Rosai-Dorfman-Destombes disease: lines of therapy
5 standard-of-care settings across 3 lines and 2 biomarker subgroups. Rows come from the cancer page's standard of care; the grid places each on its line and subgroup.
Screening, prevention and diagnosis
| Subgroup | Setting | Approach | Products and trials | Guideline | Evidence |
|---|---|---|---|---|---|
| All comers | Diagnosis and staging | Excisional biopsy with immunohistochemistry and MAPK pathway sequencing; FDG-PET/CT; MRI where neurological disease is suspected; immunoglobulins and autoimmune screen. | Consensus recommendations for Rosai-Dorfman-Destombes disease (Blood 2018) | 95 |
Third line and beyond
| Subgroup | Setting | Approach | Products and trials | Guideline | Evidence |
|---|---|---|---|---|---|
| FRα | MAPK-mutant or refractory disease | Cobimetinib (approved 2022 for histiocytic neoplasms) or trametinib. | Consensus recommendations for Rosai-Dorfman-Destombes disease (Blood 2018) | 97 |
Other settings
| Subgroup | Setting | Approach | Products and trials | Guideline | Evidence |
|---|---|---|---|---|---|
| All comers | Asymptomatic nodal or cutaneous disease | Observation, because spontaneous regression is common. | Consensus recommendations for Rosai-Dorfman-Destombes disease (Blood 2018) | 63 | |
| All comers | Single or compressive extranodal lesion | Surgical excision or debulking; radiotherapy for unresectable localised disease (orbit, airway). | Consensus recommendations for Rosai-Dorfman-Destombes disease (Blood 2018) | 93 | |
| All comers | Multifocal or organ-threatening disease | Corticosteroids for rapid control; sirolimus with prednisone, cladribine, methotrexate or lenalidomide; rituximab for IgG4-associated disease. | Consensus recommendations for Rosai-Dorfman-Destombes disease (Blood 2018) | 83 |
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.