Peranan Radiologi dan Radioterapi pada Penatalaksanaan Malignant Peripheral Nerve Sheath Tumor

Abstract

Malignant peripheral nerve sheath tumor (MPNST) is biologically an aggressive tumor of soft tissue sarcoma. MPNSTs represent 5–10% of all soft-tissue sarcomas. They correspond to malignant forms of neurofibromas and schwannomas. The main clinical symptoms of MPNST are increasing size of tumors, local or radicular pain, paraparesis, and paresthesia and/or weakness of extremities.Radiological imaging is important to determine the site and extension of the tumor, especially before surgery. Magnetic resonance imaging (MRI) is the imaging modality of choice. To some extent, MPNSTs share basic imaging characteristics with benign peripheral nerve sheath tumor, although there are some evidence of malignant transformation. Diagnosis may be challenging because there are no specific immunohistochemical or molecular markers. However, Histopathological examination is needed for definitive diagnosisThe mainstay of treatment is surgical resection. The goal of the operation is to achieve complete surgical excision of the tumor with negative (wide) margins. Together with wide surgical excision, radiation therapy offers the best outcome of local and overall survival rates.