Atypical teratoid/rhabdoid tumors

Atypical teratoid/rhabdoid tumors
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DOI:
10.1007/s00381-003-0731-3
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发表时间:
2003-04-01
影响因子:
1.4
通讯作者:
Ventureyra, ECG
Ventureyra, ECG
中科院分区:
医学4区
文献类型:
--
作者:
Dang, T;Vassilyadi, M;Ventureyra, ECG

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病例报告:我们描述了三例非典型ATRT,确定在儿童医院东安大略。讨论:在过去的十年中,中枢神经系统的非典型畸胎样/横纹肌样肿瘤(ATRTs)已成为一个独特的实体。这种肿瘤通常被误诊为原始神经外胚层肿瘤(PNET)/髓母细胞瘤。ATRT独特的免疫组织化学特征有助于将其与PNET/髓母细胞瘤区分开来。这具有临床重要性,因为ATRT患者的预后比PNET/髓母细胞瘤更差,尽管加拿大积极的手术治疗有或没有辅助化疗和放疗。
Case reports: We describe three cases of atypical ATRT that were identified at the Children's Hospital of Eastern Ontario. Discussion: Over the past decade, atypical teratoid/rhabdoid tumors (ATRTs) of the central nervous system have emerged as a distinct entity. This tumor is typically misdiagnosed as a primitive neuroectodermal tumor (PNET)/medulloblastoma. The unique immunohistochemistry profile of an ATRT helps distinguish it from a PNET/medulloblastoma. This is of clinical importance because the prognosis of a patient with an ATRT is worse than that of a PNET/medulloblastoma despite Canada aggressive surgical treatment with or without adjuvant chemotherapy and radiation therapy.