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肺上皮样血管内皮瘤:一例罕见病例及文献综述

Pulmonary epithelioid hemangioendothelioma: an unusual case and a review of the literature.

作者信息

Cronin Paul, Arenberg Douglas

机构信息

Department of Radiology, Division of Thoracic Radiology, University of Michigan Medical School, 1150 West Medical Center Drive, Ann Arbor, MI 48109, USA.

出版信息

Chest. 2004 Feb;125(2):789-93. doi: 10.1378/chest.125.2.789.

DOI:10.1378/chest.125.2.789
PMID:14769767
Abstract

We describe a case of pulmonary epithelioid hemangioendothelioma, previously known as intravascular bronchoalveolar tumor, in a 35-year-old woman with an initial diagnosis made by transbronchial biopsy. This is a rare disease, with approximately 50 cases described in the literature. All previous cases have been diagnosed by surgical lung biopsy. Although our patient underwent thoracoscopic lung biopsy, the diagnosis was initially made on transbronchial biopsy; to our knowledge, this has not been previously described in the English-language literature. We also described findings on high-resolution CT, both typical and atypical relative to previously published reports. This tumor can affect multiple organs. The prognosis is very unpredictable, with life expectancy ranging from 1 to 15 years. There is no single effective treatment, though spontaneous regression and response to chemotherapy and interferon are reported.

摘要

我们描述了一例肺上皮样血管内皮瘤病例,该肿瘤以前被称为血管内支气管肺泡瘤,患者为一名35岁女性,最初通过经支气管活检确诊。这是一种罕见疾病,文献中报道了约50例病例。此前所有病例均通过手术肺活检确诊。尽管我们的患者接受了胸腔镜肺活检,但最初的诊断是基于经支气管活检做出的;据我们所知,英文文献中此前尚未有过此类描述。我们还描述了高分辨率CT的表现,与先前发表的报告相比,既有典型表现也有非典型表现。这种肿瘤可累及多个器官。预后非常难以预测,预期寿命为1至15年。虽然有自发消退以及对化疗和干扰素治疗有反应的报道,但尚无单一有效的治疗方法。

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