Erdheim-Chester Disease: The Importance of Information Integration

Conclusion: This case highlights the need for developing a high index of suspicion for presentations of retroperitoneal fibrosis thatcould represent IgG4-related disease but fail to respond to steroids. When unusual multisystem involvement occurs, one should consider a diagnosis of a rare histiocytosis. Vemurafenib appears to be an effective treatment for even advanced cases of both ECD and Langerhans histiocytosis bearing the BRAF V600E mutation.Case Rep Oncol 2017;10:613 –619
Source: Case Reports in Oncology - Category: Cancer & Oncology Source Type: research