Management of Supratentorial Primitive Neuroectodermal Tumors in Children

Initial Management at Presentation

  • Safe surgical resection: The removal of a maximal volume of tumor is the first line of treatment.

Adjunctive Therapies

  • Craniospinal irradiation after resection if > 3 years: There are currently no standard dosing regimens, but the reported ranges include 4680–6000 cGy to the primary site and 2700–4000 cGy to the cranial and spinal fields. Currently, there is no agreement on the use of chemotherapy for children with supratentorial PNETs. The effectiveness of adjuvant combination chemotherapy for medulloblastomas has led to a similar strategy for supratentorial PNETs.

Follow-up

  • Multidisciplinary team with scheduled appointments: Follow-up is best done by a multidisciplinary team with periodic MRI scans of the brain and spine to determine the status of the disease.