Erdheim-Chester disease: consensus recommendations for evaluation, diagnosis, and treatment in the molecular era
Abstract
Erdheim-Chester disease (ECD) is a rare histiocytosis that was recently recognized as a neoplastic disorder owing to the discovery of recurrent activating MAPK (RAS-RAF-MEK-ERK) pathway mutations. Typical findings of ECD include central diabetes insipidus, restrictive pericarditis, perinephric fibrosis, and sclerotic bone lesions. The histopathologic diagnosis of ECD is often challenging due to nonspecific inflammatory and fibrotic findings on...
Paper Details
Title
Erdheim-Chester disease: consensus recommendations for evaluation, diagnosis, and treatment in the molecular era
Published Date
May 28, 2020
Journal
Volume
135
Issue
22
Pages
1929 - 1945
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