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Presented by Justin A. Bishop, M.D. and prepared by Sarah Karram, M.D.
Case 3: 45 year old man from Guatamala with a nasal mass.
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Question 1 of 1
1. Question
Week 583: Case 3
45 year old man from Guatamala with a nasal mass.images/Karram/case3_1.jpg
images/Karram/case3_2.jpg
images/Karram/case3_3.jpgCorrect
Answer: Rosai-Dorfman disease
Histology: The lesion consists of a lymphohistiocytic proliferation within the sinonasal subepithelial stroma. At intermediate power, dark-staining and lighter-staining areas seem to alternate. At high power, the predominant lesional cell type is the large histiocytic appearing cell with large round nuclei displaying prominent nucleoli. Lymphocytes and plasma cells appear to be present within the cytoplasm of the larger cells (emperipolesis), which were found to be strongly positive for CD68 (not shown) and S100.
Discussion: Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) can be nodal or extranodal. The head and neck is a relatively common site for extranodal Rosai-Dorfman disease, and the sinonasal tract is the most commonly affected head and neck subsite. Extranodal Rosai-Dorfman disease can more exhibit cell spindling and nuclear atypia than is often expected, leading to diagnostic difficulties. Emperipolesis and S100 positivity are diagnostic.
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Answer: Rosai-Dorfman disease
Histology: The lesion consists of a lymphohistiocytic proliferation within the sinonasal subepithelial stroma. At intermediate power, dark-staining and lighter-staining areas seem to alternate. At high power, the predominant lesional cell type is the large histiocytic appearing cell with large round nuclei displaying prominent nucleoli. Lymphocytes and plasma cells appear to be present within the cytoplasm of the larger cells (emperipolesis), which were found to be strongly positive for CD68 (not shown) and S100.
Discussion: Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) can be nodal or extranodal. The head and neck is a relatively common site for extranodal Rosai-Dorfman disease, and the sinonasal tract is the most commonly affected head and neck subsite. Extranodal Rosai-Dorfman disease can more exhibit cell spindling and nuclear atypia than is often expected, leading to diagnostic difficulties. Emperipolesis and S100 positivity are diagnostic.
