What is the optimal initial therapy and management of Erdheim-Chester disease without actionable mutations such as BRAF or MAPK-ERK pathway alterations?

Would refer to the latest ECD consensus guidelines and NCCN guidelines on histiocytic neoplasms for the answer to that question:

Goyal et al., PMID 32187362

In short, here are the considerations:

1. Even patients without bonafide MAPK-ERK pathway mutations can respond to a MEK-inhibitor such as cobim...