Sertoli cell tumor
ICD-9:183.0 ICD-9:256.1 icd-0:8631 MeSH:D012707
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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.
Nota bene: A tumor that produces both Sertoli cells and Leydig cells is known as a Sertoli-Leydig cell tumor.
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.
Predispositions
Peutz-Jeghers syndrome
Localization
ovarian Sertoli cell tumor
testicular Sertoli cell tumor
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.
See also
testicular tumors
- Sertoli-stromal cell tumors
- Sertoli-Leydig cell tumors
Links
References
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#