01/18/2025

Hypofractionated Radiation Therapy for Subcutaneous Rosai-Dorfman Disease: A Case Report

Advances in Radiation Oncology Manuscript
Authors Rachael M. Guenzel, William G. Breen, Ronald S. Go, Andrew L. Feldman, and Scott C. Lester

Rosai-Dorfman disease (RDD) is a rare and clinically heterogeneous histiocytic disorder often affecting children and young adults. RDD often presents as large, painless lymphadenopathy; however, extranodal involvement also occurs. Unlike children, 50% of adults with RDD present with cutaneous or subcutaneous disease. RDD is often associated with considerable fibrosis and inflammatory cells. Significant shrinkage of a mass may not be observed with many treatment regimens that do not address fibrosis.

Observation with close follow-up is a reasonable recommendation for patients that are asymptomatic, because the disease can be self-limiting and resolve on its own. Treatment is recommended for patients who are symptomatic with constitutional symptoms, pain, or enlarging lymphadenopathy. Treatment can include corticosteroids, chemotherapies, immunotherapies, sirolimus, surgery, or radiation. If disease can be excised entirely, surgery has typically been the first line of treatment. Case reports show that external beam radiation has been effective, although there are no standard dosing guidelines. Radiation doses between 30 and 50 Gy with conventional fractionation have been used, but there is no recommended fractionation schedule.

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