Head and neck rosai-dorfman disease: upper airway predominance in 42 patients

We aimed to define the distinct phenotype and outcomes of head and neck Rosai-Dorfman disease (RDD) by providing a detailed clinical characterization relevant to ENT practice.

We conducted a retrospective cohort study of 42 patients with histopathologically confirmed RDD whose initial disease manifestations originated in the head and neck at a tertiary referral center (2007-2024). Data on demographics, clinical presentation, treatment, and outcomes were analyzed. Progression-free survival (PFS) was evaluated using Kaplan-Meier estimates, and factors associated with progression were assessed with Cox regression.

The cohort demonstrated a striking female predominance (81.0%) and individuals of Asian descent (100%), with a median age of 52 years. All patients presented with primary extranodal disease, most commonly involving the upper respiratory tract (95.2%). The classic emperipolesis phenomenon was identified in only 33.3% of cases. No clinicopathological factor or treatment strategy significantly predicted PFS, although notable numerical trends were observed for absent emperipolesis (HR = 4.71) and systemic involvement at diagnosis (HR = 1.98). After a median follow-up of 73.5 months, most patients (83.3%) were in remission or had stable disease, while 16.7% experienced progression or recurrence.

RDD with head and neck onset defines a unique, ENT-centric clinical phenotype characterized by extranodal involvement, upper respiratory tract affinity, and a striking female-Asian predominance. The disease course is often indolent but inherently unpredictable, supporting the need for long-term surveillance. Future precision in care will depend on elucidating the molecular basis of this heterogeneity.