Rosai-Dorfman disease with atypical intrascrotal involvement

We describe two new cases of Rosai-Dorfman disease with intrascrotal involvement. Also known as sinus histiocytosis with massive lymphadenopathy, this is a rare entity, usually presenting with massive bilateral cervical lymphadenopathy. It is associated with extranodal involvement in up to 40% of cases, commonly in the face and neck area. Isolated intrascrotal involvement with no lymphadenopathies is extremely rare, and its definitive diagnosis is histopathological. Although they are the most frequent type, not all intrascrotal lesions are neoplastic; therefore, it is important to know other possible etiologies and their radiogical features that might help in the diagnosis. We also review other cases of intrascrotal involvement reported in the literature, as well as a review clinical features, treatment and prognosis of this entity, and a differential diagnosis of solid intrascrotal lesions.