Multiple Hybrid Granular Cell Tumor-Perineuriomas

Abstract

Hybrid peripheral nerve sheath tumors (PNSTs) are recently recognized, rarely reported entities. Very few hybrid PNSTs with elements of perineurioma have been reported. We report a case of a 47-year-old man who presented with multiple (>20) cutaneous nodules on all 4 extremities, present since childhood. The patient and his family had no history of other PNSTs, other neural-origin tumors, or genetic disorders, including neurofibromatosis. Five excised lesions are well circumscribed but unencapsulated nodular tumors composed of cytologically bland spindled cells with tapering nuclei and indistinct cytoplasm arranged in a storiform pattern in a collagenous matrix. Scattered among these cells are large granular eosinophilic cells. The spindled cells are epithelial membrane antigen-positive and S-100 protein negative. The cytoplasm of the granular cells contains abundant S-100 protein, NK1/C3, and CD68-positive granules. Sparse nerve fascicles contain neurofilament-positive axons. Each tumor also contains poorly marginated areas that histologically resemble pure granular cell tumor, and superficial biopsies of these tumors can histologically resemble pure granular cell tumors. Considering a Schwannian origin for both perineurial and granular cells can provide a logical explanation for the pathogenesis of this case. To the best of our knowledge, our case is the first report of multiple hybrid granular cell tumor-perineurioma.