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Late recurrence of primitive neuroectodermal tumor/medulloblastoma
I B Lefkowitz1, R J Packer, S G Ryan
1Neuro-Oncology Program, Children's Hospital of Philadelphia, PA 19104.
Cancer
|August 15, 1988
Summary
The risk period for primitive neuroectodermal tumor/medulloblastoma recurrence is undefined. Late recurrences occurred in 43% of patients, challenging Collins' law and suggesting an indefinite risk period for these central nervous system tumors.
Area of Science:
- Pediatric Oncology
- Neuro-oncology
- Clinical Outcomes Research
Background:
- Primitive neuroectodermal tumor/medulloblastoma (PNET/MB) recurrence risk period is not well-defined.
- Increased survival rates for PNET/MB necessitate understanding late recurrence patterns.
- Collins' law, a proposed cure criterion for embryonal tumors, is applied to PNET/MB.
Purpose of the Study:
- To determine the incidence of late PNET/MB recurrence.
- To identify factors influencing PNET/MB recurrence.
- To assess the applicability of Collins' law to PNET/MB patients.
Main Methods:
- Retrospective study of 44 PNET/MB patients diagnosed between 1970-1984.
- Analysis of disease-free survival at 5, 10, and 12 years.
- Evaluation of patient age, sex, radiotherapy dose, chemotherapy, and surgery extent.
Main Results:
- Disease-free survival rates were 54% at 5 years, 41% at 10 years, and 30% at 12 years.
- For 5-year survivors, actuarial survival was 75% at 10 years and 51% at 12 years.
- Recurrence outside the predicted 'period of risk' by Collins' law occurred in 43% of late-relapsing patients.
Conclusions:
- Collins' law appears insufficient for defining cure in PNET/MB.
- Age, sex, and treatment modalities did not predict late PNET/MB relapse.
- The 'period of risk' for PNET/MB recurrence is likely undefined and potentially indefinite.