期刊
BRAIN TUMOR PATHOLOGY
卷 32, 期 2, 页码 105-111出版社
SPRINGER JAPAN KK
DOI: 10.1007/s10014-014-0205-1
关键词
Ependymoma; C11orf95; RELA; Fusion transcript; L1CAM
资金
- National Institutes of Health/National Cancer Institute [5T32CA163185]
- CERN Foundation
Ependymomas are rare glial tumors of the central nervous system that arise from the cells lining the ventricles and central canal within the spinal cord. The distribution of these tumors along the neuroaxis varies by age, most commonly involving the spinal cord in adults and the posterior fossa in children. It is becoming evident that ependymomas of infratentorial, supratentorial, and spinal cord location are genetically distinct which may explain the differences in clinical outcomes. A novel oncogenic fusion involving the C11orf95 and RELA genes was recently described in supratentorial ependymomas that results in constitutive aberrant activation of the nuclear factor-kB signaling pathway. Ependymosarcomas are rare neoplasms in which a malignant mesenchymal component arises within an ependymoma. We here describe a case of a sarcoma developing in a patient previously treated with chemotherapy and radiation whose original ependymoma and recurrent sarcoma were both shown to carry the type 1 C11orf95-RELA fusion transcript indicating a monoclonal origin for both tumors.
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