Malignant peripheral nerve sheath tumor of the pancreas with perineurial cell differentiation

The authors report a malignant peripheral nerve sheath tumor (MPNST) that developed in the body of the pancreas of a 76-year-old woman. The well-demarcated tumor, 4.5 x 3.5 cm in size, was composed of spindle-shaped cells arranged in a storiform pattern or sweeping fascicles. Ultrastructurally, the tumor cells had elongated cytoplasmic processes with numerous pinocytotic vesicles, and were covered with discontinuous basal lamina-like substances. Immunohistochemically, tumor cells were diffusely positive for epithelial membrane antigen, vimentin, and CD34, but negative for S-100 protein and Leu 7. It was therefore concluded that the tumor was a MPNST with perineurial cell differentiation. Primary MPNST of the pancreas is extremely rare and the immunohistochemical and ultrastructural studies are necessary for its diagnosis.