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Second neoplasms in pediatric patients with primary central nervous system tumors: the St. Jude Children`s Research Hospital experience.

Al Musella's Comments: (This is his personal views and are not necessarily the views of the Musella Foundation!)


Posted on: 06/08/2004

Second neoplasms in pediatric patients with primary central nervous system tumors: the St. Jude Children's Research Hospital experience.

1: Cancer. 2004 May 15;100(10):2246-52.

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.

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
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.

Copyright 2004 American Cancer Society. PMID: 15139071 [PubMed - indexed for MEDLINE]

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