Congenital Extrarenal Non-Central Nervous System Malignant Rhabdoid Tumor
Congenital Extrarenal Non-Central Nervous System Malignant Rhabdoid Tumor
复制标题
先天性肾外非中枢神经系统恶性横纹肌样瘤
DOI：
10.1097/00043426-200205000-00020
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发表时间：
2002
期刊：
影响因子：
--
通讯作者：
M. Coppes
中科院分区：
文献类型：
--
作者：
M. Sajedi;J. Wolff;Maarten Egeler;A. Pinto;Rhiannon Hughes;Ron A Anderson;M. Coppes
恶性横纹肌样瘤（MRT）是一种罕见的肿瘤，主要发生在肾脏和中枢神经系统。预后不佳。偶尔，在出生时或出生后立即诊断出mrt。一位女性新生儿在胸壁、腋窝、右肘和骨髓行MRT检查。胸壁病变完全切除。虽然腋窝和骨髓的肿块对化疗反应迅速，但肘部病变的大小增加了。尽管进行了激烈的治疗，但肿瘤在肺部复发，患者在诊断后12个月死亡。文献回顾显示，另外20例先天性MRTs发生在肾脏和中枢神经系统以外的部位。18例患者在诊断时已出现弥散性疾病。所有患者（n = 21）的中位总生存时间为2.0个月（0-24个月）。唯一存活下来的患者在最初诊断时只有局部肿瘤。先天性、肾外、非中枢神经系统mri是侵袭性肿瘤，预后差。
Malignant rhabdoid tumor (MRT) is a rare tumor occurring mostly in kidneys and central nervous system (CNS). Its prognosis is not good. Occasionally, MRTs are diagnosed at or immediately after birth. A female neonate presented with MRT in the chest wall, axilla, right elbow, and bone marrow. Chest wall lesion was resected completely. Although the masses in axilla and bone marrow responded rapidly to chemotherapy, the elbow lesion increased in size. Despite intense treatment, the tumor relapsed in lungs and the patient died 12 months after diagnosis. Review of the literature showed twenty additional congenital MRTs arising from sites outside of the kidney and central nervous system were published in the literature. Eighteen patients had disseminated disease at diagnosis. The median overall survival time for all (n = 21) patients was 2.0 months (0–24 months). The only patient who survived had a localized tumor at initial diagnosis. Congenital, extrarenal, non-CNS MRTs are aggressive tumors with poor outcome.
DOI：
--
发表时间：
1999
期刊：
The New England journal of medicine
影响因子：
--
作者：
K. Matthay;J. Villablanca;R. C. Seeger;D. Stram;R. E. Harris;N. Ramsay;P. Swift;H. Shimada;C. T. Black;G. M. Brodeur;R. Gerbing;C. P. Reynolds
通讯作者：
K. Matthay;J. Villablanca;R. C. Seeger;D. Stram;R. E. Harris;N. Ramsay;P. Swift;H. Shimada;C. T. Black;G. M. Brodeur;R. Gerbing;C. P. Reynolds