Atypical teratoid/rhabdoid tumor of the central nervous system: Report on workshop
Atypical teratoid/rhabdoid tumor of the central nervous system: Report on workshop
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DOI：
10.1097/00043426-200206000-00004
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发表时间：
2002-06-01
影响因子：
1.2
通讯作者：
Smith, M
中科院分区：
文献类型：
--
作者：
Packer, RJ;Biegel, JA;Smith, M
儿童非典型畸胎瘤/横纹肌样肿瘤（AT/RT）的中枢神经系统（CNS）是最近描述的实体。诊断是基于独特的光学显微镜和免疫组化结果，加上分子遗传学分析。大多数AT/RT表现为22号单体或染色体带22 q11缺失，并伴有hSNF 5/INI 1基因的改变。该肿瘤的发病率尚不明确，但在婴儿中，其可能占原始CNS肿瘤的四分之一。治疗远非最佳，但偶尔会有长期幸存者，特别是年龄较大的儿童。治疗方法包括手术、化疗和放疗，但对AT/RT儿童的治疗仍需进行前瞻性临床试验。
Childhood atypical teratoid/rhabdoid tumor (AT/RT) of the central nervous system (CNS) is a recently described entity. Diagnosis is based on distinctive light microscopy and immunohistochemical findings, coupled with molecular genetic analysis. Most AT/RTs demonstrate monosomy 22 or deletions of chromosome band 22q11 with alterations of the hSNF5/INI1 gene. The tumor's incidence is still undefined, but it may comprise as big as 1 in 4 primitive CNS tumors in infants. Treatment is far from optimal, but there are occasional long-term survivors, especially among older children. Therapeutic approached have included surgery, chemotherapy, and radiotherapy.Prospective clinical trials are needed for children with AT/RTs.