Rosai-Dorfman disease of the paranasal sinuses and orbit

Hematol Oncol Stem Cell Ther. 2011;4(2):94-6. doi: 10.5144/1658-3876.2011.94.

Abstract

Rosai-Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy, is a benign indolent disorder, characterized by enlarged lymph nodes filled with histiocytes. Extranodal involvement is uncommon. The disease rarely affects the nose and paranasal sinuses. We report a case that presented with a right nasal mass, extending into all the paranasal sinuses and right orbit without any accompanying lymphadenopathy. Because of the absence of lymphadenopathy it posed a diagnostic challenge until the pathology was confirmed on histopathological examination.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Antineoplastic Agents / therapeutic use
  • Cyclophosphamide / therapeutic use
  • Female
  • Histiocytosis, Sinus / diagnosis*
  • Histiocytosis, Sinus / drug therapy
  • Histiocytosis, Sinus / pathology*
  • Humans
  • Orbit / diagnostic imaging
  • Orbit / pathology*
  • Paranasal Sinuses / diagnostic imaging
  • Paranasal Sinuses / pathology*
  • Prednisolone / therapeutic use
  • S100 Proteins / metabolism
  • Tomography, X-Ray Computed
  • Vincristine / therapeutic use

Substances

  • Antineoplastic Agents
  • S100 Proteins
  • Vincristine
  • Cyclophosphamide
  • Prednisolone