This is also called primitive neuroectodermal tumor (PNET). It arises in the cerebellum and may spread to adjacent tissues. It also can spread via the cerebrospinal fluid to the rest of the brain or the spinal cord and very rarely to sites outside the nervous system.
Standard Treatment An attempt is made to surgically remove as much tumor as possible. Studies are done during and after the operation to define the risk of relapse, and treatment is given according to the best estimate of low-stage or high-stage disease.
For low-stage disease, standard therapy after surgery is a high dose of radiation to the tumor area and a lower dose to the entire brain and spine. Lowering the dosage of radiation to reduce problems with nervous system development reduces the chance for cure. Children under three should be entered in studies that use chemotherapy and probably delayed or modified radiation therapy .
Treatment of high-stage disease involves chemotherapy in addition to surgery and radiation therapy similar to that given in low-stage disease.
These patients should be considered for entry into clinical trials to establish the best combination and sequence of chemotherapy.
