Sertoli cell tumor

Wikipedia

Definition: A Sertoli cell tumor is a sex cord-gonadal stromal tumor of a specific type that produces Sertoli cells. Although Sertoli cells normally occur only in the testis, this type of tumor can occur not only in the testis but also in the ovary.

Testicular Sertoli cell tumors are rare and usually sporadic and unifocal. The large cell calcifying Sertoli cell tumor variant is known to be associated with Carney and Peutz–Jeghers syndromes and can be bilateral in these patient populations.

Microscopy

Sertoli cell tumor has tubules lined by epithelial cells, but may also be diffuse or trabecular. They do not produce hormones. Large cell calcifying tumor is associated with Carney complex syndrome and Peutz-Jeghers syndrome.

Nota bene: A tumor that produces both Sertoli cells and Leydig cells is known as a Sertoli-Leydig cell tumor.

Bilateral Sertoli cell tumor

 Sertoli cell tumor with familial adenomatous polyposis (FAP)

• bilateral Sertoli cell tumor
• The tumors have a conventional Sertoli cell tumor morphology, but with different morphology in the left and right sites.
• Beta-catenin immunostain shows strong nuclear reactivity in the tumor cells but not the nonneoplastic Sertoli cells.
• The presence of bilaterality as well as overexpression of beta-catenin supports an association of the development of Sertoli cell tumor FAP syndrome and adenomatous polyposis coli inactivation.
• https://link.springer.com/article/10.1007%2Fs00428-012-1332-x

Localization

 ovarian Sertoli cell tumor
 testicular Sertoli cell tumor

Digital cases

 JRC:12840 : Testis Sertoli cell tumor.
 JRC:12844 : Testis Sertoli cell tumor.
 JRC:12846 : Testis Sertoli cell tumor.
 JRC:18198 : Androblastoma of ovary (Sertoli cell tumor) (Also known as luteinizing granulosa cell tumor of ovary).

Clinical synopsis

Due to excess testosterone secreted by the tumour, one-third of female patients present with a recent history of progressive masculinization.

Masculinization is preceded by anovulation, oligomenorrhea, amenorrhea and defeminization. Additional signs include acne and hirsutism, voice deepening, clitoromegaly, temporal hair recession, and an increase in musculature. Serum testosterone level is high.

Presence of an ovarian tumour plus hormonal disturbances suggests a Leydig cell tumour, granulosa cell tumour or thecoma. However, hormonal disturbances, in Leydig tumours, is present in only 2/3 of cases.

Predisposition

 Peutz-Jeghers syndrome

Microscopy

 Sertoli cell tumor shows closely packed hollow and solid tubules lined by well-differentiated cuboidal-to-columnar epithelial cells.
 Leydig cell are absent

Immunochemistry

 strong cytoplasmic staining for inhibin in tumoral Sertoli cells.
 WT1 immunostaining (17721194)

Localization

 ovarian Sertoli cell tumor
 testicular Sertoli cell tumor

Treatment and management

The usual treatment is surgery. The surgery usually is a fertility-sparing unilateral salpingo-oophorectomy.

For malignant tumours, the surgery may be radical and usually is followed by adjuvant chemotherapy, sometimes by radiation therapy.

In all cases, initial treatment is followed by surveillance.

Because in many cases Sertoli cell tumour does not produce elevated tumour markers, the focus of surveillance is on repeated physical examination and imaging.

Prognosis

The prognosis is generally good as the tumour tends to grow slowly and usually is benign: 25% are malignant. For malignant tumours with undifferentiated histology, prognosis is poor.

Y-chromosome FISH on Gonadal specimens

To evaluate fluorescence in situ hybridization (FISH) for SRY, the testis-determining gene on the Y-chromosome, in gonadal specimens from patients with intersex disorders including two older individuals presenting with Sertoli cell adenomas and clinically unsuspected androgen insensitivity syndrome (AIS). (18184266)

The demonstration of SRY using FISH is useful in the assessment of gonadal specimens from patients with intersex disorders, particularly in older individuals where the diagnosis may be unsuspected clinically. However, it may be necessary to examine multiple specimens in some cases to confirm the presence of Y-chromosomal material. (18184266)

See also

 testicular tumors

• Sertoli-stromal cell tumors
• Sertoli-Leydig cell tumors

Links

 Wikipedia

References

 Detection of Y-chromosome in gonadal tumours using fluorescence in situ hybridization: diagnostic value in intersex conditions including older patients with clinically unsuspected androgen insensitivity syndrome. Stewart CJ, Baker E, Beaton C, Crook M, Peverall J, Wallace S. Histopathology. 2008 Jan;52(2):175-82. PMID: 18184266

 Diagnostic utility of WT1 immunostaining in ovarian sertoli cell tumor. Zhao C, Bratthauer GL, Barner R, Vang R. Am J Surg Pathol. 2007 Sep;31(9):1378-86.PMID: 17721194