Immunohistochemical Demonstration of EMA/Glut1-Positive Perineurial Cells and CD34-Positive Fibroblastic Cells in Peripheral Nerve Sheath Tumors

To clarify the cellular composition of various peripheral nerve tumorous lesions (traumatic neuroma, 5 cases; schwannoma, 10 cases; neurofibroma, 14 cases; perineurioma, 3 cases; conventional malignant peripheral nerve sheath tumor (MPNST), 7 cases; perineurial MPNST, 4 cases), expression of several markers specific to nerve sheath cells, including glucose transporter protein 1 (Glut1) and CD34, were immunohistochemically investigated with highly sensitive detection methods. In normal nerves and neuromas, perineuriums were positive for Glut1 as well as for epithelial membrane antigen (EMA), and there were some CD34-positive fibroblast-like cells in the endoneurium. Schwannomas consisted principally of S-100 protein–positive Schwann cells, whereas a few CD34-positive fibroblastic cells were present in Antoni B areas. Neurofibromas and conventional MPNST exhibited a mixed proliferation of S-100 protein-, EMA/Glut1-, and CD34-positive cells, indicating a heterogeneous composition of the constituents. The catalyzed signal amplification (CSA) system demonstrated more numerous EMA-positive perineurial cells in neurofibromas than did the ENVISION+ method. Perineurial cell tumors (benign and malignant) were composed of EMA/Glut1-positive and S-100 protein–negative tumor cells. The present study confirmed the characteristic cellular composition to each nerve sheath tumor immunohistochemically and showed the usefulness of the nerve sheath cell markers. Glut1 as well as EMA are specific to perineurial cells, and CD34 seems to be immunoreactive to endoneurial fibroblasts.