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Malignant Sex Cord-Stromal Tumor

MedGen UID:
338406
•Concept ID:
C1848186
•
Neoplastic Process
Synonym: Malignant sex-cord stromal tumor
SNOMED CT: Malignant sex cord-stromal tumor (1332421008)

Definition

A gonadal sex cord-stromal tumor with malignant characteristics. It includes the poorly differentiated ovarian Sertoli-Leydig cell tumor, malignant ovarian granulosa cell tumor, malignant ovarian steroid cell tumor, and malignant ovarian thecoma. [from NCI]

Professional guidelines

PubMed

Risk Factors and Treatment Outcomes of 1,375 Patients with Testicular Leydig Cell Tumors: Analysis of Published Case Series Data.
Fankhauser CD, Grogg JB, Hayoz S, Wettstein MS, Dieckmann KP, Sulser T, Bode PK, Clarke NW, Beyer J, Hermanns T
J Urol 2020 May;203(5):949-956. Epub 2019 Dec 17 doi: 10.1097/JU.0000000000000705. PMID: 31845841
Management of ovarian disease in pregnancy.
Phelan N, Conway GS
Best Pract Res Clin Endocrinol Metab 2011 Dec;25(6):985-92. doi: 10.1016/j.beem.2011.07.007. PMID: 22115171
Ovarian sex cord-stromal tumors. Problems in differential diagnosis.
Young RH, Scully RE
Pathol Annu 1988;23 Pt 1:237-96. PMID: 2838794

Recent clinical studies

Diagnosis

Ovarian Granulosa Cell Tumor Initially Presenting as a Giant Liver Mass Radiologically Mimicking Primary Cystic Cholangiocarcinoma.
Qian X, Cong Z, Lai J
In Vivo 2023 Mar-Apr;37(2):734-737. doi: 10.21873/invivo.13135. PMID: 36881092Free PMC Article
Malignant testicular unclassified sex cord stromal tumor: a case report.
Morinaga S, Aoki S, Tsuzuki T, Kanematsu K, Kawai N, Sato S, Nakamizu K, Goto K, Niwa M, Nomura I, Sawada T, Futamachi R, Morita F, Yamada Y
J Med Case Rep 2022 Nov 6;16(1):406. doi: 10.1186/s13256-022-03640-z. PMID: 36335378Free PMC Article
Ovarian fibrosarcoma: A single-institution experience and a review of the literature.
Sun TT, Cheng NH, Cao DY, Peng P
J Ovarian Res 2020 Dec 8;13(1):142. doi: 10.1186/s13048-020-00749-x. PMID: 33292402Free PMC Article
Malignant Sex Cord-Stromal Tumor, Not Otherwise Specified, Harboring FOXL2, p53, and TERT Promoter Mutations: Report of a Case.
Mayer R, Dandulakis M, Richards S, Roque DM, Staats PN
Int J Gynecol Pathol 2020 Nov;39(6):567-572. doi: 10.1097/PGP.0000000000000651. PMID: 31789679
Adult granulosa cell tumor of the testis masquerading as hydrocele.
Vallonthaiel AG, Kakkar A, Singh A, Dogra PN, Ray R
Int Braz J Urol 2015 Nov-Dec;41(6):1226-31. doi: 10.1590/S1677-5538.IBJU.2014.0187. PMID: 26742984Free PMC Article

Therapy

Malignant Sex Cord-Stromal Tumor, Not Otherwise Specified, Harboring FOXL2, p53, and TERT Promoter Mutations: Report of a Case.
Mayer R, Dandulakis M, Richards S, Roque DM, Staats PN
Int J Gynecol Pathol 2020 Nov;39(6):567-572. doi: 10.1097/PGP.0000000000000651. PMID: 31789679

Prognosis

Malignant testicular unclassified sex cord stromal tumor: a case report.
Morinaga S, Aoki S, Tsuzuki T, Kanematsu K, Kawai N, Sato S, Nakamizu K, Goto K, Niwa M, Nomura I, Sawada T, Futamachi R, Morita F, Yamada Y
J Med Case Rep 2022 Nov 6;16(1):406. doi: 10.1186/s13256-022-03640-z. PMID: 36335378Free PMC Article
Ovarian fibrosarcoma: A single-institution experience and a review of the literature.
Sun TT, Cheng NH, Cao DY, Peng P
J Ovarian Res 2020 Dec 8;13(1):142. doi: 10.1186/s13048-020-00749-x. PMID: 33292402Free PMC Article
Malignant Sex Cord-Stromal Tumor, Not Otherwise Specified, Harboring FOXL2, p53, and TERT Promoter Mutations: Report of a Case.
Mayer R, Dandulakis M, Richards S, Roque DM, Staats PN
Int J Gynecol Pathol 2020 Nov;39(6):567-572. doi: 10.1097/PGP.0000000000000651. PMID: 31789679
Adult granulosa cell tumor of the testis masquerading as hydrocele.
Vallonthaiel AG, Kakkar A, Singh A, Dogra PN, Ray R
Int Braz J Urol 2015 Nov-Dec;41(6):1226-31. doi: 10.1590/S1677-5538.IBJU.2014.0187. PMID: 26742984Free PMC Article

Clinical prediction guides

Malignant testicular unclassified sex cord stromal tumor: a case report.
Morinaga S, Aoki S, Tsuzuki T, Kanematsu K, Kawai N, Sato S, Nakamizu K, Goto K, Niwa M, Nomura I, Sawada T, Futamachi R, Morita F, Yamada Y
J Med Case Rep 2022 Nov 6;16(1):406. doi: 10.1186/s13256-022-03640-z. PMID: 36335378Free PMC Article
Ovarian fibrosarcoma: A single-institution experience and a review of the literature.
Sun TT, Cheng NH, Cao DY, Peng P
J Ovarian Res 2020 Dec 8;13(1):142. doi: 10.1186/s13048-020-00749-x. PMID: 33292402Free PMC Article

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