Sertoli Leydig cell tumour

The tumour is subdivided into many different subtypes. The most typical is composed of tubules lined by Sertoli cells and interstitial clusters of Leydig cells.

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.

Sertoli-Leydig cell tumour is a group of tumours 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 is a member of the sex cord-stromal tumour group of ovarian and testicular cancers.
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.

Presence of an ovarian tumour plus hormonal disturbances suggests a Sertoli-Leydig cell tumour. However, hormonal disturbance 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.