Intramedullary Spinal Cord Tumors Associated with the Neurofibromatoses.

The neurofibromatoses (NF) comprise several tumor predisposition conditions, including neurofibromatosis type 1 (NF1) and neurofibromatosis type 2 schwannomatosis (NF2-SWN). Although paraspinal peripheral nerve sheath tumors (including neurofibromas and schwannomas) occur frequently in the NF conditions, there is also a risk of intramedullary spinal cord tumors in people with NF1 and NF2-SWN. Here, we discuss the presentation and diagnosis of the various forms of NF, the intramedullary spinal cord tumors that occur in NF1 and NF2-SWN, and their diagnostic and treatment considerations.