A rare case of schwannomatosis
Journal Title: Medpulse International Journal of Surgery - Year 2017, Vol 5, Issue 1
Abstract
Schwannomas are benign, slow growing, encapsulated tumour of peripheral nerve which contain of only schwann cells. They develop on the outside of the nerve, but may push it aside or against adjacent structures causing damage. Most schwannomas occur as solitary lesions. However, schwannomatosis is uncommon, Patients with schwannomatosis may develop multiple schwannomas on cranial, spinal and peripheral nerves, but they do not develop vestibular schwannomas (VS). Neurofibromatosis is an autosomal dominant disorder, which means only one copy of the affected gene is needed for the disorder to develop. Therefore, if only one parent has neurofibromatosis, his or her children have a 50% chance of developing the condition as well. The affected child could have mild NF1 even though inherited from a parent with a severe form of the disorder. Schwannomatosis, in which painful schwannomas develop on spinal and peripheral nerves. They do not develop other tumours such as meninigiomas, ependymomas or astrocytomas6. Patients with schwannomatosis usually present with pain8 whereas NF2 patients present with neurologic deficit2. Molecular and genetic analysis also suggests that schwannomatosis is a distinct genetic and clinical syndrome9. Key Words:
Authors and Affiliations
Thyagaraj . , Suhas . , Manashree . , Pritam Patil
Keywords
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