儿童头颈部结外Rosai-Dorfman病的表现

Manifestations of Pediatric Extranodal Rosai Dorfman Disease in the head and neck.

作者信息

Alwani Mohamedkazim M, Elghouche Alhasan N, Schueth Elizabeth A, Campiti Vincent J, Matt Bruce H, Yekinni Abideen O

机构信息

Department of Otolaryngology-Head and Neck Surgery, United States; Indiana University School of Medicine, United States.

出版信息

Int J Pediatr Otorhinolaryngol. 2020 Apr;131:109851. doi: 10.1016/j.ijporl.2019.109851. Epub 2019 Dec 26.

DOI:10.1016/j.ijporl.2019.109851

PMID:31901484

Abstract

OBJECTIVES

To systematically evaluate the clinico-diagnostic profile and management outcomes of otorhinolaryngologic manifestations of Extranodal Rosai-Dorfman Disease (ENRDD) in the pediatric population.

METHODS

The search terms Rosai Dorfman Disease and Sinus Histiocytosis were used to query PubMed, Ovid/Medline, and Scopus databases from inception through September 30, 2018. Studies were systematically reviewed in accordance to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) protocol. All reports of ENRDD involving at least one otorhinolaryngologic subsite in children less than 18 years were eligible for inclusion.

RESULTS

A total of 31 studies met inclusion criteria resulting in identification of 31 ENRDD cases with a mean age of 11.5 years. Of the 31 patients, 23 were male (74.2%) and 7 were female (22.6%). Extranodal lesions were limited to otorhinolaryngologic subsites in 24 patients (77.4%), while 7 patients (22.5%) were found to have extranodal lesions simultaneously involving otorhinolaryngologic and nonotorhinolaryngologic sites. The nasal vault was the most common otorhinolaryngologic site involved (n = 13, 41.9%), followed by the paranasal sinuses (n = 10, 32.3%). The most common non-otorhinolaryngologic site that was concurrently involved was the orbit (n = 4, 57.1%). Concurrent cervical lymphadenopathy was present in 19 patients (61.2%). While not documented for 2 cases, emperipolesis on histopathology was confirmed in 29 patients (93.5%). Single therapy with surgical excision was the most common modality of treatment (n = 15, 53.6%) and yielded highest remission rates (80%). In 11 instances (34.36%), ENRDD was misdiagnosed.

CONCLUSION

Pediatric ENRDD is a rare disease entity that maintains a high misdiagnosis potential. The most common otorhinolaryngologic location for extranodal manifestation is the sinonasal compartment. Surgical excision remains the most common treatment modality yielding lowest persistence and/or recurrence rates.

摘要

目的

系统评估儿童结外Rosai-Dorfman病（ENRDD）的耳鼻咽喉科表现的临床诊断特征及治疗结果。

方法

使用搜索词“Rosai Dorfman病”和“窦性组织细胞增多症”检索PubMed、Ovid/Medline和Scopus数据库，检索时间从建库至2018年9月30日。按照系统评价和Meta分析的首选报告项目（PRISMA）方案对研究进行系统评价。所有涉及18岁以下儿童至少一个耳鼻咽喉科亚部位的ENRDD报告均符合纳入标准。

结果

共有31项研究符合纳入标准，共识别出31例ENRDD病例，平均年龄为11.5岁。31例患者中，23例为男性（74.2%），7例为女性（22.6%）。24例患者（77.4%）的结外病变局限于耳鼻咽喉科亚部位，7例患者（22.5%）的结外病变同时累及耳鼻咽喉科和非耳鼻咽喉科部位。鼻腔穹窿是最常累及的耳鼻咽喉科部位（n = 13，41.9%），其次是鼻窦（n = 10，32.3%）。同时累及的最常见非耳鼻咽喉科部位是眼眶（n = 4，57.1%）。19例患者（61.2%）存在颈淋巴结肿大。虽然2例未记录，但29例患者（93.5%）经组织病理学证实有嗜血细胞现象。手术切除单一治疗是最常见的治疗方式（n = 15，53.6%），缓解率最高（80%）。11例（34.36%）中，ENRDD被误诊。