Subcutaneous Rosai-Dorfman disease: is surgical excision justified?

Rosai-Dorfman disease in soft tissue without nodal disease has been recognized as a distinct clinicopathologic entity. It may represent a diagnostic challenge and the natural history and optimal treatment has not been well clarified. We investigated a patient in whom Rosai-Dorfman disease was confined to the subcutis of the abdominal wall and recurred after incomplete excision. Complete resolution was achieved by wide surgical excision with negative margins. Pathologic examination confirmed the diagnosis of extranodal Rosai-Dorfman disease. The patient is disease-free after 1 year of follow-up. Despite the possibility that spontaneous remission may occur, our results suggest that when anatomically feasible, complete excision can be a treatment option for persistence or recurrence of exclusively extranodal disease. Larger case series and longer follow-up are needed to assess the long-term efficacy in these patients.