Sertoli-Leydig cell tumors are a cancer that starts in the female ovaries. The cancer cells produce and release a male sex hormone which may cause the development of male physical characteristics (virilization), including facial hair and a deep voice. This type of tumor is sometimes called arrhenoblastoma of the ovary or a stromal tumor. Sertoli-Leydig cell tumors are rare tumors which account for less than 0.5% of all ovarian tumors. While they can be found in women of all age groups, they are most common in young women. Treatment involves surgery and chemotherapy or radiation if the cancer has spread. Because Sertoli-Leydig cell tumors have a low chance of spreading, if the tumor is found early, chances for survival are good.

There is evidence that some Sertoli-Leydig cell tumors of the ovary might be caused by a genetic predisposition to develop cancer. Mutations in the DICER1 gene have been associated with an increased chance to develop Sertoli-Leydig cell tumors of the ovary, as well as other types of cancer. It appears that mutations in DICER1 can be passed from a parent to a child in an autosomal dominant manner.

Because Sertoli-Leydig cell tumors of the ovary are rare, there are no established guidelines for treatment. Treatment decisions are based on the unique characteristics of each tumor, particularly the stage. Surgery to remove the tumor is considered the best treatment for an early-stage tumor, whereas surgery and chemotherapy may be needed to treat a later-stage tumor.

The chance of surviving a Sertoli-Leydig cell tumor of the ovary is quite high. The outlook is most favorable for individuals diagnosed at an early stage, meaning the cancerous cells have not spread into nearby healthy organs. One study showed that 92% of individuals diagnosed with a Sertoli-Leydig cell tumor at an early stage were alive five years after their diagnosis compared to 33% of individuals with tumors diagnosed at later stages.