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Ovarian Sertoli-Leydig cell tumor

MedGen UID:
2038
•Concept ID:
C0003810
•
Neoplastic Process
Synonyms: Androblastoma of ovary; Sertoli-leydig cell tumor of the ovary
SNOMED CT: Arrhenoblastoma of ovary (254866007); Sertoli-Leydig cell tumor of ovary (254866007)
 
Monarch Initiative: MONDO:0036595

Definition

A benign or malignant sex cord-stromal tumor arising from the ovary. It is characterized by the presence of neoplastic Leydig cells. Signs and symptoms include hirsutism, menorrhagia and metrorrhagia. It may be associated with trisomy 8. [from NCI]

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
In vitro production of cyclic AMP and steroids from an ovarian Sertoli-Leydig cell tumor. Notes on clinical management.
Abrahamsson G, Dahlgren E, Hahlin M, Knutson F, Norström A, Janson PO
Acta Obstet Gynecol Scand 1995 Apr;74(4):310-7. doi: 10.3109/00016349509024456. PMID: 7732806

Recent clinical studies

Etiology

An Ovarian Sertoli-Leydig Cell Tumor with Elevated Alpha-Fetoprotein in an Adolescent: A Rare Case Report and Literature Review.
Žilinskienė G, Bužinskienė D, Šidlovska E, Rudaitis V
Medicina (Kaunas) 2024 Sep 10;60(9) doi: 10.3390/medicina60091477. PMID: 39336518Free PMC Article
Outcomes in ovarian Sertoli-Leydig cell tumor: A report from the International Pleuropulmonary Blastoma/DICER1 and Ovarian and Testicular Stromal Tumor Registries.
Nelson AT, Harris AK, Watson D, Kamihara J, Chen KS, Stall JN, Devins KM, Young RH, Olson DR, Mallinger PHR, Mitchell SG, Hoffman LM, Halliday G, Suleymanova AM, Glade Bender JL, Messinger YH, Herzog CE, Field AL, Frazier AL, Stewart DR, Dehner LP, Hill DA, Billmire DF, Schneider DT, Schultz KAP
Gynecol Oncol 2024 Jul;186:117-125. Epub 2024 Apr 23 doi: 10.1016/j.ygyno.2024.04.005. PMID: 38657450Free PMC Article
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
Pediatric imaging in DICER1 syndrome.
Bueno MT, Martínez-Ríos C, la Puente Gregorio A, Ahyad RA, Villani A, Druker H, van Engelen K, Gallinger B, Aronoff L, Grant R, Malkin D, Greer MC
Pediatr Radiol 2017 Sep;47(10):1292-1301. Epub 2017 May 4 doi: 10.1007/s00247-017-3875-0. PMID: 28474256
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

Pediatric primary extra-ovarian Sertoli Leydig cell tumor of the retroperitoneum.
Panigrahi C, Mishra P, Pati AB, Das K, Sable M
Indian J Pathol Microbiol 2023 Jan-Mar;66(1):145-147. doi: 10.4103/ijpm.ijpm_677_21. PMID: 36656226
DICER1 Mutation in Recurrent Ovarian Sertoli-Leydig Cell Tumor: A Case Report.
Liu S, Pokoradi AJ, Soboleski D, Childs T, Agrawal A
J Pediatr Adolesc Gynecol 2023 Apr;36(2):107-111. Epub 2022 Nov 17 doi: 10.1016/j.jpag.2022.11.003. PMID: 36402443
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
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.
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

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
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
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
Cervical sarcoma botryoides and ovarian Sertoli-Leydig cell tumor: a case report and review of literature.
Rosenberg P, Carinelli S, Peiretti M, Zanagnolo V, Maggioni A
Arch Gynecol Obstet 2012 Mar;285(3):845-8. Epub 2011 Aug 17 doi: 10.1007/s00404-011-2017-4. PMID: 21847587

Prognosis

An Ovarian Sertoli-Leydig Cell Tumor with Elevated Alpha-Fetoprotein in an Adolescent: A Rare Case Report and Literature Review.
Žilinskienė G, Bužinskienė D, Šidlovska E, Rudaitis V
Medicina (Kaunas) 2024 Sep 10;60(9) doi: 10.3390/medicina60091477. PMID: 39336518Free PMC Article
Outcomes in ovarian Sertoli-Leydig cell tumor: A report from the International Pleuropulmonary Blastoma/DICER1 and Ovarian and Testicular Stromal Tumor Registries.
Nelson AT, Harris AK, Watson D, Kamihara J, Chen KS, Stall JN, Devins KM, Young RH, Olson DR, Mallinger PHR, Mitchell SG, Hoffman LM, Halliday G, Suleymanova AM, Glade Bender JL, Messinger YH, Herzog CE, Field AL, Frazier AL, Stewart DR, Dehner LP, Hill DA, Billmire DF, Schneider DT, Schultz KAP
Gynecol Oncol 2024 Jul;186:117-125. Epub 2024 Apr 23 doi: 10.1016/j.ygyno.2024.04.005. PMID: 38657450Free PMC Article
Sertoli Leydig Cell Tumour Initially Misdiagnosed as Polycystic Ovarian Syndrome and Congenital Adrenal Hyperplasia: A Case Report.
Paudyal P, Gurung G, Baral J, Kharel N
JNMA J Nepal Med Assoc 2020 Nov 22;58(231):923-926. doi: 10.31729/jnma.5045. PMID: 34506429Free PMC Article
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
Management of metastatic ovarian Sertoli-Leydig cell tumor with sporadic multinodular goiter: a case report and literature review.
Chi M, Gilman AD, Iroegbu N
Future Oncol 2011 Sep;7(9):1113-7. doi: 10.2217/fon.11.89. PMID: 21919698

Clinical prediction guides

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
Pediatric imaging in DICER1 syndrome.
Bueno MT, Martínez-Ríos C, la Puente Gregorio A, Ahyad RA, Villani A, Druker H, van Engelen K, Gallinger B, Aronoff L, Grant R, Malkin D, Greer MC
Pediatr Radiol 2017 Sep;47(10):1292-1301. Epub 2017 May 4 doi: 10.1007/s00247-017-3875-0. PMID: 28474256
Deep Sequencing Reveals Spatially Distributed Distinct Hot Spot Mutations in DICER1-Related Multinodular Goiter.
de Kock L, Bah I, Revil T, Bérubé P, Wu MK, Sabbaghian N, Priest JR, Ragoussis J, Foulkes WD
J Clin Endocrinol Metab 2016 Oct;101(10):3637-3645. Epub 2016 Jul 26 doi: 10.1210/jc.2016-1328. PMID: 27459524
The co-occurrence of an ovarian Sertoli-Leydig cell tumor with a thyroid carcinoma is highly suggestive of a DICER1 syndrome.
Durieux E, Descotes F, Mauduit C, Decaussin M, Guyetant S, Devouassoux-Shisheboran M
Virchows Arch 2016 May;468(5):631-6. Epub 2016 Mar 16 doi: 10.1007/s00428-016-1922-0. PMID: 26983701
Ovarian Sertoli-Leydig cell tumor (androblastoma) with retiform pattern. A clinicopathologic study.
Talerman A
Cancer 1987 Dec 15;60(12):3056-64. doi: 10.1002/1097-0142(19871215)60:12<3056::aid-cncr2820601233>3.0.co;2-r. PMID: 3677027

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