Thyroid & parathyroid

Benign thyroid neoplasms

Rosai-Dorfman disease

Topic Completed: 1 March 2009

Minor changes: 28 April 2021

Copyright: 2003-2021,, Inc.

PubMed Search: Rosai-Dorfman disease thyroid

Shahid Islam, M.D., Ph.D.
Page views in 2020: 5,861
Page views in 2021 to date: 5,774
Cite this page: Islam S. Rosai-Dorfman disease. website. Accessed December 3rd, 2021.
Definition / general
  • Rare, < 15 cases reported in thyroid gland
  • Histiocytic proliferative disorder of unknown cause, mean age 21 years
  • Also called sinus histiocytosis with massive lymphadenopathy
Clinical features
  • Typically presents with bilateral, painless cervical lymphadenopathy, although extranodal disease of head and neck is common
  • May represent extension from lymph node
  • Usually undergoes spontaneous remission after a protracted course, but may recur and rarely involves vital organs causing death
  • Associated with autoimmune thyroiditis (Thyroid 2007;17:471)
Case reports
Microscopic (histologic) description
  • Numerous histiocytes with abundant clear to eosinophilic cytoplasm and vesicular nuclei, also sheets of lymphocytes and plasma cells with emperipolesis (phagocytosis of intact lymphocytes and plasma cells)
Negative stains
Differential diagnosis
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