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Management of Optic Chiasmatic/ Hypothalamic Astrocytomas in Children

Published online by Cambridge University Press:  14 September 2018

Paul Steinbok
Affiliation:
Division of Pediatric Neurosurgery, Department of Radiology, University of British Columbia and British Columbia’s Children’s Hospital, Vancouver, BC, Canada
Stephen Hentschel
Affiliation:
Division of Pediatric Neurosurgery, Department of Radiology, University of British Columbia and British Columbia’s Children’s Hospital, Vancouver, BC, Canada
Per Almqvist
Affiliation:
Division of Pediatric Neurosurgery, Department of Radiology, University of British Columbia and British Columbia’s Children’s Hospital, Vancouver, BC, Canada
D. Douglas Cochrane
Affiliation:
Division of Pediatric Neurosurgery, Department of Radiology, University of British Columbia and British Columbia’s Children’s Hospital, Vancouver, BC, Canada
Kenneth Poskitt
Affiliation:
Department of Surgery, and Division of Neuroradiology, Department of Radiology, University of British Columbia and British Columbia’s Children’s Hospital, Vancouver, BC, Canada
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Abstract:

Objective:

The management of optic chiasmatic gliomas is controversial, partly related to failure to separate out those tumors involving the optic chiasm only (chiasmatic tumors) from those also involving the hypothalamus (chiasmatic/hypothalamic tumors). The purpose of this study was: (i) to analyze the outcomes of chiasmatic and chiasmatic/hypothalamic tumors separately; and (ii) to determine the appropriateness of recommending radical surgical resection for the chiasmatic/hypothalamic tumors.

Methods:

A retrospective chart review of all newly diagnosed tumors involving the optic chiasm from 1982-1996 at British Columbia’s Children’s Hospital was performed.

Results:

There were 32 patients less than 16 years of age, 14 with chiasmatic and 18 with chiasmatic/hypothalamic astrocytomas, with an average duration of follow-up of 5.8 years and 6.3 years, respectively. Ten of the patients with chiasmatic tumors and none with chiasmatic/hypothalamic tumors had neurofibromatosis I. Thirteen of the 14 chiasmatic tumors were managed with observation only, and none had progression requiring active intervention. For the chiasmatic/hypothalamic tumors, eight patients had subtotal resections (>95% resection), six had partial resections (50-95%), three had limited resections (<50%), and one had no surgery. There were fewer complications associated with the limited resections, especially with respect to hypothalamic dysfunction. There was no correlation between the extent of resection (subtotal, partial, or limited) and the time to tumor progression (average 18 months).

Conclusions:

In conclusion, chiasmatic and chiasmatic/hypothalamic tumors are different entities, which should be separated out for the purposes of any study. For the chiasmatic/hypothalamic tumors, there was more morbidity and no prolongation of time to progression when radical resections were compared to more limited resections. Therefore, if surgery is performed, it may be appropriate to do a surgical procedure that strives only to provide a tissue diagnosis and to decompress the optic apparatus and/or ventricular system.

Résumé:

Résumé: Objectif:

La prise en charge des gliomes du chiasma optique est controversée, en partie parce qu’on ne fait pas de distinction entre les tumeurs impliquant seulement le chiasma optique (tumeurs chiasmatiques) et celles qui impliquent également l’hypothalamus (tumeurs chiasmatiques/hypothalamiques). Les objectifs de cette étude étaient: (i) d’analyser séparément l’issue des tumeurs chiasmatiques et chiasmatiques/hypothalamiques; et (ii) de déterminer la pertinence de recommander la résection chirurgicale radicale des tumeurs chiasmatiques/hypothalamiques.

Méthode:

Nous avons procédé à une revue rétrospective des dossiers de patients porteurs de tumeurs nouvellement diagnostiquées impliquant le chiasma optique entre 1982 et 1996 au Children’s Hospital de la Colombie Britannique.

Résultats:

L’étude porte sur 32 patients de moins de 16 ans, 14 présentant un astrocytome chiasmatique et 18 un astrocytome chiasmatique/hypothalamique. La durée moyenne du suivi était de 5,8 ans et 6,3 ans respectivement. Dix des patients porteurs de tumeurs chiasmatiques étaient atteints de neurofibromatose de type I alors qu’aucun des patients porteurs de tumeurs chiasmatiques/hypothalamiques n’en étaient atteints. La conduite pour 13 des 14 tumeurs chiasmatiques a été l’observation seulement et aucune n’a nécessité une intervention à cause de la progression de la tumeur. Quant aux patients atteints de tumeurs chiasmatiques/hypothalamiques, huit ont subi une résection subtotale (résection de plus de 95%), six ont subi une résection partielle (de 50 à 95%), trois ont subi une résection limitée (de moins de 50%) et un n’a pas été opéré. Il y a eu moins de complications associées aux résections limitées, surtout quant à la dysfonction hypothalamique. Il n’existait pas de corrélation entre l’étendue de la résection (subtotale, partielle ou limitée) et le moment de la récidive (en moyenne 18 mois).

Conclusions:

Les tumeurs chiasmatiques et chiasmatiques/hypothalamiques sont des entités différentes qui devraient être considérées séparément dans les études. Pour les tumeurs chiasmatiques/hypothalamiques, il y avait davantage de morbidité, sans prolongation du temps de rémission, quand on compare la résection radicale à la résection limitée. Si on a recours à la chirurgie, il peut être approprié de faire une intervention dont le but n’est que de fournir un diagnostic anatomopathologique et de décomprimer l’appareil optique et/ou le système ventriculaire.

Type
Original Article
Copyright
Copyright © Canadian Neurological Sciences Federation 2002

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