Thirty Year Experience with Rosai Dorfman Disease treated with Radiation, Chemotherapy, and/or Surgery.

Abstract

Rosai-Dorfman Disease (RDD) is a rare disorder with histiocytosis and massive painless lymphadenopathy. It usually develops from the accumulation of non-Langerhans type histiocytic cells inside lymph nodes. Because the etiology of RDD is not well understood, the treatment is still nonspecific and controversial. Current data on the optimal management of RDD is limited to small case reviews/series. Herein we aim to describe treatment and outcomes, including the use of and response to radiation, in a large cohort of patients with RDD. We report on 27 patients diagnosed with Rosai-Dorfman Disease, who were evaluated at a single institution. Patients were diagnosed with RDD between 1980 and 2016. All patients had pathologic confirmation of RDD. We included patients who had a minimum of 3 months of follow up after diagnosis of RDD. We included patients of any age, with unifocal or multifocal disease, lymphatic and/or extranodal disease, and patients with previous treatment for RDD at outside institutions. We report descriptive outcomes for these patients. Median length of diagnosis was 73 months (IQR: 23- 138). Median age at diagnosis was 43 (range: 3 – 81). Fifty-two percent of patients were female. Three patients (11%) had nodal involvement alone, 17 patients (63%) had extranodal disease alone, and 7 patients (26%) had nodal disease and extranodal involvement at presentation. The most common site of disease involved the head and neck in 13 patients (48%). The most common therapeutic approaches included local or systemic steroids (n=11, 41%), chemotherapy (n=11, 41%), radiotherapy (n=9, 33%), and surgery (n=15, 55%). Eighteen patients were treated with 2 or more modalities (67%). Four of nine (44%) patients treated with radiation experienced a sustained response in the irradiated field. Median dose of RT was 36 Gy (range: 20 – 50 Gy). Intensity modulated radiation therapy (IMRT) was used for 5 patients. Various chemotherapy regimens were employed over the years, Clofarabine and Cladrabine were the most common systemic treatments. Seven (out of 11) patients receiving chemotherapy had stabilization, improvement, or resolution of disease after chemotherapy. At time of last follow up, 13 patients (48%) were with no evidence of disease, 11 patients had short interval stable disease (41%), and 3 patients (11%) had progressive disease. The optimal management of RDD remains elusive. Based on our review it seems that multiple treatment options may benefit patients. Radiation could be useful as an alternative option to avoid systemic therapy. Keeping in mind that avoiding long-term treatment related side effects is paramount, clinical factors such as age, location of the lesion(s), and symptoms, must be considered when recommending therapy for this benign condition.