Pediatric Extraspinal Sacrococcygeal Ependymoma: Report of Two Cases and Literature Review

Abstract

Primary central nervous system (CNS) tumors represent the most common solid tumors in childhood. Ependymomas arise from ependymal cells lining the wall of ventricles or central canal of spinal cord and their occurrence outside the CNS is extremely rare, published in the literature as case reports or small case series. We present two cases of extra-CNS myxopapillary ependymomas treated at our institution in the past three years; both cases originate in the sacrococcygeal region and were initially misdiagnosed as epidermoid cyst and germ cell tumor, respectively. The first case, which arose in a 9-year-old girl, was treated with a surgical excision in two stages, due to the non-radical manner of the first operation; no recurrence was observed after two years of follow-up. The other case was a 12-year-old boy who was treated with a complete resection and showed no evidence of recurrence at one-year follow-up. In this paper, we report our experience in treating an extremely rare disease that lacks a standardized approach to diagnosis, treatment and follow-up; in addition, we perform a literature review of the past 35 years.

Extraspinal sacrococcygeal myxopapillary ependymoma in a teenager

“…ESME is a rare glial tumor, and presented in an unusual location in our case. Since the first case reported by Mallory [ 1 ], less than 50 cases occurring in childhood have been reported in the literature [ 2 ]. Because of its rarity, the tumor is sometimes misdiagnosed as pilonidal abscess or detected only after treatment, including antibiotics or percutaneous aspiration [ 3 , 4 ].…”

“…There is no consensus on standardized therapy for ESME, but complete excision is recommended [ 2 ]. Radiotherapy can improve progression-free survival in spinal ependymoma and a similar result can be expected in ESME, especially in unresectable or partially excised cases; however, further investigations are required.…”