Rosai-Dorfman disease with extranodal involvement

Laryngoscope. 2014 Mar;124(3):701-4. doi: 10.1002/lary.24290. Epub 2013 Jul 29.

Abstract

Rosai-Dorfman disease is a rare condition of marrow hematopoietic stem-cell origin. Patients can show extranodal involvement as well as lymphatic involvement, but only about 5% of extranodal cases involve intracranial lesions. A 53-year-old male was admitted to our hospital with bilateral cervical lymphadenopathy. Intracranial tumors and bone lesions were also detected. Cervical lymph node biopsy and intracranial tumor resection were performed, and histopathological examination revealed Rosai-Dorfman disease. The patient showed good clinical course without significant enlargement of the tumor. This study describes the case of a patient with Rosai-Dorfman disease presenting with massive cervical lymphadenopathy mimicking malignant neoplasm.

Keywords: Rosai-Dorfman disease; emperipolesis; lymphadenopathy; proliferative disorder; sinus histiocytosis.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Biopsy, Needle
  • Brain Neoplasms / diagnosis
  • Brain Neoplasms / pathology*
  • Brain Neoplasms / surgery
  • Craniotomy / methods
  • Diagnosis, Differential
  • Diagnostic Imaging / methods*
  • Fluorodeoxyglucose F18
  • Follow-Up Studies
  • Head and Neck Neoplasms / diagnosis
  • Head and Neck Neoplasms / pathology*
  • Head and Neck Neoplasms / therapy
  • Histiocytosis, Sinus / diagnosis*
  • Histiocytosis, Sinus / pathology
  • Histiocytosis, Sinus / therapy
  • Humans
  • Immunohistochemistry
  • Lymph Nodes / pathology*
  • Magnetic Resonance Imaging / methods
  • Male
  • Middle Aged
  • Neck Pain / diagnosis
  • Neck Pain / etiology
  • Photomicrography
  • Positron-Emission Tomography / methods
  • Prednisolone / therapeutic use
  • Rare Diseases
  • Severity of Illness Index
  • Tomography, X-Ray Computed / methods

Substances

  • Fluorodeoxyglucose F18
  • Prednisolone