Extraspinal ependymoma D43.4

Ependymomas belong to the gliomas and occur intracranially, but mainly in the spinal canal. They arise from the cells of the ependyma, a layer that separates the cerebrospinal fluid from the cranial nerve tissue and also lines the spinal canal. Extraspinal ependymomas are very rare.

presacral subcutis

Clinically, they impress as a soft, painless, skin-colored lump, which is interpreted as a pilonidal sinus, teratoma or adnexal tumor.

Glial tumor with ependymal differentiation and typical rosette structure. Most cases show immunoreactivity for glial fibrillary acidic protein (GFAP), S-100 protein and keratin (AE1AE3).

Surgically, resection as complete as possible. There is a not inconsiderable risk of recurrence (Tröbs RB et al. 2006).

The tumors are mostly benign. Malignant forms occur in 10-15% of all cases.

1. Goto K et al (2021) GFAP-negative subcutaneous sacrococcygeal extraspinal ependymoma. Case Rep Dermatol 13:293-297.

2. Tröbs RB et al (2006) Sacrococcygeal extraspinal myxopapillary ependymoma. Clin Padiatr 218:243-245.