Solitary Intraparotid Plexiform Neurofibroma: Case Report and Literature Review

Abstract

Solitary plexiform neurofibroma is a peripheral nerve sheath tumor with proliferation of Schwann cells accompanied by mast cells and fibroblasts laying down collagen. The occurrence of this tumor in the salivary gland of an individual in the absence of neurofibromatosis 1 (von Recklinghausen disease) is rare. We present a case of sporadic solitary intraparotid neurofibromatosis in an 8-year-old female. The 8 cm × 6 cm × 2 cm mass removed through superficial parotidectomy showed the characteristic irregular bundles of Schwann cells, fibroblasts, mast cells, and strands of collagen. Tissue biopsy is indispensable in the diagnosis of this tumor and its differentiation from other more benign condition such as pleomorphic adenoma which it mimics clinically.