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Endocrine Abstracts (2016) 41 S7.3 | DOI: 10.1530/endoabs.41.S7.3

Germany.


Childhood-onset craniopharyngiomas are rare intracranial embryonal malformations of the sellar region arising from remnants of Rathke’s pouch that require life-long control and management of the endocrine, ophthalmological and neuropsychological deficits caused by the tumors and their treatment. Craniopharyngiomas show low-grade histological malignancy and frequently affect hypothalamic/pituitary regions and the optic chiasm due to their location. Hypothalamic involvement and/or treatment-related lesions to the above structures result in impaired physical and social functionality that includes severe neuroendocrine sequelae, mainly hypothalamic obesity, with major negative impact on quality of life in surviving patient. Quality of life in craniopharyngioma patients with hypothalamic involvement is impaired by physical fatigue, reduced motivation, dyspnea, diarrhea, and non-optimal psychosocial development.

Hypothalamic obesity in craniopharyngioma patients is associated with a severe increase in BMI during the early post-operative period. During long-term follow-up of >12 years, the degree of obesity is plateauing at high levels. Patients with craniopharyngiomas involving hypothalamic structures showed reduced 10-years overall survival rates, whereas overall and progression-free survival rates are not related to degree of resection. Accordingly, gross-total resection should be avoided in cases of hypothalamic involvement to prevent further hypothalamic damage, exacerbating sequelae.

As surgical expertise has been shown to have major impact on postoperative morbidity, medical societies should establish criteria of adequate professional expertise for the treatment and care of craniopharyngioma patients. Based on these criteria, health authorities should organize the certification of centers of excellence authorized for treatment and care of patients with this childhood craniopharyngioma.

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