James Hayden

Supratentorial Primitive Neuroectodermal Tumor: Therapy

Supratentorial primitive neuroectodermal tumors (StPNET) are a group of highly aggressive cancers that account for 2–3% of all brain tumors in childhood arising within the pineal gland and at non-pineal sites. These tumors share many histological features with their infratentorial counterpart (medulloblastoma) as well as a propensity for leptomeningeal dissemination and accordingly they have traditionally been treated along similar lines. More recently, however, it has been shown that the biological mechanisms underlying the development of StPNETS and medulloblastoma differ significantly. As with medulloblastomas, StPNETs are both radio and chemo sensitive although the outcome for patients treated using similar regimens of multi-model therapy with surgery, radiotherapy and chemotherapy in clinical trials is consistently significantly inferior. Young children under the age of 3 years have a dismal prognosis. Large multicentre trials specifically for patients with StPNETs are now required, including the identification of clear prognostic clinical and biological prognostic factors. In future studies should aim to exploit StPNET biological targets to develop novel disease specific therapies to improve prognosis.