Second Primary Neoplasms (SPNS) Of The Central Nervous System (CNS) In Survivors Of Childhood Cancer - A Report From The Childhood Cancer Survivor Study (CCSS)
Diana Stripp, MD
University of Pennsylvania Cancer
Last Modified: May 14, 2001
Presenter: J. Neglia
Affiliation: Childhood Caner Survivor Study, University of Minnesota, Minneapolis, MN
- Second neoplasms of the CNS are well- recognized events among survivors of childhood cancer, often following leukemia or primary CNS tumors.
- The CCSS is a multi-institutional retrospective cohort study created as a resource for the study of late events.
Materials and Methods:
- 20,245 incident cases of common childhood cancers between 1970 and 1986, under age 21, and surviving 5+ years were identified at the 25 CCSS institutions.
- 17,344 self-administered questionnaires were mailed.
- Among 13,581 responded survivors (median age 26 years) included in this analysis, 488 SPNs were confirmed (pathology reports) among 428 individuals.
- Most common SPNs are: breast ca (17%), thyroid cancer (12.2%), malignant CNS tumor (10.2%) and meningioma (10.3%).
- Of these, 74 SPNs arose in the CNS-- 36 meningiomas (all benign), 30 glial tumors, 4 primitive neuroectodermal tumors and four other CNS tumors. Thirty-nine followed a primary diagnosis of leukemia; 23 followed a primary CNS tumor.
- Malignant CNS tumors occurred a median of 9.5 years from the original cancer and meningiomas a median of 13.3 years.
- Compared to general population, there is a significantly elevated excess risk of CNS tumors may persisted up to 30 years from diagnosis.
- In multivariate analyses, cranial radiation, age < 5 year, leukemia as an original diagnosis (relative risk of 2.56 vs. non-leukmia) and original diagnosis of CNS tumor (R.R. 2.76 vs. leukemia) were identified as independent predictors for any CNS SPN.
- Survival was poor following malignant CNS SPNs with only 4 of 17 patients with GBM or anaplastic astrocytoma alive at follow up
- The three most common predictors of developing SPN in this cohort are: youg age, leukemia and CNS tumor as an original diagnosis.
- Despite the increased risk observed, the absolute excess risk of secondary malignant tumors of the CNS was low, with 1.1 excess cases occurring per ten thousand person-years of follow up.
- Additional investigation of radiation and genetic-specific risk factors are underway and may facilitate the development of more effective primary and secondary preventive strategies.
- With novel therapeutic approaches, we might be able to modifiy the current treatment modalities in order to decrease the risk of the development of SPN following therapy.
- Need to consider the susceptability (ie. genetic, treatment related) of the host for developing SPN and develop specific treatment modalities for these high risk patients.
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