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Pineal and nonpineal supratentorial primitive neuroectodermal tumors
1Children's Hospital of Pittsburgh, 3520 Fifth Avenue, Suite 205, Pittsburgh, PA 15213, USA. jakackr@chplink.chp.edu
Abstract:
Pineal region supratentorial primitive neuroectodermal tumors (SPNETs; pineoblastomas) and nonpineal SPNETs are rare tumors that historically have carried a very poor prognosis. With multimodality therapy, including maximal surgical resection, craniospinal radiation therapy and chemotherapy, the survival for patients with pineal PNETs has significantly improved. Chemotherapy alone, at least in conventional doses, appears to be insufficient treatment for younger children with pineoblastomas, in whom there is almost universal rapid tumor progression and death. Survival of patients with nonpineal SPNETs remains in the order of 30-35% despite multimodality therapy. Unlike those with pineal SPNETs, a significant percentage of infants with nonpineal SPNETs who undergo gross total surgical resection followed by chemotherapy will be long-term survivors. This article gives an overview of the natural history, prognostic factors and treatment of both pineal and nonpineal SPNETs.
Insights
Multimodality therapy significantly improves survival for pineal primitive neuroectodermal tumors (SPNETs). However, nonpineal SPNETs still have a poor prognosis, though infants may survive with aggressive treatment.
Area of Science:
- Pediatric Oncology
- Neuro-oncology
- Tumor Biology
Background:
- Supratentorial primitive neuroectodermal tumors (SPNETs), including pineoblastomas, are rare and historically had poor prognoses.
- Multimodality therapy has improved outcomes for pineal SPNETs, but nonpineal SPNETs remain challenging.
Purpose of the Study:
- To provide an overview of the natural history, prognostic factors, and treatment strategies for pineal and nonpineal SPNETs.
- To highlight the impact of multimodality therapy on survival rates for different SPNET subtypes.
Main Methods:
- Review of existing literature on pineal and nonpineal SPNETs.
- Analysis of treatment outcomes based on tumor location and therapeutic interventions.
Main Results:
- Multimodality therapy, including surgery, radiation, and chemotherapy, has significantly improved survival for pineal SPNETs.
- Chemotherapy alone is insufficient for young children with pineoblastomas, leading to rapid progression.
- Survival for nonpineal SPNETs remains around 30-35% despite aggressive treatment.
- Infants with nonpineal SPNETs may achieve long-term survival with gross total resection and chemotherapy.
Conclusions:
- Treatment advancements have improved outcomes for pineal SPNETs.
- Nonpineal SPNETs present a significant therapeutic challenge, particularly in older children.
- Tailored treatment approaches considering tumor location and patient age are crucial for optimizing SPNET management.