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Sertoli-Leydig cell tumor

MedGen UID:
61669
•Concept ID:
C0206723
•
Neoplastic Process
Synonyms: Androblastoma; Androblastomas; Arrhenoblastoma; Arrhenoblastomas; Cell Tumor, Sertoli-Leydig; Cell Tumors, Sertoli-Leydig; Sertoli Leydig Cell Tumor; Sertoli Leydig Cell Tumors; Sertoli-Leydig Cell Tumor; Sertoli-Leydig Cell Tumors; Tumor, Sertoli-Leydig Cell; Tumors, Sertoli-Leydig Cell
 
Monarch Initiative: MONDO:0002479

Definition

A sex cord-gonadal stromal tumor consists of leydig cells; sertoli cells; and fibroblasts in varying proportions and degree of differentiation. Most such tumors produce androgens in the Leydig cells, formerly known as androblastoma or arrhenoblastoma. Androblastomas occur in the testis or the ovary causing precocious masculinization in the males, and defeminization, or virilization (virilism) in the females. In some cases, the Sertoli cells produce estrogens. [from MONDO]

Professional guidelines

PubMed

Embryonal Rhabdomyosarcoma of the Uterine Cervix: A Clinicopathologic Study of 94 Cases Emphasizing Issues in Differential Diagnosis Staging, and Prognostic Factors.
Devins KM, Young RH, Ghioni M, Burandt E, Bennett JA, Oliva E
Am J Surg Pathol 2022 Nov 1;46(11):1477-1489. Epub 2022 Aug 9 doi: 10.1097/PAS.0000000000001933. PMID: 35941719
DICER1-associated Tumors in the Female Genital Tract: Molecular Basis, Clinicopathologic Features, and Differential Diagnosis.
Han LM, Weiel JJ, Longacre TA, Folkins AK
Adv Anat Pathol 2022 Sep 1;29(5):297-308. Epub 2022 Jul 1 doi: 10.1097/PAP.0000000000000351. PMID: 35778792
DICER1-related Sertoli-Leydig cell tumor and gynandroblastoma: Clinical and genetic findings from the International Ovarian and Testicular Stromal Tumor Registry.
Schultz KAP, Harris AK, Finch M, Dehner LP, Brown JB, Gershenson DM, Young RH, Field A, Yu W, Turner J, Cost NG, Schneider DT, Stewart DR, Frazier AL, Messinger Y, Hill DA
Gynecol Oncol 2017 Dec;147(3):521-527. Epub 2017 Oct 14 doi: 10.1016/j.ygyno.2017.09.034. PMID: 29037807Free PMC Article

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
Sertoli-Leydig cell tumor of the ovary: Analysis of a single institution database and review of the literature.
Durmuş Y, Kılıç Ç, Çakır C, Yüksel D, Boran N, Karalök A, Boyraz G, Turan AT
J Obstet Gynaecol Res 2019 Jul;45(7):1311-1318. Epub 2019 May 20 doi: 10.1111/jog.13977. PMID: 31106943
DICER1 and Associated Conditions: Identification of At-risk Individuals and Recommended Surveillance Strategies.
Schultz KAP, Williams GM, Kamihara J, Stewart DR, Harris AK, Bauer AJ, Turner J, Shah R, Schneider K, Schneider KW, Carr AG, Harney LA, Baldinger S, Frazier AL, Orbach D, Schneider DT, Malkin D, Dehner LP, Messinger YH, Hill DA
Clin Cancer Res 2018 May 15;24(10):2251-2261. Epub 2018 Jan 17 doi: 10.1158/1078-0432.CCR-17-3089. PMID: 29343557Free PMC Article
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
Update on Ovarian Sex Cord-Stromal Tumors.
Ordulu Z
Surg Pathol Clin 2022 Jun;15(2):235-258. doi: 10.1016/j.path.2022.02.004. PMID: 35715160
DICER1 and Associated Conditions: Identification of At-risk Individuals and Recommended Surveillance Strategies.
Schultz KAP, Williams GM, Kamihara J, Stewart DR, Harris AK, Bauer AJ, Turner J, Shah R, Schneider K, Schneider KW, Carr AG, Harney LA, Baldinger S, Frazier AL, Orbach D, Schneider DT, Malkin D, Dehner LP, Messinger YH, Hill DA
Clin Cancer Res 2018 May 15;24(10):2251-2261. Epub 2018 Jan 17 doi: 10.1158/1078-0432.CCR-17-3089. PMID: 29343557Free PMC Article
Pediatric Gynecologic Cancers.
Pommert L, Bradley W
Curr Oncol Rep 2017 Jul;19(7):44. doi: 10.1007/s11912-017-0604-7. PMID: 28501984

Therapy

Characteristics and outcomes analysis of ovarian Sertoli-Leydig cell tumors (SLCTs): analysis of 15 patients.
Wang G, Zhang R, Li C, Chen A
J Ovarian Res 2021 Nov 4;14(1):150. doi: 10.1186/s13048-021-00909-7. PMID: 34736487Free PMC Article
Sertoli-Leydig Cell Tumors of the Ovary With Follicular Differentiation Often Resembling Juvenile Granulosa Cell Tumor: A Report of 38 Cases Including Comments on Sex Cord-Stromal Tumors of Mixed Forms (So-called Gynandroblastoma).
Ordulu Z, Young RH
Am J Surg Pathol 2021 Jan;45(1):59-67. doi: 10.1097/PAS.0000000000001544. PMID: 32815828
Ovarian Sertoli-Leydig Cell Tumor With Heterologous Hepatocytes and a Hepatocellular Carcinomatous Element.
Yamamoto S, Sakai Y
Int J Gynecol Pathol 2019 May;38(3):247-252. doi: 10.1097/PGP.0000000000000499. PMID: 29620585
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
Ovarian Sertoli-Leydig cell tumor: a report of seven cases and a review of the literature.
Xiao H, Li B, Zuo J, Feng X, Li X, Zhang R, Wu L
Gynecol Endocrinol 2013 Mar;29(3):192-5. Epub 2012 Nov 23 doi: 10.3109/09513590.2012.738723. PMID: 23173550

Prognosis

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
Update on Ovarian Sex Cord-Stromal Tumors.
Ordulu Z
Surg Pathol Clin 2022 Jun;15(2):235-258. doi: 10.1016/j.path.2022.02.004. PMID: 35715160
DICER1 tumor predisposition syndrome: an evolving story initiated with the pleuropulmonary blastoma.
González IA, Stewart DR, Schultz KAP, Field AP, Hill DA, Dehner LP
Mod Pathol 2022 Jan;35(1):4-22. Epub 2021 Oct 1 doi: 10.1038/s41379-021-00905-8. PMID: 34599283Free PMC Article
Ovarian Sertoli-Leydig cell tumours: A systematic review of relapsed cases.
Nef J, Huber DE
Eur J Obstet Gynecol Reprod Biol 2021 Aug;263:261-274. Epub 2021 Jun 28 doi: 10.1016/j.ejogrb.2021.06.036. PMID: 34245994

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
Characteristics and outcomes analysis of ovarian Sertoli-Leydig cell tumors (SLCTs): analysis of 15 patients.
Wang G, Zhang R, Li C, Chen A
J Ovarian Res 2021 Nov 4;14(1):150. doi: 10.1186/s13048-021-00909-7. PMID: 34736487Free PMC Article
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
Imaging of DICER1 syndrome.
Guillerman RP, Foulkes WD, Priest JR
Pediatr Radiol 2019 Oct;49(11):1488-1505. Epub 2019 Oct 16 doi: 10.1007/s00247-019-04429-x. PMID: 31620849
Ovarian Sex Cord-Stromal Tumors.
Schultz KA, Harris AK, Schneider DT, Young RH, Brown J, Gershenson DM, Dehner LP, Hill DA, Messinger YH, Frazier AL
J Oncol Pract 2016 Oct;12(10):940-946. doi: 10.1200/JOP.2016.016261. PMID: 27858560Free PMC Article

Recent systematic reviews

Ovarian Sertoli-Leydig cell tumours: A systematic review of relapsed cases.
Nef J, Huber DE
Eur J Obstet Gynecol Reprod Biol 2021 Aug;263:261-274. Epub 2021 Jun 28 doi: 10.1016/j.ejogrb.2021.06.036. PMID: 34245994
Multimorbidity and Genetic Characteristics of DICER1 Syndrome Based on Systematic Review.
Cai S, Zhao W, Nie X, Abbas A, Fu L, Bihi S, Feng G, Liu T, Lv Y, Ma X, Peng X
J Pediatr Hematol Oncol 2017 Jul;39(5):355-361. doi: 10.1097/MPH.0000000000000715. PMID: 27906793
Feto-maternal outcomes of pregnancy complicated by ovarian sex-cord stromal tumor: a systematic review of literature.
Blake EA, Carter CM, Kashani BN, Kodama M, Mabuchi S, Yoshino K, Matsuo K
Eur J Obstet Gynecol Reprod Biol 2014 Apr;175:1-7. Epub 2013 Dec 25 doi: 10.1016/j.ejogrb.2013.12.025. PMID: 24439718

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