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Malignant Sertoli-Leydig cell tumor of ovary

MedGen UID:
1825991
•Concept ID:
C5574973
•
Neoplastic Process
Synonyms: Androblastoma; Arrhenoblastoma; malignant ovarian Sertoli-Leydig cell tumor; malignant ovarian sertoli-Leydig cell tumor; malignant ovarian Sertoli-Leydig cell tumour; malignant ovarian sertoli-Leydig cell tumour; malignant Sertoli-Leydig cell tumor of ovary; malignant Sertoli-Leydig cell tumor of the ovary; Malignant Sertoli-Leydig cell tumor of the ovary; malignant Sertoli-Leydig cell tumour of the ovary; Ovarian malignant Sertoli-Leydig cell tumor; ovarian malignant Sertoli-Leydig cell tumor; ovarian malignant Sertoli-Leydig cell tumour; Ovarian Sertoli-Leydig cell cancer; ovarian Sertoli-Leydig cell cancer; ovarian sertoli-Leydig cell tumor, malignant; ovarian Sertoli-Leydig cell tumor, malignant; Primary malignant Sertoli-Leydig cell tumor of ovary; Virilizing ovarian tumor; virilizing ovarian tumor; virilizing ovarian tumour
SNOMED CT: Primary malignant Sertoli-Leydig cell tumor of ovary (1284849005); Ovarian malignant Sertoli-Leydig cell tumor (1284849005)
 
Monarch Initiative: MONDO:0020542
Orphanet: ORPHA99916

Definition

A rare malignant sex cord stromal tumour of ovary occurring typically in young women and characterised by manifestations of androgen excess (hirsutism, hair loss, amenorrhoea, or oligomenorrhoea) when functional. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMalignant Sertoli-Leydig cell tumor of ovary

Professional guidelines

PubMed

From diagnosis to treatment of androgen-secreting ovarian tumors: a practical approach.
Rojewska P, Meczekalski B, Bala G, Luisi S, Podfigurna A
Gynecol Endocrinol 2022 Jul;38(7):537-542. Epub 2022 Jun 1 doi: 10.1080/09513590.2022.2083104. PMID: 35647677
Management of early ovarian cancer: germ cell and sex cord-stromal tumors.
Gershenson DM
Gynecol Oncol 1994 Dec;55(3 Pt 2):S62-72. PMID: 7530680
Diagnosis and management of hirsute women.
Badawy SZ
Int J Fertil 1987 Sep-Oct;32(5):349-57. PMID: 2889680

Recent clinical studies

Etiology

Sertoli-Leydig cell tumor: a clinicopathological analysis in a comprehensive, national cohort.
Bekker P, Miland-Samuelsen AR, Smerdel MP, Schnack TH, Lauszus FF, Karstensen SH
Int J Gynecol Cancer 2023 Dec 4;33(12):1921-1927. doi: 10.1136/ijgc-2023-004945. PMID: 37907261
Imaging, clinical, and pathologic findings of Sertoli-leydig cell tumors.
Chen J, Liu Y, Zhang Y, Wang Y, Chen X, Wang Z
Sci Prog 2021 Apr-Jun;104(2):368504211009668. doi: 10.1177/00368504211009668. PMID: 33848213Free PMC Article
Ovarian sex cord-stromal tumours and their mimics.
Young RH
Pathology 2018 Jan;50(1):5-15. Epub 2017 Nov 11 doi: 10.1016/j.pathol.2017.09.007. PMID: 29132723
Gynecologic Manifestations of the DICER1 Syndrome.
Stewart CJ, Charles A, Foulkes WD
Surg Pathol Clin 2016 Jun;9(2):227-41. Epub 2016 Apr 9 doi: 10.1016/j.path.2016.01.002. PMID: 27241106
Sertoli-Leydig cell tumor.
Lantzsch T, Stoerer S, Lawrenz K, Buchmann J, Strauss HG, Koelbl H
Arch Gynecol Obstet 2001 Jan;264(4):206-8. doi: 10.1007/s004040000114. PMID: 11205710

Diagnosis

Sertoli-Leydig cell tumor: a clinicopathological analysis in a comprehensive, national cohort.
Bekker P, Miland-Samuelsen AR, Smerdel MP, Schnack TH, Lauszus FF, Karstensen SH
Int J Gynecol Cancer 2023 Dec 4;33(12):1921-1927. doi: 10.1136/ijgc-2023-004945. PMID: 37907261
Update on Ovarian Sex Cord-Stromal Tumors.
Ordulu Z
Clin Lab Med 2023 Jun;43(2):245-274. doi: 10.1016/j.cll.2023.03.001. PMID: 37169445
Ovarian Sertoli cell tumours: practical points.
Gheorghisan-Galateanu AA, Carsote M, Terzea D, Valea A, Ghemigian A
J Pak Med Assoc 2020 Jan;70(1):129-133. doi: 10.5455/JPMA.1381. PMID: 31954037
Ovarian sex cord-stromal tumours and their mimics.
Young RH
Pathology 2018 Jan;50(1):5-15. Epub 2017 Nov 11 doi: 10.1016/j.pathol.2017.09.007. PMID: 29132723
Sertoli-Leydig cell tumor.
Lantzsch T, Stoerer S, Lawrenz K, Buchmann J, Strauss HG, Koelbl H
Arch Gynecol Obstet 2001 Jan;264(4):206-8. doi: 10.1007/s004040000114. PMID: 11205710

Therapy

Granulosa and theca cell tumor in rat's ovary after modified Biskind operation.
Shamrai D, Koshik E, Melnyk N
Exp Oncol 2020 Dec;42(4):306-309. doi: 10.32471/exp-oncology.2312-8852.vol-42-no-4.15424. PMID: 33355865
INSL3 Expression in Leydig Cell Hyperplasia and Leydig Cell Tumors.
Lakis NS, Lombardo KA, Mangray S, Netto GJ, Salles D, Matoso A
Appl Immunohistochem Mol Morphol 2019 Mar;27(3):203-209. doi: 10.1097/PAI.0000000000000567. PMID: 29084059
Ovarian Sertoli-Leydig cell tumors: a single institution experience and review of the literature.
Gressel GM, Buza N, Pal L
Eur J Gynaecol Oncol 2017;38(2):214-220. PMID: 29953783
Clinicopathologic features of ovarian Sertoli-Leydig cell tumors.
Zhang HY, Zhu JE, Huang W, Zhu J
Int J Clin Exp Pathol 2014;7(10):6956-64. Epub 2014 Sep 15 PMID: 25400781Free PMC Article
Sertoli-Leydig cell tumor of the ovary: analysis of a single institution database.
Bhat RA, Lim YK, Chia YN, Yam KL
J Obstet Gynaecol Res 2013 Jan;39(1):305-10. Epub 2012 Jun 13 doi: 10.1111/j.1447-0756.2012.01928.x. PMID: 22691037

Prognosis

Review on Sertoli-Leydig Cell Tumours of the Ovary.
Muscat C, Calleja-Agius J
Discov Med 2024 Feb;36(181):234-247. doi: 10.24976/Discov.Med.202436181.22. PMID: 38409829
Sertoli-Leydig cell tumor: a clinicopathological analysis in a comprehensive, national cohort.
Bekker P, Miland-Samuelsen AR, Smerdel MP, Schnack TH, Lauszus FF, Karstensen SH
Int J Gynecol Cancer 2023 Dec 4;33(12):1921-1927. doi: 10.1136/ijgc-2023-004945. PMID: 37907261
Update on Ovarian Sex Cord-Stromal Tumors.
Ordulu Z
Clin Lab Med 2023 Jun;43(2):245-274. doi: 10.1016/j.cll.2023.03.001. PMID: 37169445
Ovarian Sertoli-Leydig cell tumors: a single institution experience and review of the literature.
Gressel GM, Buza N, Pal L
Eur J Gynaecol Oncol 2017;38(2):214-220. PMID: 29953783
Gynecologic Manifestations of the DICER1 Syndrome.
Stewart CJ, Charles A, Foulkes WD
Surg Pathol Clin 2016 Jun;9(2):227-41. Epub 2016 Apr 9 doi: 10.1016/j.path.2016.01.002. PMID: 27241106

Clinical prediction guides

Review on Sertoli-Leydig Cell Tumours of the Ovary.
Muscat C, Calleja-Agius J
Discov Med 2024 Feb;36(181):234-247. doi: 10.24976/Discov.Med.202436181.22. PMID: 38409829
Yolk Sac Tumor of the Ovary: A Report of 150 Cases and Review of the Literature.
Young RH, Wong A, Stall JN
Am J Surg Pathol 2022 Mar 1;46(3):309-325. doi: 10.1097/PAS.0000000000001793. PMID: 34469331
Imaging, clinical, and pathologic findings of Sertoli-leydig cell tumors.
Chen J, Liu Y, Zhang Y, Wang Y, Chen X, Wang Z
Sci Prog 2021 Apr-Jun;104(2):368504211009668. doi: 10.1177/00368504211009668. PMID: 33848213Free PMC Article
Ovarian Leydig cell tumor and postmenopausal hirsutism with signs of virilisation.
Ferrinho C, Silva E, Oliveira M, Sequeira Duarte J
BMJ Case Rep 2021 Mar 17;14(3) doi: 10.1136/bcr-2020-240937. PMID: 33731391Free PMC Article
Ovarian tumors: a survey of selected advances of note during the life of this journal.
Young RH
Hum Pathol 2020 Jan;95:169-206. Epub 2019 Oct 22 doi: 10.1016/j.humpath.2019.09.002. PMID: 31654691

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