EpithelioidHemangioendothelioma of the Liver: Case Report

Czech version

Authors: Z. Kinkor
Authors‘ workplace: Oddělení patologie FN Na Bulovce Praha
Published in: Čes.-slov. Patol., , 2001, No. 3, p. 114-117
Category:

Overview

Presented is a typical case of Erdheim-Chester disease (ECD) wherein the severe pulmonarymanifestation led to an open lung biopsy and eventual morphologic recognition of the nonspecificclinical symptoms.The pulmonary involvement is described in almost 20% of cases and is prognostically unfavourable.About 20 cases of Erdheim-Chester disease were published and more than half of them had lethaloutcome. The ECD affecting seriously lungs appears as non-specific interstitial lung disease andusually does not enter the broad clinical differential diagnosis. A detailed bibliography withspecial attention to the pulmonary involvement by this enigmatic disease is presented.

Key words:
Erdheim-Chester disease - lungs - skeleton - interstitial pneumonia - histiocytosis -Langerhans cells - histiocytosis X

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Article was published in

Czecho-Slovak Pathology

2001 Issue 3