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Survival and Late Mortality in Long-Term Survivors of Pediatric CNS Tumors

E. Brannon Morris, Amar Gajjar, James O. Okuma, Yutaka Yasui, Dana Wallace, Larry E. Kun, Thomas E. Merchant, Maryam Fouladi, Alberto Broniscer, Leslie L. Robison, Melissa M. Hudson

From the Departments of Oncology, Biostatistics, Radiological Sciences, Epidemiology and Cancer Control, St Jude Children's Research Hospital; University of Tennessee College of Medicine, Memphis, TN; and the Department of Public Health Sciences; School of Public Health, University of Alberta, Edmonton, Alberta, Canada

Address reprint requests to E. Brannon Morris, MD, Division of Cancer Survivorship, St Jude Children's Research Hospital, 332 N Lauderdale St, Memphis, TN 38105; e-mail: brannon.morris{at}stjude.org

Purpose To describe the pattern of survival and late mortality among contemporary long-term survivors of pediatric CNS tumor.

Patients and Methods The study population comprised 643 pediatric patients with primary CNS tumor treated at St Jude Children's Research Hospital (Memphis, TN) from 1985 to 2000 who survived 5 years from diagnosis. Patients were classified according to primary tumor type, location of tumor, and survival. Cause of death was obtained from the medical record and categorized as progression, malignant transformation, second malignancy, medical complication, or external cause.

Results Overall survival estimates for patients who survived at least 5 years postdiagnosis was 91.3% ± 2% and 86% ± 3% at 10 and 15 years postdiagnosis, respectively. A significant difference in the survival rates according to original tumor type (P = .001) was seen. Sixty-six (10%) of 643 patients experienced late mortality: 38 patients (58%) died of progressive disease while 14 patients (21%) died of second malignant tumor. Twelve patients (18%), predominantly with diencephalic tumor location, died of a specific medical cause: cardiovascular disease (n = 2), cerebrovascular accident (n = 1), metabolic collapse and/or sepsis (n = 7), respiratory failure (n = 1), or shunt malfunction (n = 1).

Conclusion Late mortality occurs in a substantial number of long-term survivors of pediatric CNS tumors and is most influenced by the initial tumor histopathology. Progressive disease remains the most common cause of death within the first decade of diagnosis. Teenage patients requiring treatment for panhypopituitarism may be especially vulnerable and deserve significant medical surveillance.

Supported by the Cancer Center Support Grant No. P30 CA 21765 from the National Institutes of Health, by the Noyes Foundation, Musicians Against Childhood Cancer, and by the American Lebanese Syrian Associated Charities.

Authors' disclosures of potential conflicts of interest and author contributions are found at the end of this article.

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