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Treatment strategies in childhood craniopharyngioma.

Puget S - Front Endocrinol (Lausanne) (2012)

Bottom Line: With this in mind, risk-adapted strategies designed to preserve hypothalamic structures have been developed.The preliminary results of these strategies appear to be encouraging.However, the long-term clinical outcome in terms of post irradiation complications and management of relapses is currently unknown.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatric Neurosurgery, Necker Hospital, Université Paris Descartes Paris, France.

ABSTRACT
The surgical management of craniopharyngiomas in children remains one of the more controversial topics in pediatric neurosurgery. Theoretically, the benign histology implies that total surgical excision would be sufficient to provide a cure. It has been widely established however, that in certain cases total excision may lead to unacceptable hypothalamic injury. The therapeutic goals for pediatric craniopharyngiomas therefore, require not just cure of the disease but also preservation of function. Over the last 15 years, there has been a growing worldwide advocacy for less extensive resection and for the utilization of multimodality therapy to limit morbidity. With this in mind, risk-adapted strategies designed to preserve hypothalamic structures have been developed. The preliminary results of these strategies appear to be encouraging. However, the long-term clinical outcome in terms of post irradiation complications and management of relapses is currently unknown.

No MeSH data available.


Related in: MedlinePlus

Pre-operative MRI classification according to hypothalamic involvement. Type 0 pre-op: no involvement of the hypothalamus, Type 1 pre-op: distortion/elevation of the hypothalamus, Type 2 pre-op: the hypothalamus is not visible due to tumor invasion.
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Figure 1: Pre-operative MRI classification according to hypothalamic involvement. Type 0 pre-op: no involvement of the hypothalamus, Type 1 pre-op: distortion/elevation of the hypothalamus, Type 2 pre-op: the hypothalamus is not visible due to tumor invasion.

Mentions: In an attempt to analyze the role of aggressive surgical resection relative to the risk of significant morbidity associated with this approach, the authors critically reviewed a retrospective series where there had been an intention of gross total resection in all cases (Puget et al., 2007). Classification of tumors at presentation was performed in order to rationalize multimodality therapy. Specifically, the pre- and post-operative MRI were graded with respect to the degree of hypothalamic involvement/injury (Figures 1 and 2). As previously shown (De Vile et al., 1996a), it was confirmed that quality of life outcomes (using the Health Utility Index 2, HUI2) were correlated with the degree of hypothalamic injury as evident on the post-operative MRI (p = 0.003). The post-operative BMI and quality of life were linked to hypothalamic involvement as assessed on the pre-operative MRI (p = 0.007 and p = 0.001 for BMI Z score and HUI2 score respectively). This finding has subsequently been confirmed in a large, multicentre prospective study where the only independent risk factor for severe obesity, on multivariate analysis, was the degree of pre-operative hypothalamic involvement (p = 0.002; Muller et al., 2011) Using the MRI grading scheme described above (Puget et al., 2007). Van Gompel et al. (2010) in a large cohort of 296 adult patients, showed a good correlation between the degree of pre-operative hypothalamic involvement and post-operative weight gain (p = 0.022).


Treatment strategies in childhood craniopharyngioma.

Puget S - Front Endocrinol (Lausanne) (2012)

Pre-operative MRI classification according to hypothalamic involvement. Type 0 pre-op: no involvement of the hypothalamus, Type 1 pre-op: distortion/elevation of the hypothalamus, Type 2 pre-op: the hypothalamus is not visible due to tumor invasion.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3367313&req=5

Figure 1: Pre-operative MRI classification according to hypothalamic involvement. Type 0 pre-op: no involvement of the hypothalamus, Type 1 pre-op: distortion/elevation of the hypothalamus, Type 2 pre-op: the hypothalamus is not visible due to tumor invasion.
Mentions: In an attempt to analyze the role of aggressive surgical resection relative to the risk of significant morbidity associated with this approach, the authors critically reviewed a retrospective series where there had been an intention of gross total resection in all cases (Puget et al., 2007). Classification of tumors at presentation was performed in order to rationalize multimodality therapy. Specifically, the pre- and post-operative MRI were graded with respect to the degree of hypothalamic involvement/injury (Figures 1 and 2). As previously shown (De Vile et al., 1996a), it was confirmed that quality of life outcomes (using the Health Utility Index 2, HUI2) were correlated with the degree of hypothalamic injury as evident on the post-operative MRI (p = 0.003). The post-operative BMI and quality of life were linked to hypothalamic involvement as assessed on the pre-operative MRI (p = 0.007 and p = 0.001 for BMI Z score and HUI2 score respectively). This finding has subsequently been confirmed in a large, multicentre prospective study where the only independent risk factor for severe obesity, on multivariate analysis, was the degree of pre-operative hypothalamic involvement (p = 0.002; Muller et al., 2011) Using the MRI grading scheme described above (Puget et al., 2007). Van Gompel et al. (2010) in a large cohort of 296 adult patients, showed a good correlation between the degree of pre-operative hypothalamic involvement and post-operative weight gain (p = 0.022).

Bottom Line: With this in mind, risk-adapted strategies designed to preserve hypothalamic structures have been developed.The preliminary results of these strategies appear to be encouraging.However, the long-term clinical outcome in terms of post irradiation complications and management of relapses is currently unknown.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatric Neurosurgery, Necker Hospital, Université Paris Descartes Paris, France.

ABSTRACT
The surgical management of craniopharyngiomas in children remains one of the more controversial topics in pediatric neurosurgery. Theoretically, the benign histology implies that total surgical excision would be sufficient to provide a cure. It has been widely established however, that in certain cases total excision may lead to unacceptable hypothalamic injury. The therapeutic goals for pediatric craniopharyngiomas therefore, require not just cure of the disease but also preservation of function. Over the last 15 years, there has been a growing worldwide advocacy for less extensive resection and for the utilization of multimodality therapy to limit morbidity. With this in mind, risk-adapted strategies designed to preserve hypothalamic structures have been developed. The preliminary results of these strategies appear to be encouraging. However, the long-term clinical outcome in terms of post irradiation complications and management of relapses is currently unknown.

No MeSH data available.


Related in: MedlinePlus