Literature DB >> 36153784

Clinical features, diagnosis, treatment and prognosis of otolaryngological extranodal sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease, RDD).

Changjiang Li1, Jian Chen1, Peijie He1, Lei Cheng1, Haitao Wu2.   

Abstract

PURPOSE: To investigate the clinical features, diagnosis, treatment and prognosis of otolaryngological extranodal RDD.
METHODS: A retrospective analysis was performed on 16 patients who were initially diagnosed and treated with otolaryngological extranodal RDD in our hospital from January 2013 to January 2019.
RESULTS: There were 9 females and 7 males. The mean diagnostic age was 45.35. Nasal, laryngeal and otic RDD were, respectively, accounting for 56.25%, 31.25% and 12.5%. The median interval diagnostic time was individually 1, 0.5 and 0.2 year for nasal, laryngeal and otic RDD. The most common symptoms were separately progressive nasal congestion, dyspnea, otorrhea in nasal, laryngeal and otic RDD. 13 had cervical lymph node swelling on MRI. Surgery or postoperative radiotherapy were conformed. In the end, 14 patients with RDD survived. The survival rate is as high as 87.5%. One of them died of RDD in the fifth year. One case was lost to follow-up 2 years after treatment. Patients survive for at least 2 years and up to 9 years. There was no significant difference in life expectancy of extranodal RDD among different parts of ENT (P = 0.508 > 0.05). The average ages of laryngeal and nasal RDD were similar (P = 0.898 > 0.05). However, the age of both was significantly higher than ear RDD (P = 0.023 and 0.019 < 0.05).
CONCLUSIONS: The average diagnostic age was more than 20 years. Nasal RDD was the most common in this area. All laryngeal RDD had infiltrated subglottis. Functional surgery and postoperative radiotherapy can be used to achieve long-term remission and survival.
© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.

Entities:  

Keywords:  Extranodal RDD; Otolaryngology; Rosai–Dorfman disease (RDD); Sinus histiocytosis with massive lymphadenopathy; Treatment

Year:  2022        PMID: 36153784     DOI: 10.1007/s00405-022-07646-7

Source DB:  PubMed          Journal:  Eur Arch Otorhinolaryngol        ISSN: 0937-4477            Impact factor:   3.236


  3 in total

1.  Rosai-Dorfman disease (RDD) in the paraglottic space: report of a case and review of literature.

Authors:  Yu-Ling Ma; Zhuo-Ping Liang; Sheng-En Xu; Zhi-Hui Yang; Yi Peng; Xiao-Qiang Sun; Gang Qin
Journal:  Int J Clin Exp Pathol       Date:  2015-10-01

2.  Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinicopathological entity.

Authors:  J Rosai; R F Dorfman
Journal:  Arch Pathol       Date:  1969-01

3.  [Adenitis with lipid excess, in children or young adults, seen in the Antilles and in Mali. (4 cases)].

Authors:  P Destombes
Journal:  Bull Soc Pathol Exot Filiales       Date:  1965 Nov-Dec
  3 in total

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