Cutaneous sclerosing perineurioma with cryptic NF2 gene deletion.
Sclerosing perineurioma is a recently described variant of perineurioma that characteristically occurs in the fingers and palms of young adults. We report 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. Additionally, clonal chromosomal abnormalities of chromosome 10 and a cryptic deletion of the 5'BCR and NF2 loci on chromosome 22 were present. These findings 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 candidate because of its involvement in other nerve sheath tumors.
Department of Pathology, University of Leuven, Belgium.
R
R
Sciot
P
P
Dal Cin
A
A
Hagemeijer
L
L
De Smet
B
B
Van Damme
H
H
Van den Berghe
D000293:Adolescent; D014408:Biomarkers, Tumor / Q000378:metabolism; D002892:Chromosomes, Human, Pair 22 / Q000235:genetics; D005260:Female; D016515:Genes, Neurofibromatosis 2; D006801:Humans; D007150:Immunohistochemistry; D017404:In Situ Hybridization, Fluorescence; D018317:Nerve Sheath Neoplasms / Q000235:genetics* / Q000378:metabolism / Q000473:pathology*; D015513:Oncogene Proteins / Q000235:genetics; D010524:Peripheral Nervous System Neoplasms / Q000235:genetics* / Q000378:metabolism / Q000473:pathology*; D011505:Protein-Tyrosine Kinases; D011518:Proto-Oncogene Proteins; D051562:Proto-Oncogene Proteins c-bcr; D012598:Sclerosis; D017384:Sequence Deletion; D012878:Skin Neoplasms / Q000235:genetics / Q000378:metabolism / Q000473:pathology*
D014408:Biomarkers, Tumor; D015513:Oncogene Proteins; D011518:Proto-Oncogene Proteins; D011505:Protein-Tyrosine Kinases; C496613:BCR protein, human; D051562:Proto-Oncogene Proteins c-bcr