Sarcoma and the nervous system

Sarcomas are neoplasms arising from mesenchymal progenitor cells which differentiates them from other central nervous system (CNS) tumors, such as gliomas, which arise from cells that are ectodermal in origin. Primary tumors arising from these cell types are infrequently seen in the CNS. Many CNS sarcomas, when they do occur, most commonly arise in the pediatric population. Our understanding of their epidemiology is lacking given rarity. This chapter will detail sarcomas which arise within the CNS, as well as those which occur adjacent to the CNS, in bony tissue for example, and can unfavorably impact the CNS. These rare tumors are often managed by multidisciplinary teams which include neuro-oncologists, pediatric neuro-oncologists, oncologists, neurosurgeons, orthopedic surgeons, radiation oncologists, general surgeons, neuroradiologists, and pathologists.