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This study aims to characterize the clinico-pathologic features and real-world outcomes of adult Erdheim-Chester Disease in South Korea.

Clinico-Pathologic Features of Adult Erdheim–Chester Disease in South Korea: A Multicenter Retrospective Study (KCSG LY22-15)

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Multicenter retrospective study reveals diverse organ involvement and treatment patterns in adults with ECD.

This study aims to characterize the clinico-pathologic features and real-world outcomes of adult Erdheim-Chester Disease in South Korea.
Retrospective multicenter cohort study of adult patients with ECD at seven institutions from October 2008 to June 2024.
Clinical and pathologic data, BRAF V600E mutation status, treatment patterns, and overall survival were analyzed using the Kaplan-Meier method.
22 patients were included with a median age of 57 years.
Organ involvement included bone (54.5%), retroperitoneal/renal (31.8%), and others, with multisystem disease in 40.9%.
BRAF V600E mutation was positive in 57.9% of tested patients.
2-year overall survival rate was 90.0% and 5-year was 74.3% with a median follow-up of 4.6 years.

Lee et al. (2026) studied this question.

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