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肝恶性横纹肌样瘤自发破裂后的长期生存

Long-term survival after spontaneous rupture of a malignant rhabdoid tumor of the liver.

作者信息

Ravindra K V, Cullinane C, Lewis I J, Squire B R, Stringer M D

机构信息

Department of Paediatric Surgery, St James's University Hospital, Leeds, England.

出版信息

J Pediatr Surg. 2002 Oct;37(10):1488-90. doi: 10.1053/jpsu.2002.35427.

Abstract

A 13-month-old girl presented with a large malignant rhabdoid liver tumor that ruptured soon after admission. Six years after an emergency right hepatectomy and subsequent chemotherapy (ifosfamide, vincristine, and actinomycin D), she remains well and disease free. Previously, these rare tumors invariably have been fatal and resistant to multimodal therapy. This is the first report of long-term survival of a patient with a malignant rhabdoid liver tumor.

摘要

一名13个月大的女童患有巨大的恶性横纹肌样肝肿瘤,入院后不久肿瘤破裂。在接受急诊右肝切除及随后的化疗(异环磷酰胺、长春新碱和放线菌素D)6年后,她状况良好,无疾病复发。此前,这些罕见肿瘤无一例外都是致命的,且对多模式治疗耐药。这是首例关于恶性横纹肌样肝肿瘤患者长期生存的报告。

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