Which histologic findings are characteristic of juvenile xanthogranuloma (JXG)?

Updated: Feb 08, 2019
  • Author: Bhupendra C K Patel, MD, FRCS; Chief Editor: Michael Taravella, MD  more...
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Lesions contain dense polyhedral histiocytes with large amounts of cytoplasm that often contain vacuoles. Touton giant cells are present in 85% of cases. [2] They have a wreath of nuclei surrounding a homogenous eosinophilic cytoplasmic center. See the image below.

Touton giant cell in juvenile xanthogranuloma. Tou Touton giant cell in juvenile xanthogranuloma. Touton giant cell with a wreath of nuclei surrounding a homogenous eosinophilic cytoplasmic center in juvenile xanthogranuloma. Touton cells are named after Karl Touton, who first described them in 1885. Touton giant cells are seen in xanthoma, juvenile and adult xanthogranulomas, dermatofibroma, and fat necrosis.

Immunohistochemistry shows the lesions to be positive for factor XIIIa, CD68, CD163, fascin, and CD14 but negative for S100 and CD1. This can be used to differentiate these lesions from Langerhans cell histiocytoses.

A prominent vascular network is often present. Evidence of tissue inflammation is seen in the swelling and degeneration of epithelial cells and redundant capillary basement membranes with perivascular edema.

Factor XIIIa is a fibrohistiocytic marker that is positive in juvenile xanthogranuloma, benign fibrous histiocytoma, malignant fibrous histiocytoma, calcifying fibrous tumors, verruciform xanthomas, fibroxanthoma, hemangiopericytoma, Erdheim-Chester disease, and other rarer diseases such as myofibroblastoma, pleomorphic hyalinizing angiectatic tumor, and storiform collagenoma.

CD68 is also called KP1 or macrosialin. It is a marker of histiocytes and is positive in a large number of histiocytic diseases besides juvenile xanthogranuloma.

CD163 stains monocytes and macrophages and is positive in juvenile xanthogranuloma and histiocytic sarcoma.

Fascin is a protein that stains in carcinoma of the colon, lung, and ovary and is positive in Hodgkin lymphoma, Langerhans cell histiocytosis, and juvenile xanthogranuloma.

CD14 is used to identify monocytes and macrophages. It is positive in histiocytic sarcoma, myelomonocytic leukemia, and juvenile xanthogranuloma.

S100 an acidic protein used as a marker for melanoma and neural lesions.

CD1a, CD1b, and CD1d are found in humans. CD1a is used to diagnose Langerhans cell histiocytosis.

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