Soffietti Riccardo, Rudà Roberta, Reardon David
Department of Neuro-Oncology, University and City of Health and Science Hospital, Turin, Italy.
Department of Neuro-Oncology, University and City of Health and Science Hospital, Turin, Italy.
Handb Clin Neurol. 2016;134:399-415. doi: 10.1016/B978-0-12-802997-8.00024-4.
This chapter describes the epidemiology, pathology, molecular characteristics, clinical and neuroimaging features, treatment, outcome, and prognostic factors of the rare glial tumors. This category includes subependymal giant cell astrocytoma, pleomorphic xanthoastrocytoma, astroblastoma, chordoid glioma of the third ventricle, angiocentric glioma, ganglioglioma, desmoplastic infantile astrocytoma and ganglioma, dysembryoplastic neuroepithelial tumor, papillary glioneuronal tumor, and rosette-forming glioneuronal tumor of the fourth ventricle. Many of these tumors, in particular glioneuronal tumors, prevail in children and young adults, are characterized by pharmacoresistant seizures, and have an indolent course, and long survival following surgical resection. Radiotherapy and chemotherapy are reserved for recurrent and/or aggressive forms. New molecular alterations are increasingly recognized.
本章描述了罕见胶质肿瘤的流行病学、病理学、分子特征、临床和神经影像学特征、治疗、预后及预后因素。这一类别包括室管膜下巨细胞星形细胞瘤、多形性黄色星形细胞瘤、成星形细胞瘤、第三脑室脊索样胶质瘤、血管中心性胶质瘤、神经节胶质瘤、促纤维增生性婴儿星形细胞瘤和神经节瘤、胚胎发育不良性神经上皮肿瘤、乳头状胶质神经元肿瘤以及第四脑室菊形团形成性胶质神经元肿瘤。这些肿瘤中的许多，尤其是胶质神经元肿瘤，在儿童和年轻人中较为常见，其特征为药物难治性癫痫发作，病程进展缓慢，手术切除后生存期长。放疗和化疗适用于复发和/或侵袭性肿瘤。新的分子改变越来越受到关注。
