European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery · 2026
Head and neck rosai-dorfman disease: upper airway predominance in 42 patients.
СтатьяНазвание и аннотация: Перевод в очереди
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Yang Yifan, Zhang Xue, Zhong Qi, Zhang Yang, Hou Lizhen, Li Shuling, He Shizhi, Zhao Yanming, Shi Qian, Lian Meng, Li Haiyang, Shi Yueyue, Zhu Siyu, Fang Jugao, Feng Ling
Аннотация
PURPOSE: 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.
METHODS: 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.
RESULTS: 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.
CONCLUSION: 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.
METHODS: 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.
RESULTS: 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.
CONCLUSION: 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.