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Craniopharyngioma

Craniopharyngiomas are benign slow growing tumours that are located within the sellar and para sellar region of the central nervous system. The point prevalence of this tumour is approximately 2/100,000. The onset of symptoms is normally insidious with most patients at diagnosis having neurological...

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Autores principales: Garnett, Matthew R, Puget, Stéphanie, Grill, Jacques, Sainte-Rose, Christian
Formato: Texto
Lenguaje:English
Publicado: BioMed Central 2007
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1855047/
https://www.ncbi.nlm.nih.gov/pubmed/17425791
http://dx.doi.org/10.1186/1750-1172-2-18
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author Garnett, Matthew R
Puget, Stéphanie
Grill, Jacques
Sainte-Rose, Christian
author_facet Garnett, Matthew R
Puget, Stéphanie
Grill, Jacques
Sainte-Rose, Christian
author_sort Garnett, Matthew R
collection PubMed
description Craniopharyngiomas are benign slow growing tumours that are located within the sellar and para sellar region of the central nervous system. The point prevalence of this tumour is approximately 2/100,000. The onset of symptoms is normally insidious with most patients at diagnosis having neurological (headaches, visual disturbances) and endocrine (growth retardation, delayed puberty) dysfunctions. Craniopharyngiomas are thought to arise from epithelial remnants of the craniopharyngeal duct or Rathke's pouch (adamantinomatous type) or from metaplasia of squamous epithelial cell rests that are remnants of the part of the stomadeum that contributed to the buccal mucosa (squamous papillary type). The neuroradiological diagnosis is mainly based on the three components of the tumour (cystic, solid and calcified) in the characteristic sellar/para sellar location. Definitive diagnosis is made following histological examination of a surgical specimen. The differential diagnosis includes other tumours in this region (pituitary adenoma), infectious or inflammatory processes (eosinophilic granuloma), vascular malformations (aneurysm) and congenital anomalies (Rathke's cleft cyst). The current treatment is gross total excision of the tumour, if there is no hypothalamic invasion or, in the presence of hypothalamic invasion, a sub-total resection with post-operative radiotherapy. Endocrine disturbances are normally permanent and need careful replacement. Overall, there is an 80% 5 year survival, though this can be associated with marked morbidity (hypothalamic dysfunction, altered neuropsychological profile).
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spelling pubmed-18550472007-04-24 Craniopharyngioma Garnett, Matthew R Puget, Stéphanie Grill, Jacques Sainte-Rose, Christian Orphanet J Rare Dis Review Craniopharyngiomas are benign slow growing tumours that are located within the sellar and para sellar region of the central nervous system. The point prevalence of this tumour is approximately 2/100,000. The onset of symptoms is normally insidious with most patients at diagnosis having neurological (headaches, visual disturbances) and endocrine (growth retardation, delayed puberty) dysfunctions. Craniopharyngiomas are thought to arise from epithelial remnants of the craniopharyngeal duct or Rathke's pouch (adamantinomatous type) or from metaplasia of squamous epithelial cell rests that are remnants of the part of the stomadeum that contributed to the buccal mucosa (squamous papillary type). The neuroradiological diagnosis is mainly based on the three components of the tumour (cystic, solid and calcified) in the characteristic sellar/para sellar location. Definitive diagnosis is made following histological examination of a surgical specimen. The differential diagnosis includes other tumours in this region (pituitary adenoma), infectious or inflammatory processes (eosinophilic granuloma), vascular malformations (aneurysm) and congenital anomalies (Rathke's cleft cyst). The current treatment is gross total excision of the tumour, if there is no hypothalamic invasion or, in the presence of hypothalamic invasion, a sub-total resection with post-operative radiotherapy. Endocrine disturbances are normally permanent and need careful replacement. Overall, there is an 80% 5 year survival, though this can be associated with marked morbidity (hypothalamic dysfunction, altered neuropsychological profile). BioMed Central 2007-04-10 /pmc/articles/PMC1855047/ /pubmed/17425791 http://dx.doi.org/10.1186/1750-1172-2-18 Text en Copyright © 2007 Garnett et al; licensee BioMed Central Ltd. http://creativecommons.org/licenses/by/2.0 This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( (http://creativecommons.org/licenses/by/2.0) ), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Review
Garnett, Matthew R
Puget, Stéphanie
Grill, Jacques
Sainte-Rose, Christian
Craniopharyngioma
title Craniopharyngioma
title_full Craniopharyngioma
title_fullStr Craniopharyngioma
title_full_unstemmed Craniopharyngioma
title_short Craniopharyngioma
title_sort craniopharyngioma
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1855047/
https://www.ncbi.nlm.nih.gov/pubmed/17425791
http://dx.doi.org/10.1186/1750-1172-2-18
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