A Rare Case of Metastatic Primary Peritoneal Ependymoma: A Case Report and Literature Review

Abstract

Background. Primary peritoneal ependymoma is an exceedingly rare tumour with only four cases reported in the literature. It typically follows an indolent disease course. We describe a rare case of metastatic primary peritoneal ependymoma which was treated with chemotherapy and radiotherapy resulting in prolonged survival to date for 10 years. Case Presentation. The patient was a 23-year-old female on presentation. She presented with right upper quadrant pain associated with an abdominal mass. Computed tomography demonstrated a large mass displacing the liver. Debulking surgery was done revealing a tumour arising from the peritoneum as well as multiple metastatic pleural and peritoneal nodules. Pathology was consistent with primary peritoneal ependymoma. The patient was then treated with multiple lines of chemotherapy containing etoposide as the backbone. She also received palliative radiotherapy to the thoracic metastases with good and durable response. Conclusion. We reported a rare case of metastatic primary peritoneal ependymoma. Etoposide containing the chemotherapy regimen is effective in the treatment of peritoneal ependymoma. Radiotherapy is also effective for palliation of local symptoms with durable response.

‘Case of the Month’ from The University of Texas MD Anderson Cancer Center, Houston, Texas, USA: ependymoma of the urinary bladder

“…Extra‐CNS ‘ectopic’ ependymomas have been reported in the mediastinum, the ovary, and peritoneum and are believed to originate from embryonic remnants of the neural tube (ependymal rests). These extra‐neural lesions behave variably, with poor outcomes including recurrence and metastasis [6]. Treatment with a combination of surgery, chemotherapy, and radiotherapy has been reported, although early‐stage tumours arising from the ovary have been cured by surgical excision alone [7].…”

Primary Peritoneal Ependymoma: A Case Report and Literature Review

“…Primary peritoneal ependymomas are extremely rare, with 4 previous cases described (Table 1). 7–10 We present a case of primary peritoneal ependymoma discovered in a 58-year old woman after removal of a pelvic mass.…”