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[原发性肝血管肉瘤:8例回顾性分析]

[Primary hepatic angiosarcoma: A retrospective analysis of eight cases].

作者信息

Cazorla Arnault, Félix Sophie, Delabrousse Eric, Valmary-Degano Séverine

机构信息

Service de pathologie, CHRU de Besançon, 2, boulevard A.-Fleming, 25000 Besançon, France.

Service de radiologie, CHRU de Besançon, 2, boulevard A.-Fleming, 25000 Besançon, France.

出版信息

Ann Pathol. 2014 Dec;34(6):462-8. doi: 10.1016/j.annpat.2014.10.004. Epub 2014 Nov 4.

Abstract

Angiosarcoma is a malignant tumor of vascular endothelial cell origin. Primary hepatic angiosarcoma is rare, most often associated with chronic exposure to toxic substances. The diagnosis of angiosarcoma is based on histological examination. Presenting symptoms are nonspecific, including abdominal pain, impaired general condition and fever. Primary hepatic angiosarcoma is a fast-growing tumor and the diagnosis is usually made at an advanced stage of the disease. The prognosis is poor. Surgical resection is recommended as the curative choice in localized forms, highlighting the key role of screening programs of occupational medicine that may help to diagnose tumors at an earlier, localized stage. Radiotherapy and chemotherapy are considered to have a limited efficacy. Here, we report a series of eight cases of primary hepatic angiosarcoma diagnosed at the University Hospital of Besançon between 2001 and 2012. Clinical, radiological, histological and therapeutic characteristics of the patients are described and analyzed.

摘要

血管肉瘤是一种起源于血管内皮细胞的恶性肿瘤。原发性肝血管肉瘤罕见,大多与长期接触有毒物质有关。血管肉瘤的诊断基于组织学检查。其出现的症状不具有特异性,包括腹痛、全身状况不佳及发热。原发性肝血管肉瘤是一种生长迅速的肿瘤,通常在疾病晚期才得以诊断。预后较差。对于局限性病例,建议采用手术切除作为治愈性选择,这凸显了职业医学筛查项目的关键作用,该项目可能有助于在疾病早期局限性阶段诊断肿瘤。放疗和化疗的疗效被认为有限。在此,我们报告了2001年至2012年间在贝桑松大学医院确诊的一系列8例原发性肝血管肉瘤病例。对患者的临床、放射学、组织学及治疗特征进行了描述和分析。

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