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Focal Midbrain Glioma: Long Term Survival in a Cohort of 16 Patients and the Implications for Management

Published online by Cambridge University Press:  18 September 2015

Mark G. Hamilton*
Affiliation:
Department of Clinical Neuroscience, University of Calgary, Calgary
Carl Lauryssen
Affiliation:
Department of Clinical Neuroscience, University of Calgary, Calgary
Neil Hagen
Affiliation:
Department of Clinical Neuroscience, University of Calgary, Calgary
*
Alberta Children’s Hospital, 1820 Richmond Road S.W., Calgary, Alberta, Canada T2T 5C7
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Abstract

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Background: Focal gliomas involving the midbrain tectum and tegmentum have been identified as having a better prognosis than diffuse tumors affecting the brain stem. However, only limited information is available concerning treatment effectiveness and long term outcome for these patients. Methods: A retrospective, population-based cancer registry survey was performed to assess the clinical features and treatment courses of patients with focal midbrain tumors. Results: Sixteen patients with midbrain gliomas were identified; eight had tectal gliomas and eight tegmental gliomas. Thirteen patients presented with symptoms related to hydrocephalus, and 12 required a ventriculoperitoneal shunt. Seven patients underwent surgery directed at the tumor. Eight patients underwent initial radiation therapy and none had initial chemotherapy. One patient diagnosed at age 18 months had a rapidly growing tumor after 14 months of follow up which has responded to chemotherapy. The mean survival of this patient population was 84 months (range 3-280 months) after diagnosis, with only one tumor related death occurring (280 months after diagnosis). Survival was not affected by tumor location within the midbrain (tegmental or tectal) or by whether radiation therapy was or was not administered. Conclusions: Patients with focal midbrain gliomas require symptom control aimed at treatment of hydrocephalus, or mass effect from the tumor. However the extended survival of this population suggests that routine aggressive surgical debulking is often not required. Furthermore, the routine use of radiation therapy or chemotherapy for all such patients is questioned.

Résumé

Résumé

Gliome en foyer du mésencéphale: survie à long terme dans une cohorte de 16 patients et implications pour le traitement. Introduction: On sait que les gliomes en foyer impliquant le toit du mésencéphale ou la calotte, ont un meilleur pronostic que les tumeurs diffuses du tronc cérébral. Cependant, il existe peu d’information sur l’efficacité du traitement et l’issue à long terme chez ces patients. Méthodes: Nous avons effectué une étude rétrospective de population au moyen d’un registre de cancers afin de déterminer les caractéristiques cliniques et le mode de traitement des patients atteints de tumeurs en foyer du mésencéphale. Résultats: Seize patients porteurs de gliomes du mésencéphale ont été identifiés; huit avaient des gliomes du toit et huit avaient des gliomes de la calotte. Treize patients avaient consulté pour des symptômes en relation avec une hydrocéphalie et 12 avaient eu besoin d’une dérivation ventriculopéritonéale. Sept patients ont subi une chirurgie pour leur tumeur. Huit patients ont eu d’abord de la radiothérapie et aucun n’a eu initialement une chimiothérapie. Un patient, diagnostiqué à l’âge de 18 mois, avait une tumeur dont la croissance a été rapide au cours des 14 mois de suivi. Ce patient a bien répondu à la chimiothérapie. La survie moyenne de cette population de patients était de 84 mois (écart de 3 à 280 mois) après le diagnostic. Un seul décès était en relation avec la tumeur (280 mois après le diagnostic). La survie n’était pas influencée par le site de la tumeur dans le mésencéphale (à la calotte ou au toit) ou par l’administration de radiothérapie. Conclusions: Nous concluons que les patients qui ont des gliomes en foyer du mésencéphale ont besoin qu’on contrôle leurs symptômes en traitant leur hydrocéphalie ou l’effet de masse de la tumeur. Cependant, la survie prolongée de ces patients suggère qu’une chirurgie agressive effectuée de routine n’est souvent pas requise. De plus, nous remettons en question l’administration de routine de radiothérapie ou de chimiothérapie chez ces patients.

Type
Original Articles
Copyright
Copyright © Canadian Neurological Sciences Federation 1996

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