Rosai-Dorfman disease: two case reports and diagnostic role of fine-needle aspiration cytology

J Pediatr Hematol Oncol. 2006 Feb;28(2):103-6. doi: 10.1097/01.mph.0000200686.33291.d1.

Abstract

Rosai-Dorfman disease, or sinus histiocytosis with massive lymphadenopathy (SHML), is a rare and self-limiting benign disorder that most commonly involves the cervical lymph nodes. The authors describe two cases of SHML. Fine-needle aspiration of the lymphadenopathy was performed in both patients. Immunocytochemical and histologic features, as the evidence of emperipolesis and S100 protein positivity on immunostaining, were typical of SHML. Fine-needle aspiration cytology plays an important diagnostic role in SHML and may be conclusive in a typical clinical setting. The diagnosis of SHML should be considered in the differential diagnosis of massive, painless cervical lymphadenopathy. Long-term follow-up is necessary to observe the complete regression of the massive lymphadenopathy. However, specific therapy is available and should be limited to patients with compressive symptoms or extranodal disease.

Publication types

  • Case Reports

MeSH terms

  • Biopsy, Fine-Needle*
  • Child
  • Diagnosis, Differential
  • Female
  • Histiocytes / pathology
  • Histiocytosis, Sinus / diagnosis*
  • Histiocytosis, Sinus / diagnostic imaging
  • Histiocytosis, Sinus / genetics
  • Histiocytosis, Sinus / pathology
  • Humans
  • Male
  • Neck
  • Remission, Spontaneous
  • Tomography, X-Ray Computed
  • Ultrasonography