Preoperative staging in childhood craniopharyngioma: standardization as a first step towards improved outcome
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EDITORIAL
Preoperative staging in childhood craniopharyngioma: standardization as a first step towards improved outcome Hermann L. Mu¨ller1
Received: 23 September 2015 / Accepted: 4 November 2015 Ó Springer Science+Business Media New York 2015
Introduction Childhood-onset craniopharyngiomas (CP) are classified by WHO as brain tumors of low-grade histological malignancy. During the last decades, the prognosis of pediatric patients with low-grade brain tumors of other histology has been continuously und substantially improved by implementation of standardized treatment protocols used in national and international trials. A mainstay of this success—illustrated by improved survival and outcome—has been the standardized staging at primary diagnosis. However, in CP such national and international efforts on standardization of preoperative staging and treatment strategies are still missing. CPs are rare embryonal malformations. Hypothalamic involvement and/or treatment-related lesions result in impaired physical and social functionality and severe neuroendocrine sequelae [1]. Quality of life in CP with hypothalamic involvement is impaired by severe obesity, physical fatigue, reduced motivation, dyspnea, diarrhea, and non-optimal psychosocial development [2]. Patients with CP involving hypothalamic structures showed reduced 10-years overall survival, whereas overall and progressionfree survival rates are not related to the degree of surgical resection [2]. However, these results are based on a multicenter study that spans nearly five decades during which dramatic changes in surgical and radiation therapy
& Hermann L. Mu¨ller [email protected]; http://www.kraniopharyngeom.net 1
Department of Pediatrics, Klinikum Oldenburg, Medical Campus University Oldenburg, Rahel-Straus-Strasse 10, 26133 Oldenburg, Germany
occurred. In fact, there was no uniform treatment paradigm and no assessment of relative surgical (or radiation oncology) experience, which has been reported as a critical factor for long-term outcome [3–5]. Furthermore, contemporary series show relatively equivalent 5- and 10-year progression-free survival rates associated with radical surgery or partial resection combined with irradiation. Information on the impact of salvage therapies on survival and quality of life in the 20–30 % of patients with recurrent disease is limited. Several grading systems for assessment of pre and/or postoperative location and hypothalamic involvement of CP and algorithms for specific treatment strategies based on these grading systems have been published [4–11]. Mortini et al. [12] are publishing an analysis of their single center cohort of exclusively adult-onset CPs comparing three of the above-mentioned grading systems [4–6, 9] in terms of prediction value for outcome and the development of hypothalamic syndrome.
Grading systems for hypothalamic involvement/ lesions in CP Novel risk-adapted treatment strategies are reportedly focusing on the following main goals: (a) relief of raised intracranial pressure, (
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