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The incidence of brainstem primitive neuroectodermal tumors of childhood based on SEER data

Omar Chamdine1, Ghada Ahmad Saad Elhawary2, Ahmad Samir Alfaar3

  • 1King Fahad Specialist Hospital, Dammam, Saudi Arabia.

Child'S Nervous System : Chns : Official Journal of the International Society for Pediatric Neurosurgery
|January 5, 2018
PubMed

Insights

This study estimates the incidence of Black Swan primitive neuroectodermal tumors (BS-PNET) in children. Older children and those receiving multi-modal therapy showed improved survival outcomes.

Area of Science:

  • Pediatric Oncology
  • Cancer Epidemiology

Background:

  • Black Swan primitive neuroectodermal tumors (BS-PNET) incidence in children is largely unreported.
  • Understanding BS-PNET incidence and outcomes is critical for pediatric cancer research.

Purpose of the Study:

  • To estimate the incidence of pediatric BS-PNET.
  • To report the treatment outcomes for children diagnosed with BS-PNET.

Main Methods:

  • Utilized the Surveillance Epidemiology and End Results (SEER) cancer registry data.
  • Identified 83 pediatric patients (aged 0-21 years) diagnosed between 1973 and 2013.
  • Analyzed outcomes based on age groups and treatment modalities.

Main Results:

  • Median overall survival for pediatric BS-PNET patients was 53 months.
  • Older patients (4-21 years) demonstrated a significant survival advantage (P < 0.001).
  • Multi-modal therapy (surgery, chemotherapy, radiation) and gross/subtotal resection correlated with better survival (P < 0.001).

Conclusions:

  • This study provides the first incidence estimate and largest cohort of pediatric BS-PNET.
  • A high index of suspicion is vital for timely diagnosis, treatment, and improved outcomes in pediatric BS-PNET cases.
Abstract

Keywords:
BiopsyBrainstem tumorEmbryonal tumorOutcome

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