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Sertoli cell tumor

MedGen UID:: 11387
•Concept ID:: C0036769
•: Neoplastic Process

Synonyms:	Sertoli Cell Tumor; Tumor, Sertoli Cell
SNOMED CT:	Sertoli cell adenoma (89089007); Testicular adenoma (89089007); Sertoli cell tumor (89089007); Tubular androblastoma (89089007); Pick tubular adenoma (89089007)

Monarch Initiative:	MONDO:0002696

Definition

A sex cord-stromal tumor of the testis or the ovary. It is characterized by the presence of Sertoli cells forming tubules. Leydig cells are rare or absent. It may be associated with Peutz-Jeghers syndrome. In males, the presenting symptom is a slow growing testicular mass. Most cases follow a benign clinical course. In females it may present with estrogenic or androgenic manifestations. The vast majority of cases have a benign clinical course. [from NCI]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVSertoli cell tumor

Pathological Conditions, Signs and Symptoms
- Pathological process
  - Disease
    - Neoplasm
      - Sertoli cell tumor
        Benign sertoli cell tumor
        Benign testicular sertoli cell tumor
        Testicular Sclerosing Sertoli Cell Tumor
        Testicular Sertoli Cell Tumor, Lipid Rich Variant
        Malignant Sertoli cell tumor
        Malignant Testicular Sertoli Cell Tumor
        Ovarian Sertoli Cell Tumor
        Ovarian Sertoli Cell Tumor with Lipid Storage
        Testicular sertoli cell tumor
        Testicular Large Cell Calcifying Sertoli Cell Tumor

Professional guidelines

PubMed

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

Peutz-Jeghers Syndrome: Pathobiology, Pathologic Manifestations, and Suggestions for Recommending Genetic Testing in Pathology Reports.

Meserve EE, Nucci MR
Surg Pathol Clin 2016 Jun;9(2):243-68. doi: 10.1016/j.path.2016.01.006. PMID: 27241107

Clinical and molecular features of the Carney complex: diagnostic criteria and recommendations for patient evaluation.

Stratakis CA, Kirschner LS, Carney JA
J Clin Endocrinol Metab 2001 Sep;86(9):4041-6. doi: 10.1210/jcem.86.9.7903. PMID: 11549623

See all (8)

Recent clinical studies

Etiology

Comprehensive review of imaging features of sex cord-stromal tumors of the ovary.

Javadi S, Ganeshan DM, Jensen CT, Iyer RB, Bhosale PR
Abdom Radiol (NY) 2021 Apr;46(4):1519-1529. Epub 2021 Mar 16 doi: 10.1007/s00261-021-02998-w. PMID: 33725145

Sertoli cell tumor and intratubular germ cell neoplasia located in separate gonads in an adolescent patient with complete androgen insensitivity: a case report and review of literature.

Lin MH, Shamszadeh M, Pitukcheewanont P
J Pediatr Endocrinol Metab 2012;25(5-6):547-51. doi: 10.1515/jpem-2012-0026. PMID: 22876554

Bilateral large-cell calcifying Sertoli cell tumor of the testes with Peutz-Jeghers syndrome: a case report.

Dreyer L, Jacyk WK, du Plessis DJ
Pediatr Dermatol 1994 Dec;11(4):335-7. doi: 10.1111/j.1525-1470.1994.tb00100.x. PMID: 7899185

Testicular tumors other than germ cell tumors.

Young RH, Talerman A
Semin Diagn Pathol 1987 Nov;4(4):342-60. PMID: 2832918

Sex cord-stromal tumours of the ovary.

Fox H
J Pathol 1985 Feb;145(2):127-48. doi: 10.1002/path.1711450202. PMID: 2983057

See all (86)

Diagnosis

Large-Cell Calcifying Sertoli Cell Tumor.

Gupta S, Erickson LA
Mayo Clin Proc 2024 Aug;99(8):1347-1348. doi: 10.1016/j.mayocp.2024.05.031. PMID: 39097341

Sertoli-Leydig cell tumor.

Villegas Cruz C, Berlanga Narro S, Saltijeral SN, Roman Montalvo MA
Int J Gynecol Cancer 2024 Sep 2;34(9):1476-1478. doi: 10.1136/ijgc-2023-004321. PMID: 38088150

Practical Review of Ovarian Sex Cord-Stromal Tumors.

Hanley KZ, Mosunjac MB
Surg Pathol Clin 2019 Jun;12(2):587-620. doi: 10.1016/j.path.2019.02.005. PMID: 31097116

Testicular tumours.

Peckham MJ, McElwain TJ
Clin Endocrinol Metab 1975 Nov;4(3):665-92. doi: 10.1016/s0300-595x(75)80052-1. PMID: 58746

Testicular tumors.

Markland C
Curr Probl Surg 1968 Sep:3-51. doi: 10.1016/s0011-3840(68)80001-2. PMID: 4386486

See all (255)

Therapy

Large-cell calcifying Sertoli cell tumors of the testes in pediatrics.

Gourgari E, Saloustros E, Stratakis CA
Curr Opin Pediatr 2012 Aug;24(4):518-22. doi: 10.1097/MOP.0b013e328355a279. PMID: 22732638 Free PMC Article

Phase I trial of sulofenur (LY186641) given orally on a daily x 21 schedule.

Brown TD, O'Rourke TJ, Kuhn JG, Craig JB, Havlin K, Burris HA 3rd, Cagnola J, Hamilton JM, Grindey GB, Satterlee WG
Anticancer Drugs 1994 Apr;5(2):151-9. doi: 10.1097/00001813-199404000-00005. PMID: 8049498

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

Management of germ cell and stromal tumors of the ovary.

Slayton RE
Semin Oncol 1984 Sep;11(3):299-313. PMID: 6091275

Testicular tumours.

Peckham MJ, McElwain TJ
Clin Endocrinol Metab 1975 Nov;4(3):665-92. doi: 10.1016/s0300-595x(75)80052-1. PMID: 58746

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Prognosis

Characteristic ultrasound appearance of a rare testis tumor: Bilateral multiple large-cell calcifying Sertoli cell tumor.

Ayaz E, Okur N, Çelebi C, Özekinci SÖ
J Clin Ultrasound 2023 Oct;51(8):1419-1421. Epub 2023 Aug 16 doi: 10.1002/jcu.23535. PMID: 37584318

Comprehensive review of imaging features of sex cord-stromal tumors of the ovary.

Javadi S, Ganeshan DM, Jensen CT, Iyer RB, Bhosale PR
Abdom Radiol (NY) 2021 Apr;46(4):1519-1529. Epub 2021 Mar 16 doi: 10.1007/s00261-021-02998-w. PMID: 33725145

Practical Review of Ovarian Sex Cord-Stromal Tumors.

Hanley KZ, Mosunjac MB
Surg Pathol Clin 2019 Jun;12(2):587-620. doi: 10.1016/j.path.2019.02.005. PMID: 31097116

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 sex cord-stromal tumors: recent progress.

Young RH, Scully RE
Int J Gynecol Pathol 1982;1(1):101-23. doi: 10.1097/00004347-198201000-00011. PMID: 6307900

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Clinical prediction guides

The sclerosing sertoli cell tumor of the testis: a case report.

Tang X, Hu Y, Zhou H, Zhou Y
Diagn Pathol 2023 May 15;18(1):61. doi: 10.1186/s13000-023-01351-7. PMID: 37189109 Free PMC Article

Large cell calcifying Sertoli cell tumour: a contemporary multi-institutional case series highlighting the diagnostic utility of PRKAR1A immunohistochemistry.

Anderson WJ, Gordetsky JB, Idrees MT, Al-Obaidy KI, Kao CS, Cornejo KM, Wobker SE, Cheville JC, Vargas SO, Fletcher CDM, Hirsch MS, Acosta AM
Histopathology 2022 Mar;80(4):677-685. Epub 2022 Jan 5 doi: 10.1111/his.14599. PMID: 34780072

Sertoli Cell Tumors of the Testes: Systematic Literature Review and Meta-Analysis of Outcomes in 435 Patients.

Grogg J, Schneider K, Bode PK, Kranzbühler B, Eberli D, Sulser T, Lorch A, Beyer J, Hermanns T, Fankhauser CD
Oncologist 2020 Jul;25(7):585-590. Epub 2020 Feb 11 doi: 10.1634/theoncologist.2019-0692. PMID: 32043680 Free PMC Article

Recently Described and Clinically Important Entities in Testis Tumors: A Selective Review of Changes Incorporated Into the 2016 Classification of the World Health Organization.

Ulbright TM
Arch Pathol Lab Med 2019 Jun;143(6):711-721. Epub 2018 Jun 27 doi: 10.5858/arpa.2017-0478-RA. PMID: 29949388

Phase I trial of sulofenur (LY186641) given orally on a daily x 21 schedule.

See all (73)