A rare case of schwannomatosis

Abstract

Schwannomas are benign,slowly growing, encapsulated peripheral nerve tumors. Schwannomas are homogenous tumors and consist of only Schwann cells. They develop outside of the nerve, but may push it aside or against adjacent structure causing damage to the nerve. Most schwannomas occur as solitary lesions. Presence of multiple schwannomas in a single patient suggeststumorogenesis and a possible association with one of the syndromes such as neurofibromatosis 2 or with Schwannomatosis. Schwannomatosis is diagnosed in the presence of multiple pathologically proven schwannomas in the absence of neurofibromatosis2 (NF2). We report a very rare case of multiple schwannomasin the absence of typical signs and symptoms of NF1 and NF2. Key word: Multiple schwannomas, Schwannomatosis,Schwannoma, Neurofibromatosis I. Introduction: Schwannomas also known as Neurilemmoma or Schwann cell tumor are benign nerve sheath tumor composed of Schwann cells. Their function is to form myelin sheath of nerves and facilitate transmission of nervous impulses. Theycan be found in various part of body with most common site being head. Unlike neurofibromas, schwannomas rarely metastasize and symptoms are because of mass effect of the tumor.Schwannomatosis is defined, therefore, as an extremely rare tumor syndrome characterized by the presence of multiple schwannomas in the absence of typical signs of NF1 and NF2 syndromes. Usually, patients with Schwannomatosis develop cranial, spinal or peripheral nerve schwannomas but do not develop vestibular lesion(VS), typical of NF2 1 .