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Population-based Study of Medulloblastoma: Outcomes in Alberta from 1975 to 1996

Published online by Cambridge University Press:  02 December 2014

Gloria Roldán ,
Penny Brasher ,
Giacomo Vecil ,
Donna Senger ,
Barry Rewcastle ,
Gregory Cairncross ,
Peter Forsyth  and
Mark Hamilton
Gloria Roldán
Affiliation:
Department of Clinical Neurosciences, Foothills Medical Centre, University of Calgary Department of Oncology, Heritage Medical Research Building,Heritage Medical Research Building, Calgary, Alberta, Canada Clark Smith Integrated Brain Tumor Cancer Centre, Heritage Medical Research Building,Heritage Medical Research Building, Calgary, Alberta, Canada
Penny Brasher
Affiliation:
Department of Anatomic Pathology, Foothills Medical Centre, University of Calgary Department of Oncology, Heritage Medical Research Building,Heritage Medical Research Building, Calgary, Alberta, Canada Division of Population Health and Information, Heritage Medical Research Building,Heritage Medical Research Building, Calgary, Alberta, Canada
Giacomo Vecil
Affiliation:
Department of Neurosurgery, University of Texas, Health Science Center, San Antonio, Texas, USA.
Donna Senger
Affiliation:
Department of Clinical Neurosciences, Foothills Medical Centre, University of Calgary Department of Oncology, Heritage Medical Research Building,Heritage Medical Research Building, Calgary, Alberta, Canada
Barry Rewcastle
Affiliation:
Department of Clinical Neurosciences, Foothills Medical Centre, University of Calgary Department of Anatomic Pathology, Foothills Medical Centre, University of Calgary
Gregory Cairncross
Affiliation:
Department of Clinical Neurosciences, Foothills Medical Centre, University of Calgary Department of Oncology, Heritage Medical Research Building,Heritage Medical Research Building, Calgary, Alberta, Canada Clark Smith Integrated Brain Tumor Cancer Centre, Heritage Medical Research Building,Heritage Medical Research Building, Calgary, Alberta, Canada
Peter Forsyth*
Affiliation:
Department of Clinical Neurosciences, Foothills Medical Centre, University of Calgary Department of Oncology, Heritage Medical Research Building,Heritage Medical Research Building, Calgary, Alberta, Canada Clark Smith Integrated Brain Tumor Cancer Centre, Heritage Medical Research Building,Heritage Medical Research Building, Calgary, Alberta, Canada
Mark Hamilton
Affiliation:
Department of Clinical Neurosciences, Foothills Medical Centre, University of Calgary Division of Neurosurgery, Foothills Medical Centre, University of Calgary Clark Smith Integrated Brain Tumor Cancer Centre, Heritage Medical Research Building,Heritage Medical Research Building, Calgary, Alberta, Canada
*
Clark Smith Brain Tumor Center, Rm. 2AA19 Health Research Innovation Center, 3330 Hospital Drive NW, Calgary, Alberta, T2N 4N1, Canada
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Abstract

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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:

<span class='bold'>RÉSUMÉ:</span> <span class='bold'> <span class='italic'>Contexte:</span></span>

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.

<span class='bold'> <span class='italic'>Méthodes:</span></span>

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.

<span class='bold'> <span class='italic'>Résultats:</span></span>

La survie globale à 5 ans était de 50%. La longueur de la survie avait tendance à être associée à l’étendue de la chirurgie, sans atteindre le seuil de la signification statistique (Long rank test, p < 0,06). La médiane de survie sans récidive était de 22,4 mois (écart de 6,4 à 192,3 mois) après le diagnostic et la survie médiane après la récidive était de 9,3 mois (écart de 0,4 à 64,9 mois). La courbe de suivie ne semblait pas atteindre de plateau et elle était influencée par le décès dû à la tumeur chez 3 des 21 survivants à long terme dont le diagnostic avait été posé dans l’enfance. La moyenne de survie sans récidive chez ces 3 patients était de 11,7 ans après le diagnostic et donc ils sont des contrevenants à la loi de Collins.

<span class='bold'> <span class='italic'>Conclusion:</span></span>

Le taux de survie dans une population non sélectionnée de patients atteints de médulloblastome est faible. Une résection agressive de la tumeur prolonge la survie. Les contrevenants à la loi de Collins étaient relativement fréquents et nous proposons que ce concept soit abandonné en ce qui concerne le médulloblastome.

Type
Original Articles
Information
Canadian Journal of Neurological Sciences , Volume 35 , Issue 2 , May 2008 , pp. 210 - 215
Copyright
Copyright © The Canadian Journal of Neurological 2008

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