Extranodal rosai-dorfman disease: Clinicopathologic series of 10 patients with radiologic correlation and review of the literature

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25 Scopus citations


Objectives: To describe the radiologic and clinicopathologic features of extranodal Rosai-Dorfman disease (RDD) in our patient population. Methods: Via a data mining engine, we evaluated 13 cases of extranodal RDD in 10 patients treated at our institution from 2000 to 2014. Results: There was a marked female predominance (90%) in our series. The most common clinical presentation was a palpable, painless mass, which often simulated a neoplasm. Only two cases occurred in children. Multicentric and recurrent disease were uncommon. Histologically, all cases showed large histiocytes with emperipolesis in a mixed inflammatory background, with areas of dense, storiform collagen fibrosis. Positive S-100 and CD68 with negative CD1a in histiocytes are characteristic. Conclusions: Extranodal RDD is rare and its manifestations varied. It may constitute a clinical and pathologic diagnostic challenge. Clinical suspicion and recognition of its histologic features are necessary for correct diagnosis and avoiding unnecessary treatment. Resection is curative in most cases.

Original languageEnglish (US)
Pages (from-to)211-221
Number of pages11
JournalAmerican Journal of Clinical Pathology
Issue number2
StatePublished - Feb 1 2016



  • Bone
  • Breast
  • Gastrointestinal
  • Hematopathology
  • Soft tissue

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

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