Subcutaneous sacrococcygeal myxopapillary ependymoma misdiagnosed as pilonidal disease

Abstract

Ependymomas are neoplasms which arise from the radial glial cells, which many recent studies have proposed are neural stem cells. Extracranial ependymomas are rare. We present the case report and supporting multimedia of a 37-year-old man who presented with a painless intergluteal swelling which was diagnosed clinically as a pilonidal cyst. However, on excision, he was found to have a subcutaneous sacrococcygeal myxopapillary ependymoma based on histological findings. His management and follow-up are presented and discussed. Given the rare nature of this condition, there is a lack of published guidelines on management and follow-up protocols. Supporting evidence is limited to sporadic case reports. This case highlights the diagnostic challenges and management strategies adopted supported by the best available evidence.

Subcutaneous Sacro Coccygeal Myxopapillary Ependymoma: A Case Report and a Comprehensive Review of the Literature Reappraising Its Current Diagnostic Approach and Management

“…Postsacral ependymomas usually present as subcutaneous tissue tumors primary to the sacrococcygeal skin with no discernible relation to the filum terminale or spinal cord [ 16 ]. Postsacral ependymomas can also form near the gluteal cleft and can be mistakenly diagnosed as a pilonidal disease [ 2 ]. Pilonidal sinus, epidermal inclusion cyst, meningocele, lipoma, sacrococcygeal teratoma, and neurogenic tumors are all differential diagnoses for sacrococcygeal lesions [ 8 ].…”

“…While these lesions account for 60% of glial tumors originating in the spine, the overall incidence is extremely low. Recent studies have proposed that these neoplasms arise from radial glia, cells that act as scaffolds for cell migration during CNS development and then normally differentiate into neurons and other mature glial cells [ 2 ]. Myxopapillary variants of ependymomas are also rare.…”

“…Extraspinal ependymomas arising in the sacrococcygeal region are also observed, as either presacral or postsacral masses. While rare, these show a stronger propensity to spread systemically through lymphovascular dissemination [ 2 , 4 , 5 ]. Presacral masses can exert pressure on the bowel or bladder, while postsacral masses can present as intergluteal swellings [ 2 , 5 , 6 ].…”

“…While rare, these show a stronger propensity to spread systemically through lymphovascular dissemination [ 2 , 4 , 5 ]. Presacral masses can exert pressure on the bowel or bladder, while postsacral masses can present as intergluteal swellings [ 2 , 5 , 6 ]. The differential diagnoses for these sacrococcygeal masses also include pilonidal sinus, neurogenic tumor, sacrococcygeal teratoma, soft tissue sarcoma, and metastatic carcinoma [ 3 ].…”

Extraspinal sacrococcygeal myxopapillary ependymoma in a teenager

“…On physical examination, a few nodules were palpable near the anus, with no redness or discharge. Suspecting ing antibiotics or percutaneous aspiration [3,4]. Myxopapillary ependymoma (ME) has some unique histologic features [5].…”