Primary extraneural ependymoma of the broad ligament: A rare entity

Extraneural broad ligament ependymoma is a rare entity. Herein, we present a case of unusually large broad ligament ependymoma in a 32-year-old female with pain and lump in the lower abdomen. Contrast-enhanced computed tomography abdomen revealed multiple heterogeneously enhancing pelvic masses with lobulated surface in bilateral adnexa along with multiple peritoneal nodules. Her relevant serum tumor markers were unremarkable. Core biopsy revealed tumor composed of elongated cells arranged predominantly in true and pseudoperivascular rosettes. The histopathological differentials included ependymoma, primitive neuroectodermal tumor, and teratoma with neural differentiation. Results of immunohistochemistry favored the diagnosis of ependymoma. Surgical exploration and optimal cytoreduction were done, and a final diagnosis of primary broad ligament ependymoma with peritoneal metastasis was made. The patient received six cycles of adjuvant chemotherapy and is doing well after 8-month follow-up. The present case highlights the diagnostic workup and management of a rare and an unusually large broad ligament ependymoma with peritoneal metastasis.