No coin nor oath required. For personal study only.
Objectives: To determine the long-term, event-free survival (EFS), survival (S), timing and pattern of disease-relapse, and incidence of secondary tumors in children with medulloblastoma (MB) treated on a multi-national, multi-institutional study. Methods: 379 eligible patients, between 3 and 21 years of age with MB, were treated on a prospective randomized study using post-operative reduced-dose (2400 cGy) craniospinal radiotherapy (RT) and adjuvant chemotherapy with vincristine during RT and cisplatin and vincristine plus cyclophosphamide or CCNU post-RT for 8 cycles. Results: At 8 years, EFS was 81Ϯ2% and S was 82Ϯ2%, and did not differ on the basis of type of chemotherapy received. Disease progression, 5 years after treatment, was infrequent, occurring in 7 patients; in 6 it was at the primary site alone. No child had late "spinal" relapse. In comparison, 66% of 61 patients who relapsed prior to year 5 had some component of dissemination. Second malignant neoplasm (SMN) occurred in 8 patients 5 years after diagnosis, including 3 malignant gliomas. Adding these to 4 SMNs which occurred earlier, the 5-year and 8-year SMN incidence was 1.3% and 3.5% (1.4 -5.6%). Conclusions: Long-term survival for children greater than 3 years with nondisseminated medulloblastoma treated in the fashion outlined remains encouraging. Relapse "late" after treatment is relatively infrequent and usually occurs at the primary site. However, secondary, often malignant tumors, are an increasing issue. Long-term follow-up strategies should focus on this pattern of relapse, the detection of secondary neoplasms, and the reality that the majority of patients will survive into adulthood.
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