Second Neoplasms in Pediatric Patients with Primary Central Nervous System Tumors: The St. Jude Children's Research Hospital Experience

Alberto Broniscer, Weiming Ke, Christine E. Fuller, Jianrong Wu, Amar Gajjar, Larry E. Kun

Research output: Contribution to journalReview articlepeer-review

96 Scopus citations


BACKGROUND. Details on second neoplasms (SNs) following pediatric central nervous system (CNS) tumors are scant, because of the rarity of such SNs. The goal of the current study was to investigate and characterize these rare SNs. METHODS. The authors reviewed clinical and treatment data on all institutional patients age < 22 years at diagnosis of a primary CNS tumor who developed any type of SN. Patients with neurofibromatosis type 1 were excluded. Cumulative incidence rates were estimated, and putative risk factors were analyzed. RESULTS. The SNs investigated in the current study included 10 gliomas (42%), 5 meningiomas (21%), 2 desmoid tumors, 2 myelodysplastic syndromes, 2 basal cell carcinomas, 1 leukemia, 1 malignant fibrous histiocytoma, and 1 thyroid carcinoma. Twenty-one patients had previously received radiotherapy, and 12 patients had received chemotherapy. The SN was related to a genetic cause in 7 patients (29%). Eleven patients died of their SNs, including 8 patients with glioma and 2 patients with myelodysplastic syndromes. The estimated 15-year cumulative incidence rate for malignant SNs was 4%. Children with choroid plexus tumors had an estimated 10-year cumulative incidence rate of 20.2%; 2 of those patients had germline TP53 mutations. Age ≤ 2 years was a significant risk factor (P = 0.016) for development of an SN only when patients with genetic conditions were included in the analysis. No significant difference in the estimated cumulative incidence of SNs was found among patients who had received different types of therapy. CONCLUSIONS. The risk of lethal SNs after pediatric CNS tumors is small. Young patients and patients with choroid plexus tumors appear to have an increased risk of SNs that is associated with genetic factors.

Original languageEnglish
Pages (from-to)2246-2252
Number of pages7
Issue number10
StatePublished - May 15 2004


  • Brain
  • Central nervous system
  • Children
  • Genetic predisposition
  • Risk
  • Second neoplasms

ASJC Scopus subject areas

  • Oncology
  • Cancer Research


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