Central nervous system involvement from malignant mixed Müllerian tumor (MMMT) of the uterus

The central nervous system is traditionally considered as an uncommon site for metastatic disease from the female genital tract, and cerebral metastasis as the primary manifestation of an occult gynecological malignancy is even more rare. Here, we report the case of a 61-year-old female who presented with neurological symptoms of confusion, headache, cerebellar ataxia and right-sided weakness. Magnetic resonance imaging of the brain revealed two solid lesions in the frontal lobe and the left cerebellar hemisphere. Endometrial biopsy of a uterine mass detected during search for the primary lesion showed malignant mixed Müllerian tumor (MMMT). The patient refused surgery. Cranial radiotherapy for progressive cerebral disease led to resolution of her neurological symptoms. Two months after the diagnosis of MMMT the patient died from local complications of advanced pelvic disease. At autopsy, only the epithelial component of the tumor had metastasized to the brain. Attention should be paid to possibility of unusual distant metastases associated to MMMT in order to avoid delay in diagnosis and treatment of these patients.