Rosai-Dorfman disease of the pleura: a rare extranodal presentation

Hum Pathol. 2003 Nov;34(11):1210-1. doi: 10.1016/s0046-8177(03)00402-7.


We present a case of Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) manifesting primarily as a pleural disease without involvement of the lung parenchyma. The patient is an 81-year-old man who presented with increasing shortness of breath. Radiographic studies revealed a large loculated left-sided pleural effusion and parietal pleural thickening. Video-assisted thoracoscopic biopsy of the pleura showed the characteristic S100-positive histiocytes exhibiting emperipolesis. In this rare case of pleural Rosai-Dorfman disease, the extensive involvement of the pleural lymphatics by the characteristic histiocytes appears to have been responsible for the pleural effusion.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Aged, 80 and over
  • Histiocytes / metabolism
  • Histiocytes / pathology
  • Histiocytosis, Sinus / complications
  • Histiocytosis, Sinus / metabolism
  • Histiocytosis, Sinus / pathology*
  • Humans
  • Male
  • Pleural Diseases / complications
  • Pleural Diseases / metabolism
  • Pleural Diseases / pathology*
  • Pleural Effusion / etiology
  • S100 Proteins / metabolism


  • S100 Proteins