Chemotherapy for Supratentorial PNETs in Children

Treatment

• As with high-risk medulloblastomas: Chemotherapy for supratentorial PNETs is the same as that for high-risk medulloblastomas.

Adjuvant Therapy

• See medulloblastomas

Complications

• See medulloblastomas

Outcome

• 30–60% cure rate: The general cure rate varies from 30–60% as a function of the tumor’s location. Tumors in the pineal location have a worse prognosis than do those arising in the CNS parenchyma. The inferior cure rate of this disease is likely related to a different underlying molecular biology than that of medulloblastoma despite the similar histology of the two tumors (50).
• More intense chemotherapy may improve prognosis: In a recent report describing the treatment of a small cohort of 16 children with a more intense chemotherapeutic regimen coupled with a lowering of radiation to 23.4 Gy, 5-year EFS rates were 75% for those with <1.5 cm2 residual tumor after surgery and no dissemination (standard risk) and 60% for those with residual disease or dissemination (high risk) (8). The 5-year EFS rates for patients with pineal and non-pineal tumors were 54 ± 26% and 78 ± 14%, respectively.
• Infants fare worse than older children: Infants treated with protocols of intensive chemotherapy alone had an overall 5-year EFS rate of 39%. Those with tumors in the pineal region had a 5-year EFS rate of 16%. These outcomes have resulted in the consideration of involved field radiation following submyeloablative therapy in this challenging group (15).