Medulloblastoma is the most common malignant brain tumor of the central nervous system (CNS) in children, accounting for approximately 20% of all pediatric brain tumors.1,2 It is comparatively rare in adults, with a reported incidence rate of 0.05/100,000 per year, accounting for 1% of all primary tumors of the CNS. 3,4 There are few reported studies concerning adults and when reported, adults constitute a small part of the study group. [5][6][7] Previous studies of outcomes in medulloblastoma patients are based on case series or institutional experiences which were limited by referral and selection biases. 8,9 To our knowledge, there are no population-based studies of children and ABSTRACT: Background: The purpose of this study was to determine incidence, survival rate, and prognostic factors as well as the frequency of Collins' Law Violators (CLVs) in an unselected population of medulloblastoma patients. Collins' Law dictates that 'cure' of a child with a tumor occurs after a period that includes the child's age at diagnosis plus 9 months. Methods: Using the Alberta Cancer Registry a population-based review identified 49 patients with medulloblastoma (19 adults, 30 children) diagnosed from 1975-96. Pathology was reviewed in all cases. All patients had surgical resection, followed by radiotherapy in 47 patients and chemotherapy in 17. Results: The overall 5-year survival was 50%. There was a trend for the extent of resection to be associated with a longer survival (Long rank test, p< 0.06) but this was not significant. Tumor recurrence occurred a median of 22.4 months (range, 6.4-192.3) after diagnosis and median survival after recurrence was 9.3 months (range, 0.4-64.9). The survival curve did not appear to plateau but was affected by tumor-related deaths in 3 (21.4%) of the 21 long-term survivors diagnosed in childhood. These three patients had recurrences a mean of 11.7 years after diagnosis and are designated as CLVs. Conclusions: The survival rate in an unselected population of patients with medulloblastoma is poor. Aggressive resection of the tumors prolongs survival. The Collins' Law Violators were relatively common and we suggest this concept be abandoned in medulloblastoma.
RÉSUMÉ: Étude de population sur le médulloblastome en Alberta de 1975 à 1996.Contexte : Le but de cette étude était d'évaluer l'incidence, le taux de survie et les facteurs influençant le pronostic ainsi que la fréquence des contrevenants à la loi de Collins dans une population non sélectionnée de patients atteints de médulloblastome. Selon la loi de Collins, un enfant atteint d'une tumeur est guéri après un temps défini par l'âge de l'enfant au moment du diagnostic plus 9 mois. Méthodes : Nous avons identifié 49 patients atteints de médulloblastome (19 adultes et 30 enfants) dans le Alberta Cancer Registry, entre 1975 et 1996. L'anatomopathologie a été révisée dans tous les cas. Tous les patients avaient subi une résection chirurgicale suivie de radiothérapie chez 47 patients et de chimiothérapie chez 17 patients. Résultats : La su...