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Extraspinal ependymomaD43.4
Synonym(s)
DefinitionThis section has been translated automatically.
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.
LocalizationThis section has been translated automatically.
presacral subcutis
Clinical featuresThis section has been translated automatically.
Clinically, they impress as a soft, painless, skin-colored lump, which is interpreted as a pilonidal sinus, teratoma or adnexal tumor.
HistologyThis section has been translated automatically.
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).
TherapyThis section has been translated automatically.
Surgically, resection as complete as possible. There is a not inconsiderable risk of recurrence (Tröbs RB et al. 2006).
Progression/forecastThis section has been translated automatically.
The tumors are mostly benign. Malignant forms occur in 10-15% of all cases.
LiteratureThis section has been translated automatically.
Goto K et al (2021) GFAP-negative subcutaneous sacrococcygeal extraspinal ependymoma. Case Rep Dermatol 13:293-297.
- Tröbs RB et al (2006) Sacrococcygeal extraspinal myxopapillary ependymoma. Clin Padiatr 218:243-245.