DOI: 10.36347/sjams.2016.v04i08.068

Pages: 3083-3086

Malignant peripheral nerve sheath tumor (MPNST) is an extremely rare malignancy in the general population, occurring more frequently in patients with Neurofibromatosis type 1 (NF1). We report imaging features in a 45 year old female patient with Neurofibromatosis 1 and MPNST in upper part of fibular region. Plain radiography and Magnetic Resonance Imaging (MRI) were performed for a correct therapeutic planning. These findings were correlated with histopathological diagnosis which showed Malignant Peripheral Nerve Sheath Tumor (MPNST) with extensive necrosis. The diagnosis of MPNST is extremely difficult due to the lack of conclusive immunohistochemistry or unique chromosomal anomaly, universal distinctive histopathology and clinical criteria. The clinical, radiological and histological presentation of MPNST is important in its diagnosis and this case report will help clinicians in the diagnosis of this rare clinical entity.