• Also known as Rosai-Dorfman disease
• May present as soft tissue mass with nodal involvement in 25%
• Usually women, mean 46 years, range 24-66 years
• Usually extremities (52%), also trunk (26%), head and neck (13%), retroperitoneum (9%)
• May recur after surgery
• Difficult diagnosis in non-nodal tissue

• 26 year old man with pleural effusion and cervical lymphadenopathy (J Korean Med Sci 2009;24:760)
• 49 year old woman with nasal obstruction and epistaxis (Cytojournal 2011;8:3)
• Case confused with inflammatory pseudotumor (Pathology 1998;30:14)

Thigh lesion

• Large histiocytic cells, frequently spindled, with less conspicuous emperipolesis than nodal lesions; fibroinflammatory component (Am J Surg Pathol 1992;16:122)

Cervical lymph node

Extranodal:

Subcutaneous right thigh

CD68

S100

Cervical lymph node

• S100, CD68, lysozyme

• Inflammatory myofibroblastic tumor

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