Evidence map›Paper›PMID 42760371›Full record

ArticleEuropean 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 Surgery2026

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

Yifan Yang, Xue Zhang, Qi Zhong, Yang Zhang, Lizhen Hou, Shuling Li, Shizhi He, Yanming Zhao, Qian Shi, Meng Lian and 5 more

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Article in 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. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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1 · What the graph read from it

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2 · The registry

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4 · The record

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5 · Who and what money

Authors and funding

15 authors.

Yifan YangDepartment of Otorhinolaryngology, Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University, 1 Dongjiaominxiang Street, Beijing, 100730, China.
Xue ZhangKey Laboratory of Otorhinolaryngology, Head and Neck Surgery, Beijing Institute of Otorhinolaryngology, 1 Dongjiaominxiang Street, Beijing, 100730, China.
Qi ZhongDepartment of Otorhinolaryngology, Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University, 1 Dongjiaominxiang Street, Beijing, 100730, China.
Yang ZhangDepartment of Otorhinolaryngology, Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University, 1 Dongjiaominxiang Street, Beijing, 100730, China.
Lizhen HouDepartment of Otorhinolaryngology, Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University, 1 Dongjiaominxiang Street, Beijing, 100730, China.
Shuling LiDepartment of Radiology, Beijing Tongren Hospital, Capital Medical University, 1 Dongjiaominxiang Street, Beijing, 100730, China.
Shizhi HeDepartment of Otorhinolaryngology, Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University, 1 Dongjiaominxiang Street, Beijing, 100730, China.
Yanming ZhaoDepartment of Otorhinolaryngology, Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University, 1 Dongjiaominxiang Street, Beijing, 100730, China.
Qian ShiDepartment of Otorhinolaryngology, Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University, 1 Dongjiaominxiang Street, Beijing, 100730, China.
Meng LianDepartment of Otorhinolaryngology, Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University, 1 Dongjiaominxiang Street, Beijing, 100730, China.
Haiyang LiDepartment of Otorhinolaryngology, Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University, 1 Dongjiaominxiang Street, Beijing, 100730, China.
Yueyue ShiDepartment of Otorhinolaryngology, Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University, 1 Dongjiaominxiang Street, Beijing, 100730, China.
Siyu ZhuDepartment of Otorhinolaryngology, Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University, 1 Dongjiaominxiang Street, Beijing, 100730, China.
Jugao FangDepartment of Otorhinolaryngology, Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University, 1 Dongjiaominxiang Street, Beijing, 100730, China.
Ling FengDepartment of Otorhinolaryngology, Head and Neck Surgery, Beijing Shijitan Hospital, Capital Medical University, 10 Tieyi Road, Beijing, 100038, China. fengling@ccmu.edu.cn.ORCID http://orcid.org/0009-0001-6533-4795

Funding

Beijing Hospitals Authority Youth Programme QML20230206Beijing Natural Science Foundation 7244307Beijing Natural Science Foundation 7252194
6 · The paper itself

Abstract

purposeWe 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.

methodsWe 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.

resultsThe 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.

conclusionRDD 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.

Indexed as

Extranodal diseaseHead and neck neoplasmsProgression-free survivalRosai-Dorfman disease

Identifiers

PMID42760371

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