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

Authors

  • 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

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Published

2013-12-09

Issue

Vol. 6 No. 1 (2014)

Section

Case Studies

License

South African Journal of Gynaecological Oncology (SAJGO) Copyright is held by South African Society of Gynaecologic Oncology (SASGO). Copyright of the articles is held by the authors. The work is licensed under a Creative Commons Attribution-Non-Commercial Works 4.0 South Africa License (CC BY NC). Material submitted for publication in the SAJGO is accepted provided it has not been published elsewhere. The SAJGO does not hold itself responsible for statements made by the authors. The opinions expressed in this publication are those of the authors. They do no purport to reflect the opinions or views of SASGO or its members.