24-year-old woman with an internal auditory canal mass. Hybrid peripheral nerve sheath tumor with schwannoma/perineurioma components.

Benign peripheral nerve sheath tumors are divided into schwannomas, neurofibromas and perineuriomas. In recent years, tumors with hybrid features, composed of multiple, discrete areas of different histological types, were described. These tumors may represent a diagnostic challenge. A 24-year-old woman with multiple sclerosis was found to have a 1.3 cm TV × 0.7 cm AP T2 intermediate lesion within the left internal auditory canal. Gross examination revealed a tan-white, well circumscribed mass. Histologic examination demonstrated a well demarcated, cellular, solid neoplasm with a biphasic pattern. Most of the tumor was composed of spindle cells arranged in fascicles with focal Verocay body formation and diffuse S100 positivity. A second, minor area showed concentric proliferation of neoplastic spindle cells around one or more axons. Tumor cells in this area were positive for perineurial markers, claudin-1 and Glut-1, and focally immunopositive for CD34.We present here a case of a benign peripheral nerve sheath tumor with histological and immunohistochemical features consistent with a dual pattern of differentiation of schwannoma and perineurioma, in the VIIIth cranial nerve. This is, to our knowledge, the first case of a hybrid perineurioma/schwannoma reported in a cranial nerve.