Sertoli-Leydig cell tumour

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Sertoli-Leydig cell tumour

A sex-cord/stromal tumour affecting both the testis and ovaries, which is characterised by a mixed population of Sertoli and Leydig cells, of which the Leydig cells produce and secrete testosterone, causing virilisation.

Molecular pathology
Some cases are linked a mutation on DICER1, a gene on chromosome 14q32.13 which encodes a protein that acts as a ribonuclease and is required by the RNA interference and small temporal RNA (stRNA) pathways to produce the active small RNA component which represses gene expression. DICER1 cases occur in younger patients with multinodular goiter, and have an increased risk of primitive tumours—e.g., pleuropulmonary blastoma, Wilms tumor and rhabdomyosarcoma of the cervix.

Conservative surgery, especially in young women.

Generally good; clinical malignancy develops in ±20%.