Intracranial Meningiomas in Patients with Uterine Sarcoma Treated with Long-Term Megestrol Acetate Therapy

OBJECTIVE: To report a series of four patients with uterine sarcoma, including one with mullerian adenosarcoma (MA) and three with low-grade endometrial stromal sarcoma (LGESS), who developed intracranial meningiomas while receiving the progesterone agonist megestrol acetate. METHODS: The hospital records, imaging studies, and pathology slides of four patients who were treated for uterine sarcomas and subsequently developed intracranial meningiomas were reviewed. RESULTS: All patients underwent surgery for their gynecologic cancers and received maintenance therapy with long-term hormonal suppression with megestrol acetate. Each of the four patients later developed neurologic symptoms secondary to intracranial meningiomas. Three patients had more than one meningioma. Histopathologic examination of all excised tumors showed strong immunoreactivity for progesterone receptors (PRs). CONCLUSIONS: Patients with uterine sarcoma subtypes LGESS and MA may be predisposed to develop meningiomas, particularly in the setting of long-term treatment with megestrol acetate. Alternatively, preexisting, clinically silent meningiomas in these patients may have progressed to the point of clinical symptoms in the presence of the progesterone agonist megestrol acetate. Without previous imaging studies showing the presence or absence of meningioma before initiation of megestrol acetate treatment, there is no way to draw definitive conclusions regarding this possibility. Clinical and neuroradiologic surveillance for meningiomas should be strongly considered in patients with these uterine sarcoma subtypes, particularly in patients undergoing long-term suppressive therapy with megestrol acetate.