Extraspinal ependymoma D43.4

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 03.08.2021

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Synonym(s)

Extraspinal ependymoma; Extraspinal myxopapillary ependymoma; Soft Tissue Dendymoma

Definition
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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.

Localization
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presacral subcutis

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

Histology
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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).

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

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

Literature
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  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.

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 03.08.2021