[1][6] The primary Schwann cell differentiation and neoplastic proliferations are characteristics of peripheral nerve sheath tumors.

Neurofibromas and malignant peripheral nerve sheath tumors can be difficult to distinguish from each other and may require additional testing, including PET scans (18FDG-PET).

[1] Plexiform neurofibromas (associated with neurofibromatosis type 1) have a higher risk of transforming into malignant tumors and require individual treatment plans.

[1] Surgical resection is the treatment of choice for symptomatic plexiform neurofibromas, though a new drug, selumetinib was approved in 2020, as a systemic therapeutic for inoperable cases in pediatric patients.

[1] Post-surgical radiotherapy has shown some promise in improving recurrence-free survival in intermediate and high grade tumors.

[1] Chemotherapy for malignant spinal nerve sheath tumors has shown mixed results and is typically only used in patients in which surgery is not an option, or with aggressive or metastatic disease.

[12] Gamma knife radiosurgery, a subset of SBRT, is an option for peripheral nerve sheath tumors growing near the brain.