Perivascular Epithelioid Cell Tumor of the Cervix with Metastasis to the Brain

Perivascular Epithelioid Cell tumors (PEComas) are rare mesenchymal neoplasms with distinctive histologic and immunohistochemical profiles that exhibit a predominantly perivascular distribution of neoplastic cells. The rarity and paucity of characteristic imaging features of PEComas, particularly in the gynecologic organs, pose a diagnostic challenge. PEComas have been reported at many anatomic locations including uterus, vulva, and retroperitoneum, with some cases associated with the tuberous sclerosis complex. PEComas of the uterus are especially difficult to diagnose preoperatively and are often found incidentally postoperatively. This case report represents the fourth reported case of a PEComa metastasizing to the brain, and the first case of a cervical PEComa diagnosed with brain metastasis. A 65-year-old postmenopausal woman presented to the clinic with postmenopausal vaginal bleeding, and she was found to have masses in the cervix and uterus. The patient had no known genetic predispositions such as tuberous sclerosis complex (TSC) and declined genetic testing. A cervical mass was eventually diagnosed as a PEComa, following histopathological and immunohistochemical analysis. While initially successful, the patient later developed neurologic symptoms, and imaging revealed a brain metastasis. Craniotomy with gross total resection of the metastatic lesion was performed, and the pathology confirmed that the brain lesion originated from the cervical PEComa. This report highlights the unpredictable metastatic behavior of PEComas, even to the brain, and the importance of rapid and accurate diagnosis, followed by extensive surgical resection of the primary tumor and any metastatic foci. Additional research is required to better understand the biology of PEComas, to improve diagnosis, and to develop treatment strategies, especially for the group with evidence of metastatic disease.