Consensus recommendations for the diagnosis and clinical management of Rosai-Dorfman-Destombes disease
The first expert guidance on Rosai-Dorfman disease: how to confirm the diagnosis, which patients can be watched, and which need surgery, steroids, chemotherapy or targeted treatment.
Overview
Consensus from the Histiocyte Society and international experts on Rosai-Dorfman-Destombes disease covering histopathology (S100 and CD68 positive, CD1a negative histiocytes with emperipolesis), the clinical spectrum from isolated lymph node disease to extranodal, cutaneous and IgG4-related forms, molecular findings (KRAS and MAP2K1 mutations in a subset), and management: observation for asymptomatic disease, surgery for isolated lesions, corticosteroids, sirolimus, cladribine or other chemotherapy, and MEK inhibitors for MAPK-mutant disease.
The treatment rows on the Rosai-Dorfman page, from watchful waiting to cobimetinib, follow these recommendations.
- Consensus on case series; no prospective trial exists.
- The proportion of cases that are clonal neoplasms versus reactive is still debated.
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