Retrospective analysis of treatment strategies for nasal Rosai-Dorfman disease: Physiological perspective

Abstract

Background and Aim: Rosai-Dorfman disease (RDD) is a rare form of lymphadenopathy with sinus histiocytosis, whose pathogenesis is undefined. Therefore, the purpose of this study was to explore the best treatment of RDD in the nasal cavity (NC), paranasal sinus (PS), and nasopharynx. Methods: We conducted a review on the treatment stratigies published between 1969 and 2014 with duration of follow-up for nasal RDD along with 5 patients who received treatment in our hospital. Results: The clinical data from literature review and combining with our 5 patients, we have 87 patients in total. There is a slight male to female predominance (male:Female = 1.81:1), average age was 37.36-year, the average duration of follow-up was 23.6-month. Lesions on 32 cases (36.8%) limited to the NC or PSs and 37 cases (42.5%) with involvement of other extranodal sites and 34 cases (40.2%) with lymphadenopathy (one unknown). 62 patients received operations for their initial treatment, 30 (48.39%) patients with recurrence, 21 of the patients received enlarged resection, and 5 (23.81%) patients with recurrence; 41 patients received cytoreductive surgery, and 25 (60.98%) patients recurrence; 20 cases received simple medical treatment, 16 (80.00%) patients with no treatment effect and 1 patients died. 5 patients recieved no therapy, survived with the disease with no progress. Conclusion: Therapeutic effect of medical treatment was was not reported adequately in literature. Enlarged resection was the best way to treat nasal RDD. Enlarged resection combining with medical treatment could reduce the rate of recurrence, but this theory is just from few case reports and literature review. Finally, we found there was no relation between recurrence rate with age, sex, lymphadenopathy and other extranodal sites.