ESPE Abstracts (2019) 92 ERN1.1

Life Long Management of Childhood Craniopharyngioma

Hermann L. Müller


Dep. of Pediatrics and Pediatric Hematology/Oncology, University Childrens Hospital, Klinikum Oldenburg, Oldenburg, Germany


Craniopharyngiomas (CP) are rare, partly cystic and calcified embryonic malformations of the sellar/parasellar region with low histological grade (WHO Io). A bimodal age distribution has been shown, with peak incidence rates in childhood-onset at 5–14 yr and adult-onset CP at 50–74 yr. Clinical manifestations are related to hypothalamic/pituitary deficiencies, visual impairment, and increased intracranial pressure. Recent insight in molecular pathogenesis of CP opens new perspectives on targeted therapy. Further research to elucidate pathogenic mechanisms and hopefully prevent hypothalamic involvement of CP is warranted. If the tumor is favorably localized, therapy of choice is complete resection, with care taken to preserve optical and hypothalamic functions. In patients with unfavorable tumor localization (i.e., hypothalamic involvement), recommended therapy is limited hypothalamus-sparing surgical strategy followed by local irradiation. Surgical treatment strategies should be based on a multidisciplinary approach involving experienced teams. Centralizing treatment of CP in experienced "centres of excellence" is recommended. However, such centralization includes high thresholds concerning infrastructure not achievable in all health systems. Alternatives such as multicenter-based networks for reference assessments should be considered to assure high standards of treatment quality. Even though overall survival rates are high (92%), recurrences and progressions are frequent. Irradiation has proven effective in reducing recurrences and progression. Proton beam therapy – available on a wider range in the near future – will help to avoid radiooncological side effects. Anatomical involvement and/or surgical lesions of posterior hypothalamic areas can result in serious quality of life compromising sequelae such as hypothalamic obesity, psychopathological symptoms, and/or cognitive problems. Novel insights into neuropsychological sequelae after CP should be the basis for the development of future therapeutic neuropsychological interventions. It is crucial that CP should be managed as a frequently chronic disease, providing ongoing care of pediatric and adult patients' clinical and quality of life consequences by experienced multidisciplinary teams.

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