Erdheim-Chester disease, Rosai-Dorfman disease and other histiocytic neoplasms: lines of therapy
4 standard-of-care settings across 1 lines and 2 biomarker subgroups. Rows come from the cancer page's standard of care; the grid places each on its line and subgroup.
Other settings
| Subgroup | Setting | Approach | Products and trials | Guideline | Evidence |
|---|---|---|---|---|---|
| All comers | Rosai-Dorfman disease | Observation for asymptomatic nodal disease; surgery for isolated masses; steroids, sirolimus, cladribine or MEK inhibitors for symptomatic or multifocal disease. | Consensus recommendations for RDD (Blood 2018) | 47 | |
| All comers | Histiocytic sarcoma | Lymphoma-type chemotherapy (CHOP, ICE, or similar), radiotherapy for localised disease, MAPK-pathway inhibitors where mutations are found; clinical trials. | 79 | ||
| BRAF | ECD, BRAF V600E-mutant, needing treatment | Vemurafenib (FDA approval 2017) or dabrafenib, often with a MEK inhibitor to reduce toxicity; long-term treatment at the lowest effective dose. | Consensus recommendations for ECD (Blood 2020) | 84 | |
| BRAF | ECD or RDD, BRAF wild-type or intolerant of BRAF inhibitors | Cobimetinib (FDA approval October 2022 for histiocytic neoplasms) or another MEK inhibitor; interferon alfa or pegylated interferon as an alternative. | NCCN · Category 2A | 47 |
Lines and subgroups are parsed from the setting text of each standard-of-care row and can misclassify an unusual phrasing; the row’s own setting is always shown. Guideline chips reflect the NCCN category and ESMO-MCBS grade recorded on the cancer page, checked on its stated date. Not medical advice.