Sertoli cell tumour | |
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Classification and external resources | |
Micrograph of a Sertoli cell tumour. H&E stain. |
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ICD-9 | 183.0, 256.1 |
ICD-O: | 8631 |
MeSH | D012707 |
A Sertoli cell tumour, also Sertoli cell tumor (US spelling), 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[1] but also in the ovary.[2]
A tumor that produces both Sertoli cells and Leydig cells is known as a Sertoli-Leydig cell tumor.
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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. A conclusive diagnosis is made via histology, as part of a pathology report made during or after surgery. See also Sex cord-stromal tumour.
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 Leydig cell tumour does not produce elevated tumour markers,[3] the focus of surveillance is on repeated physical examination and imaging.
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.[3]
Sertoli cell tumors are known to occur in other species, including domestic ducks,[4] dogs,[5][6] and horses.
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