A case study on Rosai-Dorfman disease occurring within the pelvis

Abstract

Rosai-Dorfman disease, also termed sinus histiocytosis with massive lymphadenopathy (SHML), represents a rare proliferative disorder of histiocytes, that commonly affects the cervical lymph nodes. It was first described as a unique clinicopathological entity by Rosai and Dorfman in 1969.1 Extranodal disease is encountered in almost half of all cases, presenting within the skin, soft tissue, salivary glands, bone or central nervous system. We describe a case of a young female who presented with an iliac fossa mass. Radiological imaging failed to demonstrate abnormalities within the uterus or the adnexae. A biopsy of the mass revealed sinus histiocytosis with emperipolesis, consistent with a diagnosis of Rosai-Dorfman disease. The patient was treated with conventional chemotherapy, followed by surgical intervention in the form of complete pelvic lymph node clearance. The patient has remained disease free to date.