Second neoplasms in pediatric patients with
primary central nervous system tumors: the St. Jude Children's Research Hospital
Broniscer A, Ke W, Fuller CE, Wu J, Gajjar A, Kun LE
Department of Hematology-Oncology, St. Jude Children's Research
Hospital, Memphis, Tennessee 38105, USA. email@example.com
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.
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
Cumulative incidence rates were estimated, and
putative risk factors were analyzed.
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
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.
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
Copyright 2004 American Cancer Society
PMID: 15139071 [PubMed - indexed for MEDLINE]