Alwani Mohamedkazim M, Elghouche Alhasan N, Schueth Elizabeth A, Campiti Vincent J, Matt Bruce H, Yekinni Abideen O
Department of Otolaryngology-Head and Neck Surgery, United States; Indiana University School of Medicine, United States.
Department of Otolaryngology-Head and Neck Surgery, United States; Indiana University School of Medicine, United States.
Int J Pediatr Otorhinolaryngol. 2020 Apr;131:109851. doi: 10.1016/j.ijporl.2019.109851. Epub 2019 Dec 26.
To systematically evaluate the clinico-diagnostic profile and management outcomes of otorhinolaryngologic manifestations of Extranodal Rosai-Dorfman Disease (ENRDD) in the pediatric population.
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.
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.
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.
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。
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。
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。
儿童ENRDD是一种罕见病，误诊可能性高。结外表现最常见的耳鼻咽喉科部位是鼻窦区。手术切除仍然是最常见的治疗方式，持续性和/或复发率最低。
