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Adrenal Cortical Sex Cord-Stromal Tumor

MedGen UID:
1667866
•Concept ID:
C4745370
•
Neoplastic Process
Synonym: Adrenal Cortex Sex Cord-Stromal Tumor

Definition

An extremely rare sex cord-stromal tumor that arises from the adrenal cortex. The reported cases were solitary and unilateral. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAdrenal Cortical Sex Cord-Stromal Tumor

Professional guidelines

PubMed

Genetic engineering to study testicular tumorigenesis.
Yan W, Burns KH, Matzuk MM
APMIS 2003 Jan;111(1):174-81; discussion 182-3. doi: 10.1034/j.1600-0463.2003.1110121.x. PMID: 12760377
Arrhenoblastoma of the ovary. Presentation of a case report and a discussion of the differential diagnosis.
Radman HM, Bhagavan BS
Am J Obstet Gynecol 1970 Apr 15;106(8):1187-90. PMID: 4314521

Recent clinical studies

Etiology

A comparison of human H295R and rat R2C cell lines as in vitro screening tools for effects on aromatase.
Heneweer M, van den Berg M, Sanderson JT
Toxicol Lett 2004 Jan 15;146(2):183-94. doi: 10.1016/j.toxlet.2003.10.002. PMID: 14643970
Anti-alpha-inhibin: marker of choice for the consistent distinction between adrenocortical carcinoma and renal cell carcinoma in fine-needle aspiration.
Fetsch PA, Powers CN, Zakowski MF, Abati A
Cancer 1999 Jun 25;87(3):168-72. PMID: 10385449
Direct luteinizing hormone action triggers adrenocortical tumorigenesis in castrated mice transgenic for the murine inhibin alpha-subunit promoter/simian virus 40 T-antigen fusion gene.
Rilianawati, Paukku T, Kero J, Zhang FP, Rahman N, Kananen K, Huhtaniemi I
Mol Endocrinol 1998 Jun;12(6):801-9. doi: 10.1210/mend.12.6.0117. PMID: 9626655

Diagnosis

Postmenopausal hyperandrogenism due to an ovarian sex cord-stromal tumour causing elevated dehydroepiandrosterone sulphate: a case report.
Sumanatilleke M, de Silva NL, Ranaweera G, Appuhamy C, Karunaratne K, de Silva MVC
BMC Womens Health 2022 Jul 17;22(1):297. doi: 10.1186/s12905-022-01879-8. PMID: 35843927Free PMC Article
Melanocytic marker Melan-A detects molluscum contagiosum bodies.
Vincek E, Rudnick E
J Histotechnol 2022 Mar;45(1):36-38. Epub 2021 Aug 19 doi: 10.1080/01478885.2021.1964872. PMID: 34407745
Value of calretinin immunostaining in diagnostic pathology: a review and update.
Ordóñez NG
Appl Immunohistochem Mol Morphol 2014 Jul;22(6):401-15. doi: 10.1097/PAI.0b013e31829b6fbd. PMID: 24185118
From the radiologic pathology archives: precocious puberty: radiologic-pathologic correlation.
Chung EM, Biko DM, Schroeder JW, Cube R, Conran RM
Radiographics 2012 Nov-Dec;32(7):2071-99. doi: 10.1148/rg.327125146. PMID: 23150859
Unilateral testicular tumour associated to congenital adrenal hyperplasia: Failure of specific tumoral molecular markers to discriminate between adrenal rest and leydigioma.
Fenichel P, Bstandig B, Roger C, Chevallier D, Michels JF, Sadoul JL, Hieronimus S, Brucker-Davis F
Ann Endocrinol (Paris) 2008 Nov;69(5):453-8. Epub 2008 Jul 11 doi: 10.1016/j.ando.2008.05.002. PMID: 18620332

Therapy

Unilateral testicular tumour associated to congenital adrenal hyperplasia: Failure of specific tumoral molecular markers to discriminate between adrenal rest and leydigioma.
Fenichel P, Bstandig B, Roger C, Chevallier D, Michels JF, Sadoul JL, Hieronimus S, Brucker-Davis F
Ann Endocrinol (Paris) 2008 Nov;69(5):453-8. Epub 2008 Jul 11 doi: 10.1016/j.ando.2008.05.002. PMID: 18620332
Hepatic adenocarcinoma expressing inhibin in a young patient on oral contraceptives.
Vrettou E, Hytiroglou P, Sikas N, Soultoyannis I, Goodman ZD
Virchows Arch 2005 May;446(5):560-5. Epub 2005 Apr 7 doi: 10.1007/s00428-005-1241-3. PMID: 15815932
Coexisting true hermaphroditism and partial hydatidiform mole developing metastatic gestational trophoblastic tumors. A case report.
Shiina H, Oka K, Okane M, Tanno W, Kawasaki T, Nakayama M
Virchows Arch 2002 Nov;441(5):514-8. Epub 2002 Aug 22 doi: 10.1007/s00428-002-0681-2. PMID: 12447683
Steroid hormone-producing tumors in man.
Freeman DA
Endocr Rev 1986 May;7(2):204-20. doi: 10.1210/edrv-7-2-204. PMID: 3013606
Ovarian adrenal rest tumor (masculinovoblastoma) simulating adrenal virilism; report of a case with hormone studies.
EPSTEIN JA, LEVINSON CJ, KUPPERMAN HS
Am J Obstet Gynecol 1957 Nov;74(5):982-8. doi: 10.1016/0002-9378(57)90141-2. PMID: 13469882

Prognosis

High expression of neuropeptide y receptors in tumors of the human adrenal gland and extra-adrenal paraganglia.
Körner M, Waser B, Reubi JC
Clin Cancer Res 2004 Dec 15;10(24):8426-33. doi: 10.1158/1078-0432.CCR-04-0821. PMID: 15623622
Carney's complex of primary pigmented nodular adrenocortical disease and pigmentous and myxomatous lesions.
Cheung PS, Thompson NW
Surg Gynecol Obstet 1989 May;168(5):413-6. PMID: 2711295
Steroid hormone-producing tumors in man.
Freeman DA
Endocr Rev 1986 May;7(2):204-20. doi: 10.1210/edrv-7-2-204. PMID: 3013606

Clinical prediction guides

Genetic engineering to study testicular tumorigenesis.
Yan W, Burns KH, Matzuk MM
APMIS 2003 Jan;111(1):174-81; discussion 182-3. doi: 10.1034/j.1600-0463.2003.1110121.x. PMID: 12760377
Anti-Müllerian hormone is a specific marker of sertoli- and granulosa-cell origin in gonadal tumors.
Rey R, Sabourin JC, Venara M, Long WQ, Jaubert F, Zeller WP, Duvillard P, Chemes H, Bidart JM
Hum Pathol 2000 Oct;31(10):1202-8. doi: 10.1053/hupa.2000.18498. PMID: 11070112
Transcriptional regulation of P450scc gene expression in neural and steroidogenic cells: implications for regulation of neurosteroidogenesis.
Zhang P, Rodriguez H, Mellon SH
Mol Endocrinol 1995 Nov;9(11):1571-82. doi: 10.1210/mend.9.11.8584034. PMID: 8584034
Thecal metaplasia in the adrenal gland of a man with acquired bilateral testicular atrophy.
Romberger CF, Wong TW
Arch Pathol Lab Med 1989 Sep;113(9):1071-5. PMID: 2549908

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