Jp. Rank et Sw. Rostad, PERINEURIOMA WITH OSSIFICATION - A CASE-REPORT WITH IMMUNOHISTOCHEMICAL AND ULTRASTRUCTURAL STUDIES, Archives of pathology and laboratory medicine, 122(4), 1998, pp. 366-370
We present the case of a peripheral nerve sheath tumor arising from th
e sciatic nerve in the thigh of a 42-year-old woman. Histologically, t
he tumor was composed of fascicles of spindle cells with extremely att
enuated cytoplasmic processes. Regions of the tumor were heavily miner
alized and fibrotic. The tumor was epithelial membrane antigen-positiv
e and S100-negative. Staining for Leu-7 was positive in a patchy distr
ibution. Type IV collagen was present between cells, and CD34 was nega
tive in the tumor cells. Ultrastructural features included elongate ce
llular processes surrounded by discontinuous basal lamina and collagen
. The features of the tumor therefore meet criteria for perineurioma.
This rare tumor is found most often in the extremities in middle-aged
woman. To our knowledge, the presence of bone formation in a perineuri
oma has not been described previously. The differential diagnosis incl
udes other peripheral nerve sheath tumors, low-grade fibrous tumors, a
nd synovial (monophasic) tumors. Evidence provided by electron microsc
opy and immunohistochemistry supports the diagnosis and classification
of this unusual nerve sheath tumor as perineurioma with ossification.
These findings expand our knowledge and criteria for classifying peri
pheral nerve sheath tumors.