Sertoli–Leydig cell tumour

Sertoli–Leydig cell tumor is a group of tumors composed of variable proportions of Sertoli cells, Leydig cells, and in the case of intermediate and poorly differentiated neoplasms, primitive gonadal stroma and sometimes heterologous elements.High magnification micrograph of a Leydig cell tumour. H&E stain.High magnification micrograph of a Sertoli cell tumour. H&E stain. Sertoli–Leydig cell tumor is a group of tumors composed of variable proportions of Sertoli cells, Leydig cells, and in the case of intermediate and poorly differentiated neoplasms, primitive gonadal stroma and sometimes heterologous elements. Sertoli–Leydig cell tumour (a sex-cord stromal tumor), is a testostrone-secreting ovarian tumor and is a member of the sex cord-stromal tumour group of ovarian and testicular cancers. The tumour occurs in early adulthood (not seen in newborn), is rare, comprising less than 1% of testicular tumours. While the tumour can occur at any age, it occurs most often in young adults. Recent studies have shown that many cases of Sertoli–Leydig cell tumor of the ovary are caused by germline mutations in the DICER1 gene. These hereditary cases tend to be younger, often have a multinodular thyroid goiter and there may be a personal or family history of other rare tumors such as pleuropulmonary blastoma, Wilms tumor and cervical rhabdomyosarcoma. Closely related terms include arrhenoblastoma and androblastoma. Both terms are classified under Sertoli–Leydig cell tumour in MeSH. Due to excess testosterone secreted by the tumour, one-third of adult females present with a recent history of progressive masculinization. Masculinization is preceded by anovulation, oligomenorrhoea, amenorrhoea and defeminization. Additional signs include acne and hirsutism, voice deepening, clitoromegaly, temporal hair recession, and an increase in musculature. Serum testosterone level is high.