Primitive neuroectodermal tumors (PNETs) are rare, malignant tumors thought to arise from primitive nerve cells. Several treatment options are available; treatment typically includes a combination of surgery, radiation therapy, chemotherapy and possibly shunt placement.
PNETs can develop in the central nervous system or peripheral nervous system. This page discusses those that arise in the central nervous system. For more information about PNETs found in the peripheral nervous system, where they are also called Ewing sarcoma, please visit the page on the Spine Hospital site here.
The primitive nerve cells from which PNETs develop are left over from the development of the nervous system during gestation. Normally, these immature cells do not form tumors, but develop into neurons, in a process that continues well into adulthood. But in rare instances, a tumor can arise.
Located typically in the cerebrum, PNETs are fast-growing and counted among the most aggressive kinds of tumors found in children. The tumors tend to spread easily through cerebrospinal fluid. In some cases, PNETs may spread by means other than cerebrospinal fluid beyond the central nervous system. Also, these tumors can block the flow of cerebrospinal fluid, causing a condition called hydrocephalus.
A note on nomenclature: As medical research advances, more becomes known about the characteristics of each disease, as well as the relationships among disorders. New evidence has emerged in the study of PNET, and its classification is being refined. This tumor type has now technically been incorporated into the category embryonal tumor with multilayered rosettes (ETMR). The neurosurgeons at Columbia stay up-to-date on such developments; however, PNET is the more familiar term among patients in the clinic, so the name PNET is used on this page.
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