Cutaneous sclerosing perineurioma with cryptic NF2 gene deletion

Sclerosing perineurioma is a recently described variant of perineurioma tha t characteristically occurs in the fingers and palms of young adults. We re port a cutaneous sclerosing perineurioma with preservation of the axons and Schwann cells in the center of the whorls of perineurial cells, a feature that previously was reported to be typically absent in these lesions. Addit ionally, clonal chromosomal abnormalities of chromosome 10 and a cryptic de letion of the 5'BCR and NF2 loci on chromosome 22 were present. These findi ngs further support the hypothesis that a gene on chromosome 22 may play a role in the pathogenesis of perineurioma. The NF2 gene is a logical candida te because of its involvement in other nerve sheath tumors.