Open AccessMalignant peripheral nerve sheath tumor presenting as orbito temporal lump: Case report and review of literature
Author(s) -
Souvagya Panigrahi,
Sudhansu Sekhar Mishra,
Subhankar Mishra,
Srikant Das
Publication year - 2016
Publication title -
asian journal of neurosurgery
Language(s) - English
Resource type - Journals
ISSN - 1793-5482
DOI - 10.4103/1793-5482.145065
Subject(s) - medicine , malignant peripheral nerve sheath tumor , soft tissue , histopathology , sarcoma , soft tissue sarcoma , trunk , radiation therapy , nerve sheath neoplasm , radiology , neurofibroma , surgery , neurofibromatosis , pathology , ecology , biology
Malignant peripheral nerve sheath tumor (MPNST) is a rare soft tissue sarcoma. The most common anatomical sites include the upper and lower extremities and trunk and less commonly the head and neck. To our knowledge, few patients with a cranial or facial MPNST have been reported. We report such a lesion in a 35-year-old woman who presented with left sided rapidly progressive proptosis and visual loss due to an orbital lump extending up to the temporal lobe. Cranial imaging showed a huge mass invading the orbital wall and temporal bone. The presumptive diagnosis was a malignant orbital tumor. Preoperative fine needle aspiration cytology of the orbital mass came to be neurofibroma. Near total resection of the tumor was done. Histopathology revealed MPNST which was subsequently confirmed on the basis of immunopositivity for S-100. The patient recovered uneventfully and was discharged 8 days after surgery with an advice to attend cancer institute for possible radiotherapy.