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成人肝脏未分化(胚胎性)肉瘤:一例病例报告及文献复习

Hepatic undifferentiated (embryonal) sarcoma in an adult: a case report and review of the literature.

作者信息

Tokunaga Y, Ryo J, Hoppou T, Kitaoka A, Tokuka A, Osumi K, Tanaka T

机构信息

Department of Surgery, Maizuru Municipal Hospital, Kyoto, Japan.

出版信息

Eur J Gastroenterol Hepatol. 2000 Nov;12(11):1247-51. doi: 10.1097/00042737-200012110-00014.

Abstract

Undifferentiated (embryonal) sarcoma of the liver (USL) is a rare malignant tumour with a poor prognosis. The absence of specific symptoms, the rapid tumour growth, the normality of the common tumour markers, and the consequential delay in the diagnosis often result in significant enlargement of the tumour. To our knowledge, there have been only 42 reported cases of USL in adults worldwide during the 40 years since the clinicopathological entity of USL was defined. We report here a 27-year-old male with USL who has been treated successfully with surgical resection and adjuvant chemotherapy using ifosfamide, adriamycin and cisplatin. Although the prognosis of the disease remains generally poor, long-term survival has been achieved recently in patients with a combination of surgery and multi-agent chemotherapy.

摘要

肝未分化(胚胎性)肉瘤(USL)是一种罕见的恶性肿瘤,预后较差。缺乏特异性症状、肿瘤生长迅速、常见肿瘤标志物正常以及随之而来的诊断延迟,常常导致肿瘤显著增大。据我们所知,自USL的临床病理实体被定义以来的40年间,全球仅报告了42例成人USL病例。我们在此报告一名患有USL的27岁男性,他通过手术切除以及使用异环磷酰胺、阿霉素和顺铂的辅助化疗成功治愈。尽管该病的总体预后仍然较差,但最近手术联合多药化疗的患者已实现长期生存。

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