Laryngeal Rosai-Dorfman Disease (Sinus Histiocytosis with Massive Lymphadenopathy): A Retrospective Study of 5 Cases

BioMed Research International. 2017;2017 DOI 10.1155/2017/8521818

 

Journal Homepage

Journal Title: BioMed Research International

ISSN: 2314-6133 (Print); 2314-6141 (Online)

Publisher: Hindawi Limited

LCC Subject Category: Medicine

Country of publisher: United Kingdom

Language of fulltext: English

Full-text formats available: PDF, HTML, ePUB, XML

 

AUTHORS

Yanyan Niu (Department of ENT, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China)
Yongjin Li (Department of ENT, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China)
Jian Wang (Department of ENT, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China)
Xiaofeng Jin (Department of ENT, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China)
Dahai Yang (Department of ENT, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China)
Hong Huo (Department of ENT, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China)
Wuyi Li (Department of ENT, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China)

EDITORIAL INFORMATION

Blind peer review

Editorial Board

Instructions for authors

Time From Submission to Publication: 19 weeks

 

Abstract | Full Text

This study was performed to investigate the clinical manifestations, treatment methods, and prognosis of Rosai-Dorfman disease (RDD) with laryngeal involvement. Five clinical cases of RDD with laryngeal involvement diagnosed between 1986 and 2015 were retrospectively analyzed. The laryngeal lesions of these 5 patients mostly involved the glottis and subglottis, with the main symptoms being a hoarse voice and airway obstruction. In addition, the patients mostly exhibited a unilateral or asymmetric onset that was manifested by a laryngeal submucosal nodular mass. The patients were subjected to a regimen of hormone treatment combined with surgical resection. The median follow-up duration was 101 months (8–384 months). One case was lost, and the remaining 4 subjects are alive with disease. The follow-up examinations revealed that 4 subjects had stable laryngeal conditions, whereas one showed minor progression. RDD with laryngeal involvement is clinically rare and differs considerably from classical RDD in age of onset, gender composition, and extranodal involvement. The regimen of hormone treatment combined with surgical resection can stabilize the patient’s general condition and laryngeal lesion. Tracheotomies are recommended for patients with dyspnea. After their conditions stabilize, decannulation can be successfully performed in most cases. This therapeutic regimen generally delivers a good prognosis.