PMID = 19957226.001).
  • [ISSN] 0927-3042
  • [Journal-full-title] Cancer treatment and research
  • [ISO-abbreviation] Cancer Treat. Res.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 137
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62. Hirano H, Yoshida T, Sakamoto T, Yoshimura H, Fukuoka M, Tachibana S, Saito H, Ohkubo E, Nakasho K, Nishigami T: Pulmonary pleomorphic carcinoma producing hCG. Pathol Int; 2007 Oct;57(10):698-702
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  • [Title] Pulmonary pleomorphic carcinoma producing hCG.
  • An 80-year-old woman with a pleomorphic carcinoma (PC) producing hCG was admitted to Nippon Steel Hirohata Hospital because of an abnormal shadow on CT seen during a follow-up examination after surgery for breast cancer.
  • On histology the tumor consisted mostly of intermingled spindle and polygonal cells, while evidence of poorly differentiated adenocarcinoma was seen in a few areas.
  • A diagnosis of PC was made due to hCG expression in approximately 20% of the spindle and polygonal cells on immunohistology.
  • Six months after the operation metastasis to the liver and adrenal gland was seen on CT.
  • [MeSH-major] Adenocarcinoma / metabolism. Biomarkers, Tumor / metabolism. Carcinoma, Non-Small-Cell Lung / metabolism. Carcinosarcoma / metabolism. Chorionic Gonadotropin / metabolism. Lung Neoplasms / metabolism

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  • (PMID = 17803660.001).
  • [ISSN] 1320-5463
  • [Journal-full-title] Pathology international
  • [ISO-abbreviation] Pathol. Int.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Australia
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Chorionic Gonadotropin
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63. Ren R, Guo M, Sneige N, Moran CA, Gong Y: Fine-needle aspiration of adrenal cortical carcinoma: cytologic spectrum and diagnostic challenges. Am J Clin Pathol; 2006 Sep;126(3):389-98
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  • [Title] Fine-needle aspiration of adrenal cortical carcinoma: cytologic spectrum and diagnostic challenges.
  • We reviewed the cytologic features of 20 adrenal cortical carcinomas (ACCs; 9 primary and 11 metastatic) from 19 patients and highlighted diagnostic pitfalls.
  • Primary and metastatic ACCs were cytologically similar and showed a wide range of features varying from well-differentiated tumor resembling a benign cortical lesion or low-grade neuroendocrine tumor to poorly differentiated pleomorphic tumor mimicking poorly differentiated carcinoma, melanoma, or high-grade sarcoma.
  • Cytologic, immunophenotypic, and ultrastructural findings should be correlated with clinical and radiologic information for achieving a proper cytologic diagnosis.
  • [MeSH-major] Adrenal Cortex / pathology. Adrenal Cortex Neoplasms / pathology. Adrenocortical Carcinoma / pathology

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  • (PMID = 16880150.001).
  • [ISSN] 0002-9173
  • [Journal-full-title] American journal of clinical pathology
  • [ISO-abbreviation] Am. J. Clin. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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64. 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


65. Argyriou P, Zisis C, Alevizopoulos N, Kefaloyannis EM, Gennatas C, Petraki CD: Adrenocortical oncocytic carcinoma with recurrent metastases: a case report and review of the literature. World J Surg Oncol; 2008;6:134
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Adrenocortical oncocytic carcinoma with recurrent metastases: a case report and review of the literature.
  • BACKGROUND: Adrenal cortex oncocytic carcinoma (AOC) represents an exceptional pathological entity, since only 22 cases have been documented in the literature so far.
  • CASE PRESENTATION: Our case concerns a 54-year-old man with past medical history of right adrenal excision with partial hepatectomy, due to an adrenocortical carcinoma.
  • Finally, approximately five years post disease onset, the patient died due to massive metastatic disease.
  • CONCLUSION: Histological classification of adrenocortical oncocytic tumours has been so far a matter of debate.
  • [MeSH-major] Adrenal Cortex Neoplasms / pathology. Adrenocortical Carcinoma / pathology

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  • (PMID = 19091123.001).
  • [ISSN] 1477-7819
  • [Journal-full-title] World journal of surgical oncology
  • [ISO-abbreviation] World J Surg Oncol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC2630932
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66. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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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67. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • 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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68. Chen CH, Wu HC, Chang CH: An accessory spleen mimics a left adrenal carcinoma. MedGenMed; 2005;7(2):9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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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69. 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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70. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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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71. 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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72. Kaur A, Falleiro JJ, Ghosh AK, Mani NS: Pulmonary carcinosarcoma masquerading as a cryptic disseminated malignancy. Indian J Pathol Microbiol; 2009 Oct-Dec;52(4):517-9
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  • Pulmonary carcinosarcoma is an aggressive rare malignant tumor comprising a mixture of carcinoma and sarcoma components containing differentiated mesenchymal elements, such as malignant cartilage, bone, and skeletal muscle.
  • At autopsy, it was a stage IV lung malignancy and histopathology revealed a carcinosarcoma comprising an adenocarcinoma and an osteosarcoma with metastasis to the heart, lymph nodes, and both adrenals.
  • The present case had an unusual clinical presentation and its elusive nature towards diagnosis despite dissemination is noteworthy and unique in the literature.
  • [MeSH-major] Carcinosarcoma / diagnosis. Carcinosarcoma / pathology. Lung Neoplasms / diagnosis. Lung Neoplasms / pathology
  • [MeSH-minor] Adrenal Gland Neoplasms / pathology. Adrenal Gland Neoplasms / secondary. Adult. Autopsy. Heart Neoplasms / pathology. Heart Neoplasms / secondary. Histocytochemistry. Humans. Immunohistochemistry. Lymph Nodes / pathology. Male. Severity of Illness Index

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  • (PMID = 19805960.001).
  • [ISSN] 0974-5130
  • [Journal-full-title] Indian journal of pathology & microbiology
  • [ISO-abbreviation] Indian J Pathol Microbiol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] India
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73. 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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74. 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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  • [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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75. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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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76. 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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77. Lichtenauer UD, Shapiro I, Geiger K, Quinkler M, Fassnacht M, Nitschke R, Rückauer KD, Beuschlein F: Side population does not define stem cell-like cancer cells in the adrenocortical carcinoma cell line NCI h295R. Endocrinology; 2008 Mar;149(3):1314-22
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  • [Title] Side population does not define stem cell-like cancer cells in the adrenocortical carcinoma cell line NCI h295R.
  • Recent evidence suggests the existence of a stem cell-like subpopulation of cells in hematological and solid tumor entities, which determine the malignant phenotype of a given tumor through their proliferative potential and chemotherapy resistance.
  • A recently used technique for the isolation of this cell population is through exclusion of the vital dye Hoechst 33342, which defines the so-called side population (SP).
  • Herein we demonstrate the presence of SP cells in a variety of adrenal specimens, including primary cultures of human adrenocortical tumors and normal adrenal glands as well as established human and murine adrenocortical cancer cell lines by fluorescence-activated cell sorter analysis and confocal microscopy.
  • On a functional level, SP cells from the human adrenocortical tumor cell line NCI h295R revealed an expression pattern consistent with a less differentiated phenotype, including lower expression of steroidogenic enzymes such as steroid acute regulatory protein (StAR) and side-chain cleavage enzyme (P450scc) in comparison with non-SP cells.
  • Furthermore, re-sorting and tracing experiments revealed the capacity for both cell types to give rise to the original SP- and non-SP-containing cell population.
  • Similarly to the baseline growth kinetics, no survival benefit was evident in SP cells after treatment with cytotoxic agents commonly used in adrenocortical carcinomas.
  • Taken together, these findings provide evidence that Hoechst dye exclusion, in contrast to what has been reported for other tumor entities, is not a major tumor stem cell defining marker in adrenocortical NCI h295R tumor cells.
  • [MeSH-major] Adrenal Cortex Neoplasms / pathology. Adrenocortical Carcinoma / pathology. Neoplastic Stem Cells / cytology
  • [MeSH-minor] Adrenal Glands / cytology. Antineoplastic Agents / pharmacology. Antineoplastic Agents / therapeutic use. Cell Cycle / physiology. Cell Differentiation / physiology. Cell Line, Tumor. Cell Proliferation. Cholesterol Side-Chain Cleavage Enzyme / metabolism. Coloring Agents. Drug Resistance, Neoplasm / physiology. Humans. Phenotype. Phosphoproteins / metabolism. Tumor Cells, Cultured

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  • (PMID = 18063677.001).
  • [ISSN] 0013-7227
  • [Journal-full-title] Endocrinology
  • [ISO-abbreviation] Endocrinology
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Coloring Agents; 0 / Phosphoproteins; 0 / steroidogenic acute regulatory protein; EC 1.14.15.6 / Cholesterol Side-Chain Cleavage Enzyme
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78. 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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79. 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


80. 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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81. Iitaka D, Ikoma H, Kawaguchi T, Murayama Y, Komatsu S, Shiozaki A, Kuriu Y, Nakanishi M, Ichikawa D, Fujiwara H, Okamoto K, Ochiai T, Kokuba Y, Sonoyama T, Konishi H, Yoshikawa T, Otsuji E: [A case report--locally advanced pancreatic adenocarcinoma was resected after chemotherapy]. Gan To Kagaku Ryoho; 2010 Nov;37(12):2358-60
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  • [Title] [A case report--locally advanced pancreatic adenocarcinoma was resected after chemotherapy].
  • Based on these results and biopsy, the patient was diagnosed with pancreatic and SMA invasion of duodenal cancer that was considered to be unresectable.
  • We performed a pancreatic tail resection along with combined resection of third and fourth portions of the duodenum, transverse colon and splenic flexure, and left adrenal gland.
  • The case was diagnosed to be well-differentiated invasive ductal pancreatic cancer with duodenal invasion.
  • Cancer invasion was not observed in any of the stripped surfaces surrounding the pancreas.
  • In this case, the treatment was initially started by considering the case as one of duodenal cancer, but the final results of a pathological diagnosis revealed that it was pancreatic cancer.
  • However, either way, even though the case was unresectable before the chemotherapy performed for duodenal cancer was significantly effective for the pancreatic cancer.
  • [MeSH-major] Adenocarcinoma / therapy. Pancreatic Neoplasms / therapy

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  • (PMID = 21224572.001).
  • [ISSN] 0385-0684
  • [Journal-full-title] Gan to kagaku ryoho. Cancer & chemotherapy
  • [ISO-abbreviation] Gan To Kagaku Ryoho
  • [Language] jpn
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Antimetabolites, Antineoplastic; 0 / Antineoplastic Agents; 0 / Drug Combinations; 0 / Taxoids; 150863-82-4 / S 1 (combination); 1548R74NSZ / Tegafur; 15H5577CQD / docetaxel; 5VT6420TIG / Oxonic Acid; Q20Q21Q62J / Cisplatin
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82. 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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83. 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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84. 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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85. 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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91. 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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92. 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.
  • [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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93. 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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94. 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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95. Castillo O, Sánchez-Salas R, Vidal I: Laparoscopic adrenalectomy. Minerva Urol Nefrol; 2008 Sep;60(3):177-84
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • 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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96. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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 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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97. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Combined therapy of advanced adrenal cortical adenocarcinoma].
  • OBJECTIVE: To analyze the clinical efficacy of combined therapy in the treatment of advanced adrenal cortical adenocarcinoma.
  • METHODS: The clinical data of 12 cases with advanced adrenal cortical adenocarcinoma at our hospital from 1986 - 2006 were analyzed.
  • Pathological diagnosis was all of adrenal cortical adenocarcinoma.
  • According to evaluation criterion of chemotherapeutic effect by WHO in 1987, the results were: CR (complete remission) (n = 0), PR (partial remission) (n = 7), SD (stable disease) (n = 3) and PD (progressive disease) (n = 2).
  • CONCLUSION: Combined therapy of adrenal cortical adenocarcinoma is effective to prolong the patient lifespan.
  • Making an early diagnosis and offering a novel therapy yield a better outcome.
  • [MeSH-major] Adrenal Cortex Neoplasms / pathology. Adrenal Cortex Neoplasms / therapy. Adrenocortical Carcinoma / pathology. Adrenocortical Carcinoma / therapy

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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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98. Haluska P, Worden F, Olmos D, Yin D, Schteingart D, Batzel GN, Paccagnella ML, de Bono JS, Gualberto A, Hammer GD: Safety, tolerability, and pharmacokinetics of the anti-IGF-1R monoclonal antibody figitumumab in patients with refractory adrenocortical carcinoma. Cancer Chemother Pharmacol; 2010 Mar;65(4):765-73
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Safety, tolerability, and pharmacokinetics of the anti-IGF-1R monoclonal antibody figitumumab in patients with refractory adrenocortical carcinoma.
  • PURPOSE: Insulin-like growth factor 1 receptor signaling through upregulation of the stimulatory ligand IGF-II has been implicated in the pathogenesis of adrenocortical carcinoma.
  • As there is a paucity of effective therapies, this dose expansion cohort of a phase 1 study was undertaken to determine the safety, tolerability, pharmacokinetics, and effects on endocrine markers of figitumumab in patients with adrenocortical carcinoma.
  • METHODS: Figitumumab was administered on day 1 of each 21-day cycle at the maximal feasible dose (20 mg/kg) to a cohort of patients with metastatic, refractory adrenocortical carcinoma.
  • RESULTS: Fourteen patients with adrenocortical carcinoma received 50 cycles of figitumumab at the 20 mg/kg.
  • Pharmacokinetics of figitumumab was comparable to patients with solid tumors other than adrenocortical carcinoma.
  • Eight of 14 patients (57%) had stable disease.
  • CONCLUSIONS: The side effect profile and pharmacokinetics of figitumumab were similar in patients with adrenocortical carcinoma in comparison to patients with other solid tumors.
  • The majority of patients receiving protocol therapy with single agent figitumumab experienced stability of disease, warranting further evaluation.
  • [MeSH-major] Adrenal Cortex Neoplasms / drug therapy. Adrenocortical Carcinoma / drug therapy. Antibodies, Monoclonal / pharmacokinetics. Receptor, IGF Type 1 / antagonists & inhibitors

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  • (PMID = 19649631.001).
  • [ISSN] 1432-0843
  • [Journal-full-title] Cancer chemotherapy and pharmacology
  • [ISO-abbreviation] Cancer Chemother. Pharmacol.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / K12 CA090628; United States / NCI NIH HHS / CA / K12 CA090628-05
  • [Publication-type] Clinical Trial, Phase I; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Immunoglobulins, Intravenous; 0 / Insulin; 9002-72-6 / Growth Hormone; EC 2.7.10.1 / Receptor, IGF Type 1; VE267FC2UB / figitumumab
  • [Other-IDs] NLM/ NIHMS190253; NLM/ PMC2875253
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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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  • [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.
  • 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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  • [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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