Rosai-Dorfman-Destombes disease

Section editor
	Gaurav Goyal, MD UAB Birmingham, AL GauravGoyalMD

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Guidelines

Consensus guidelines

• 2018: Abla et al. Consensus recommendations for the diagnosis and clinical management of Rosai-Dorfman-Destombes disease PubMed

NCCN

• NCCN Guidelines - Histiocytic Neoplasms

All lines of therapy

Cobimetinib monotherapy

Regimen

FDA-recommended dose

Study	Dates of enrollment	Evidence
Diamond et al. 2019 (MSK 15-216)	2016-NR	Phase 2, <20 pts in this arm (RT)

Note: there were N=2 patients with this type of histiocytosis.

Targeted therapy

• Cobimetinib (Cotellic) 60 mg PO once per day on days 1 to 21

28-day cycles

References

1. MSK 15-216: Diamond EL, Durham BH, Ulaner GA, Drill E, Buthorn J, Ki M, Bitner L, Cho H, Young RJ, Francis JH, Rampal R, Lacouture M, Brody LA, Ozkaya N, Dogan A, Rosen N, Iasonos A, Abdel-Wahab O, Hyman DM. Efficacy of MEK inhibition in patients with histiocytic neoplasms. Nature. 2019 Mar;567(7749):521-524. Epub 2019 Mar 13. link to original article contains dosing details in manuscript link to PMC article PubMed https://clinicaltrials.gov/ct2/show/NCT02649972