Does the pleomorphic xanthoastrocytoma exist? Problems in the application of immunological techniques to the...

A case of a fibrous xanthomatous tumor of the meninges is reported. This is a rare tumor of childhood in which the characteristic pleomorphic histology contrasts with the good clinical prognosis. These tumors were reclassified as pleomorphic xanthoastrocytomas (PXA) due to their glial fibrillary acidic protein (GFAP) positivity. In the present tumor, GFAP was absent from nearly all cell bodies in most of the leptomeningeal regions of the tumor but could be detected with greater frequency at the cortical-leptomeningeal border zones and in the areas in which the tumor had infiltrated the cortex. All the tumor cells expressed vimentin and in, addition, most expressed alpha-1-antitrypsin, alpha-1-antichymotrypsin, tartrate-resistant acid phosphatase, common leukocyte antigen, and OKM1. This spectrum of marker staining corresponded not only to the pattern observed in two cutaneous fibrous histiocytomas and one malignant fibrous histiocytoma, but also to the results previously published in the literature with regard to fibrohistiocytic tumors. By contrast, this spectrum of monocytic-histiocytic marker staining was not seen in gliomas. We, therefore, regard the PXA as a mesenchymal tumor of the meninges, identical to benign fibrous histiocytomas elsewhere in the body. The possible reasons why this mesenchymal tumor can show GFAP positivity in the leptomeningeal border zone are discussed.