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Presented by Pedram Argani, M.D. and prepared by Aatur Singhi, M.D., Ph.D.
Case 5: This is a three year female with a cheek lesion.
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Week 425: Case 5
This is a three year female with a cheek lesion.images/1Alex/02222010case5image1.jpg
images/1Alex/02222010case5image2.jpg
images/1Alex/02222010case5image3.jpgCorrect
Answer: Juvenile xanthogranuloma
Histology: The dermis of the skin is involved by a mixed cellular infiltrate which includes macrophages, spindled fibroblastic cells, lymphocytes, and Touton-like giant cells. These latter cells have wreath-like nuclei and foamy cytoplasm at their periphery. These are the typical features of juvenile xanthogranuloma.
Discussion: Rosai Dorfman disease (sinus histiocytosis with massive lymphadenopathy) would feature larger histiocyte-like cells which label for S100 protein. These cells demonstrate emperipolesis of adjacent lymphocytes and plasma cells. Langerhans cell histiocytosis would feature histiocytes with grooved nuclei which label for S100 protein and CD1a. Mastocytoma would feature epithelioid cells with bland nuclei and frequently an associated eosinophilic infiltrate. The variation in cell type in the current lesion would not be present. Mastocytoma would label for CD117 and mast cell tryptase.
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Answer: Juvenile xanthogranuloma
Histology: The dermis of the skin is involved by a mixed cellular infiltrate which includes macrophages, spindled fibroblastic cells, lymphocytes, and Touton-like giant cells. These latter cells have wreath-like nuclei and foamy cytoplasm at their periphery. These are the typical features of juvenile xanthogranuloma.
Discussion: Rosai Dorfman disease (sinus histiocytosis with massive lymphadenopathy) would feature larger histiocyte-like cells which label for S100 protein. These cells demonstrate emperipolesis of adjacent lymphocytes and plasma cells. Langerhans cell histiocytosis would feature histiocytes with grooved nuclei which label for S100 protein and CD1a. Mastocytoma would feature epithelioid cells with bland nuclei and frequently an associated eosinophilic infiltrate. The variation in cell type in the current lesion would not be present. Mastocytoma would label for CD117 and mast cell tryptase.