Neurologia medico-chirurgica
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Neurol. Med. Chir. (Tokyo) · Jul 1999
Meta AnalysisClinicopathological characteristics of atypical teratoid/rhabdoid tumor.
The clinicopathological features of atypical teratoid/rhabdoid tumor, a new entity among malignant pediatric brain tumors, and the differential diagnosis from primitive neuroectodermal tumor (PNET)/medulloblastoma, and germ cell tumor are described. Histologically, atypical teratoid/rhabdoid tumor is defined as a polymorphous neoplasm often featuring rhabdoid, PNET, epithelial, and mesenchymal components. Atypical teratoid/rhabdoid tumors usually include PNET components and occur mainly in the posterior fossa, so mimic medulloblastoma. ⋯ The same is true of germ cell marker in tumor tissues and serum. The prognosis of atypical teratoid/rhabdoid tumor is far less favorable than that of PNET/medulloblastoma of malignant or germ cell tumor. Meta-analysis of 133 cases, including 15 new and 118 reported cases, confirm that atypical teratoid/rhabdoid tumor is as a clinicopathological entity and emphasizes the necessity for distinguishing this unique tumor from other pediatric central nervous system neoplasms.