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

  • Donald John Babu Tata Cancer Hospital
  • Amita Maheshwari Tata Cancer Hospital
  • Manju Sengar Tata Cancer Hospital
  • Santosh Menon Tata Cancer Hospital
Keywords: sinus histiocytosis, lymphadenopathy, emperipolesis, 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.

Author Biographies

Donald John Babu, Tata Cancer Hospital
MBBS, MS, FCPS Surgical Oncology Resident Department of Gynaecology and Surgical Oncology Tata Cancer Hospital Mumbai India
Amita Maheshwari, Tata Cancer Hospital
MBBS, MD Professor Department of Gynaecology and Surgical Oncology Tata Cancer Hospital Mumbai India
Manju Sengar, Tata Cancer Hospital
MBBS, MD, DM Professor Department of Hematology and Medical Oncology Tata Cancer Hospital Mumbai
Santosh Menon, Tata Cancer Hospital
MBBS, MD, DNB Associate Professor Department of Pathology Tata Cancer Hospital Mumbai
Published
2013-12-09
Section
Case Studies