Extracranial metastasis of an anaplasic ependymoma, RELA fusion positive : a rare occurrence.

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Résumé

Primary intracranial ependymomas originate from ependymal cells. Theymay migrate mainly in the spinal cord but rarely metastasize outside the cen-tral nervous system. Metastases outside the central nervous system are rare.Metastatic diffusion from the central nervous system is low due to the uniqueinteraction of the brain and the tumor with the blood-brain barrier. Never-theless, three main hypotheses have been mentioned in the literature, the tu-mor growth, the surgical manipulation (which may be considered to be thecase in our patient), and the aggressiveness of the tumor according to theKi67 index. We report the case of a 16-year-old female, who underwent com-plete surgical removal of a left occipital 2007 WHO grade II ependymoma. 3years later, the patient presented multiple cervical and occipital induratedmasses. MRI showed a left hemispheric meningeal infiltration, with multiplenodules located on the neck, occiput and mastoid. Histopathological study of a left temporal surgical biopsy and resection of an occipital subcutaneous nodule turned to be metastases of an anaplastic ependymoma. The ependy-moma considered as a benign tumor could very quickly turn into malignancyby its metastatic potential. Early diagnosis and longer follow-up of patients would be recommended for a rapid management

Mots-clés

Anaplasie Ependymoma, Extracranial, Metastasis, Extracranial, RELA Fusion