Current state of spinal nerve sheath tumor management and future advances

Nerve sheath tumors are the most common tumors of the spine after meningiomas. They include schwannomas, neurofibroma, and malignant peripheral nerve sheath tumors. These can arise sporadically or in association with tumor predisposition syndromes, including neurofibromatosis type 1, neurofibromatosis type 2, and schwannomatosis. Though surgery is the traditional mainstay of treatment for these tumors, the discovery of the genetic and molecular basis of these diseases in recent decades has prompted investigation into targeted therapies. Here, we give a clinical overview of spinal nerve sheath tumors, their imaging features, current management practices, and explore ongoing advances in systemic therapies.