Multi-disciplinary treatment of a rare pelvic cavity ependymoma

Abstract

Ependymomas usually develop from neuroectodermal organs. Here, we present an ependymoma arising from the pelvic cavity. A 27-year-old Korean female was admitted to the hospital with a sensation of abdominal fullness. Imaging studies revealed a huge heterogeneous nodular mass in the pelvis and lower abdomen. Laparotomy showed that two large masses with multiple nodules were located between the uterus and rectum and uterus and bladder, respectively. Histologically, the tumor was characterized by compact columnar neoplastic cells divided by fibrovascular septae. The neoplastic cells formed true ependymal rosettes and perivascular pseudorosettes. Immunohistochemical staining showed a strong positive reaction for glial fibrillary acidic protein (GFAP) and vimentin and a partial positive reaction for S100 and EMA. The tumor was thus diagnosed as an ependymoma arising from the pelvic cavity. The patient was treated with a debulking operation and chemotherapy based upon the in vitro chemosensitivity test results. The patient was free of cancer for 4 years following surgery. This is a rare case of extraneural ependymoma for which an in vitro chemosensitivity test was critical in determining the multidisciplinary approach for treatment.

Fig. 1

Two 20 × 20cm and 8 × 8cm-sized large masses with clear cut margins were located between the uterus and rectum and the uterus and bladder, respectively (A and B). Carcinomatosis and seeding metastasis on Rt. subhepatic space were also observed (A and B). On gross pathology, the cut surface of one of the masses shows a pale brown fish flesh solid and partly white pale brown friable appearance with several hemorrhagic foci (C).

Fig. 2

Tumor cells are arranged in true perivascular pseudorosettes (A) and ependymal rosettes (B). Tumor cells show positive immunoreactivity for GFAP (C). Ultrastructural examination revealed compact arrangement of the tumor cells interlacing with the cytoplasmic process and abundant intermediate filaments in the cytoplasmic process. The presence of cilia in the cyst and intracytoplasmic lumen and intercellular junctions; Original magnification (D): × 12,000, right upper: × 30,000.