Clinical review: Functioning gonadotroph adenomas

CONTEXT: Functioning gonadotroph adenomas (FGAs) are pituitary tumors secreting biologically active gonadotropins. The published literature includes only small case series or individual case reports. This review summarizes the published data on this rare entity and, based on them, suggests guidance on the follow-up of these patients.

EVIDENCE ACQUISITION: A review of articles in English retrieved from the PubMed up to December 2013 was conducted. The following terms were used for the search: "functioning gonadotroph adenomas," "FSH secreting adenomas," "LH secreting adenomas," "gonadotroph adenomas," "ovarian hyperstimulation," "macroorchidism," "testicular enlargement," and "precocious puberty."

EVIDENCE SYNTHESIS: All reported cases of FGA were assessed, and information on presenting manifestations, management approaches, and long-term outcome was reviewed.

CONCLUSIONS: FGAs cause distinct manifestations and, based on the limited published literature, they are mostly macroadenomas. Their pathogenesis remains enigmatic. Systematic series on their optimal management are lacking, but the primary therapy remains surgical excision of the adenoma. Given the risk of recurrence, long-term clinical and imaging follow-up is needed, and radiotherapy may be required. There is little evidence that medical therapies are particularly helpful, certainly in terms of tumor control. Central registration would enhance our insight regarding their pathology and optimal management.