Clinicopathologic Spectrum of Rosai Dorfman Disease through a Six-Case Series

Abstract

Background: Rosai-Dorfman disease (RDD), or Sinus histiocytosis with massive lymphadenopathy, is a rare idiopathic disorder characterized by histiocytic proliferation. While lymph node involvement is predominant, extranodal manifestations pose diagnostic challenges, and only limited cases involving the breast have been reported. Methods: We present a series of RDD cases, including two in cervical nodes, one in perisplenic and aortocaval nodes, one in retroperitoneal nodes, one in the breast, and one in the nasopharynx. Clinical and histopathological features are examined to enhance our understanding of this complex disorder. Discussion: Comparison with literature reveals RDD predominantly affecting young and middle-aged individuals, with nodal involvement more prevalent than extranodal. Clinical and imaging challenges in extranodal cases underscore the importance of accurate diagnosis through histopathological and immunohistochemical analyses. Successful outcomes with various treatment modalities reaffirm the manageable nature of RDD. Conclusion: This series contributes to understanding RDD's varied clinical presentations, diagnostic intricacies, and successful management outcomes. Increased awareness, especially regarding rare extranodal involvements such as the breast, is crucial for accurate diagnosis and optimal therapeutic interventions.

Keywords

• rosai-dorfman disease
• sinus histiocytosis
• lymphadenopathy
• extranodal