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Ann Endocrinol (Paris). 2014 Oct;75 Suppl 1:S46-54. doi: 10.1016/S0003-4266(14)70026-5.

[Craniopharyngiomas: progress in pathogenesis and therapeutics].

[Article in French]

Author information

1
Service d'endocrinologie et des maladies de la reproduction, Centre de référence des maladies endocriniennes rares de la croissance, Hôpital de Bicêtre (APHP), F94275 Le Kremlin-Bicêtre ; Université Paris-Sud 11 (ML, PC), UMR S693, Faculté de médecine Paris-Sud, F94276 Le Kremlin-Bicêtre ; INSERM U693 (ML, PC), faculté de médecine, F94276 Le Kremlin-Bicêtre, France.
2
Service d'endocrinologie et des maladies de la reproduction, Centre de référence des maladies endocriniennes rares de la croissance, Hôpital de Bicêtre (APHP), F94275 Le Kremlin-Bicêtre ; Université Paris-Sud 11 (ML, PC), UMR S693, Faculté de médecine Paris-Sud, F94276 Le Kremlin-Bicêtre ; INSERM U693 (ML, PC), faculté de médecine, F94276 Le Kremlin-Bicêtre, France. Electronic address: philippe.chanson@bct.aphp.fr.

Abstract

This review focuses on new aspects in craniopharyngiomas with emphasis on pathogenic mechanisms and treatment strategies that were presented at the joined Endocrine Society/International Society of Endocrinology meeting in Chicago in June 2014. Craniopharyngiomas are benign epithelial tumors arising from the pituitary stalk or gland. Two subtypes could be distinguished: an adamatinomatous form that is more common in children, and a papillary form that is observed almost exclusively in adults. Besides these histological differences, these two variants differ in some molecular features that have been recently identified and could have important therapeutic implications. Despite its histologically benign nature, the morbidities related to the tumor itself or its treatment raise many concerns and excess mortality rates up to nine times higher than in the general population has been reported. Among the potential sequelae of craniopharyngiomas, obesity seems the most frequent. The difficulty in the management of this obesity lies in its complex underlying pathophysiological mechanisms. Complete resection of localized tumors should be attempted while a limited hypothalamus-sparing surgery followed or not by radiotherapy should be adopted in tumors involving the hypothalamus. A multidisciplinary approach including, in particular, a dedicated neurosurgeon, and a therapeutic strategy tailored to the individual presentation of the craniopharyngioma in any patient should be initiated at diagnosis for improving the prognosis of these tumors.

KEYWORDS:

Craniopharyngioma; Craniopharyngiome; Obesity; Obésité; Pathogenesis; Pathogénie; Therapeutics; Thérapeutique

PMID:
25617922
DOI:
10.1016/S0003-4266(14)70026-5
[Indexed for MEDLINE]

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