Extraspinal ependymoma of the broad ligament

Extraspinal ependymoma is a rare tumor, occurring most commonly in the sacrococcygeal region, and only a small number of cases have been reported to arise in the uterine ligament. Herein is reported a case of extraspinal ependymoma arising in the broad ligament of a 27-year-old woman. The lesion was 14 cm in diameter with an intra-abdominal implant in the omentum. On cut section the tumor was found to be solid, and demonstrated hemorrhaging, necrosis, myxoid foci, and central cystic spaces. Microscopically the tumor was composed of a proliferation of short spindle or polygonal cells arranged in short fascicles or in a solid sheet-like fashion with occasional perivascular pseudorosettes, together with myxoid areas and variable histological architectures exhibiting cribriform, pseudopapillary, and variable-sized cystic patterns. On immunohistochemistry most tumor cells were positively reactive to glial fibrillary acidic protein (GFAP), CD99, estrogen receptor, and progesterone receptor. The patient has remained disease-free for 6 months after the adjuvant chemoradiotherapy. Extraspinal ependymoma should be considered as a differential diagnosis when examining unusual intrapelvic tumors, especially in young female patients. The identification of characteristic histological features such as perivascular pseudorosettes and immunohistochemical expression of GFAP are helpful for confirming the diagnosis.