Patients with neurofibromatosis type 1 (NF1), one of the most common genetic disease affecting the nervous system, develop multiple neurofibromas that can transform into aggressive sarcomas known as malignant peripheral nerve sheath tumors (MPNSTs). Studies of human tumors and newly developed transgenic mouse models indicate that Schwann cells are the primary neoplastic cell type in neurofibrom...

Malignant peripheral nerve sheath tumours are uncommon aggressive soft tissue sarcomas that originate from peripheral nerves or cells associated with the nerve sheath. Scalp malignant peripheral nerve sheath tumours are extremely rare and the role of multimodality management is unclear. We report a 43-year-old female with scalp swelling and rapid growth. Imaging demonstrated an ...

BACKGROUND Malignant peripheral nerve sheath tumors (MPNSTs) arise from a peripheral nerve or display nerve sheath differentiation. Most MPNSTs typically originate on the trunk, extremities, head, neck, and paravertebral regions. Gastrointestinal MPNSTs are rare entities with only 10 cases reported worldwide in the literatures. CASE PRESENTATION Here, we report the first Chinese case of a mal...

Background and Objective: Approximately 45% of benign peripheral nerve sheath tumors (PNST) occur in head and neck regions. Malignant peripheral nerve sheath tumors (MPNST) are less common and only 815%of these tumors occur in head and neck region. In this study, we aimed at evaluating the demographic characteristics, pathologic findings, locations, main clinical presentations and family histor...

Malignant triton tumor (MTT) is a rare variant of malignant peripheral nerve sheath tumor (MPNST) with rhabdomyosarcomatous differentiation. We report the case of a 54-year-old male without a history of neurofibromatosis type 1 (NF1) who had a growing abdominal wall tumor diagnosed as MTT. Computed tomography (CT), magnetic resonance imaging (MRI) and 2-[F-18]-fluoro-2-deoxy-D-glucose positron ...

BACKGROUND AND IMPORTANCE Malignant peripheral nerve sheath tumors are the most common malignant mesenchymal tumors of soft tissues, but they are very rare when found to arise from a cranial nerve and when not in association with neurofibromatosis. These tumors are highly malignant and carry a poor prognosis with survival usually less than 6 months. CLINICAL PRESENTATION The authors report th...