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.