Destombes — The Forgotten Pathologist: Rosai-Dorfman Disease, From Initial Recognition to the Latest and Greatest

Context.—: As part of the 100th anniversary of Archives of Pathology & Laboratory Medicine, we revisit a seminal article published by Juan Rosai, MD, and Ronald Dorfman, MBBCh, FRCPath, in this journal in 1969. This is one of the initial series describing the entity now known as Rosai-Dorfman-Destombes disease (RDD), following the case series of the French pathologist Pierre-Paul Louis Lucien Destombes, MD, which describes a striking but enigmatic entity that now bears their names. Rosai and Dorfman described this process using the moniker sinus histiocytosis with massive lymphadenopathy, with presentation in pediatric and young adults, with massive, painless cervical lymphadenopathy and a benign, but potentially protracted course. Histiocytes show characteristic cytomorphologic features (making it one of the most beloved entities by pathologists and board examiners alike), including round, hypochromatic nuclei with prominent nucleoli and abundant pale eosinophilic cytoplasm, with trafficking of inflammatory elements termed emperipolesis. Objective.—: To review the historical context surrounding the recognition of RDD and key clinicopathologic and molecular features. Data Sources.—: Historical papers as well as more recent studies advancing our understanding of RDD were identified from the literature, using PubMed and other search engines. Conclusions.—: RDD remains a potentially challenging entity for diagnosis and treatment given variation in presentation, morphologic features, and often a lack of known molecular drivers, which may occur in isolation or in patients with altered immune function, germline disorders, or other neoplastic conditions.