Rosai-Dorfman disease

Digital cases

 HPC:290

Localization

 lymph node (nodal Rosai-Dorfman disease)
 extranodal Rosai-Dorfman disease

• skin (#12558621#), hypodermis (#1498408#, #3401030#)
• pleura (#14652824#)
• intracranial (#14608579#, #12637307#), brain (#12601463#), meninges
• nasal region (#12761707#)
• breast (#12760958#, #9416494#, #9199644#)
• thyroid (#12699598#, #12683901#)
• bone (#12652340#, #9653919#, #9526780#, #2013464#)
• gastrointestinal tract (#12562257# #10746683#, #17400399#)
• eye (#12479625#), lacrymal gland
• kidneys (#10587134#, #10471758#, #9072605#, #1456886#)
• pancreas (#10370692#, #11555773#)
• salivary glands (#9472588#)
• liver (#8771474#)
• larynx
• heart (cardiac Rosai-Dorfman disease) (#19962176#)

Types

 mostly isolated
 rarely multicentric (#12622452#)

Synopsis

 large, pale, histiocytic cells (Rosai-Dorfman cells) exhibiting emperipolesis (cytophagocytosis)

Immunohistochemistry

 S-100+
 CD68+
 CD1a-

Electron microscopy

 Birbeck granules are absent

Associations

 acute lymphoblastic leukemia (##11496358#)
 lymphoma (#9031615#, #9006752#)
 follicular lymphoma (#10786808#)
 nodular lymphocyte predominant Hodgkin disease (#10786808#, #8522313#)
 chronic autoimmune thyroiditis (#10710276#)
 inflammatory pseudotumor (#9059366#)
 systemic lupus erythematosus (#11555773#)

References