Survival and late mortality in long-term survivors of pediatric CNS tumors.
OBJECTIVE
To describe the pattern of survival and late mortality among contemporary long-term survivors of pediatric CNS tumor.


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 > or = 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).


CONCLUSIONS
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.
Morris
E Brannon
EB
Department of Oncology, St Jude Children's Research Hospital, Memphis, TN 38105, USA. brannon.morris@stjude.org
Gajjar
Amar
A
Okuma
James O
JO
Yasui
Yutaka
Y
Wallace
Dana
D
Kun
Larry E
LE
Merchant
Thomas E
TE
Fouladi
Maryam
M
Broniscer
Alberto
A
Robison
Leslie L
LL
Hudson
Melissa M
MM
D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D002423:Cause of Death; D016543:Central Nervous System Neoplasms / Q000401:mortality* / Q000473:pathology / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D018450:Disease Progression; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D015994:Incidence; D008297:Male; D009367:Neoplasm Staging; D011379:Prognosis; D012042:Registries; D012189:Retrospective Studies; D018570:Risk Assessment; D017678:Sex Distribution; D016019:Survival Analysis; D017741:Survivors / Q000706:statistics & numerical data*; D013997:Time Factors