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      The Diagnosis and Treatment of Craniopharyngioma



      S. Karger AG

      Craniopharyngioma, Hypothalamus, Pituitary, Neurosurgery, Irradiation

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          Craniopharyngioma (CP) is a rare embryonic malformation of the sellar/parasellar region with a low histological grade. Clinical manifestations are related to hypothalamic/pituitary deficiencies, visual impairment, and increased intracranial pressure. Recent insight into the molecular pathogenesis of CP opens new perspectives on targeted therapy in papillary CP harboring BRAF-V600E mutations. Further research to elucidate pathogenic mechanisms and hopefully prevent hypothalamic involvement of CP is warranted. If the tumor is favorably localized, the therapy of choice is complete resection, with care taken to preserve the optical and hypothalamic functions. In patients with unfavorable tumor localization (i.e., hypothalamic involvement), the recommended therapy is a limited hypothalamus-sparing surgical strategy followed by local irradiation. Surgical treatment strategies should be based on a multidisciplinary approach involving experienced teams. Centralizing the treatment of CP in experienced “centers of excellence” and multicenter-based networks for reference assessments should be considered to assure a high standard of treatment quality. CP recurrence and progression are frequent. Irradiation has proven effective in reducing recurrences and progression. Proton beam therapy, available in a wider range in the near future, will help to avoid radio-oncological side effects. Anatomical involvement and/or surgical lesions of posterior hypothalamic areas can result in serious sequelae that compromise quality of life (QoL), such as hypothalamic obesity and psychopathological symptoms. Novel insights into neuropsychological sequelae after CP occurrence should be the basis for the development of therapeutic neuropsychological interventions. CP should be managed as a frequently chronic disease, providing ongoing care of pediatric and adult patients’ clinical and QoL consequences by experienced multidisciplinary teams.

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          Most cited references 68

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          Pituitary magnetic resonance imaging for sellar and parasellar masses: ten-year experience in 2598 patients.

          Sellar and parasellar masses present with overlapping clinical and radiological features ranging from asymptomatic incidental presentations and hormonal effects to compressive local mass effects. Pituitary masses are diagnosed with increased frequency with magnetic resonance imaging (MRI) advancements and availability, but indications and diagnostic outcomes of MRI screening for sellar lesions are not defined. Although pituitary adenomas are the most frequently encountered sellar mass lesions, other etiologies should be considered in the differential diagnosis of a sellar mass.
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            MAPK pathway activation in the embryonic pituitary results in stem cell compartment expansion, differentiation defects and provides insights into the pathogenesis of papillary craniopharyngioma.

            Despite the importance of the RAS-RAF-MAPK pathway in normal physiology and disease of numerous organs, its role during pituitary development and tumourigenesis remains largely unknown. Here we show that the over-activation of the MAPK pathway, through conditional expression of the gain-of-function alleles BrafV600E and KrasG12D in the developing mouse pituitary, results in severe hyperplasia and abnormal morphogenesis of the gland by the end of gestation. Cell-lineage commitment and terminal differentiation are disrupted, leading to a significant reduction in numbers of most of the hormone-producing cells before birth, with the exception of corticotrophs. Of note, Sox2+ve stem cells and clonogenic potential are drastically increased in the mutant pituitaries. Finally, we reveal that papillary craniopharyngioma (PCP), a benign human pituitary tumour harbouring BRAF p.V600E also contains Sox2+ve cells with sustained proliferative capacity and disrupted pituitary differentiation. Together, our data demonstrate a critical function of the MAPK pathway in controlling the balance between proliferation and differentiation of Sox2+ve cells and suggest that persistent proliferative capacity of Sox2+ve cells may underlie the pathogenesis of PCP.
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              Outcome of craniopharyngioma in children: long-term complications and quality of life


                Author and article information

                S. Karger AG
                August 2020
                04 November 2019
                : 110
                : 9-10
                : 753-766
                Department of Pediatrics and Pediatric Hematology/Oncology, University Children’s Hospital, Klinikum Oldenburg AöR, Oldenburg, Germany
                Author notes
                *Hermann L. Müller, MD, Department of Pediatrics and Pediatric Hematology/Oncology, University Children’s Hospital, Klinikum Oldenburg AöR, Rahel-Straus-Strasse 10, DE–26133 Oldenburg (Germany), E-Mail mueller.hermann@klinikum-oldenburg.de
                504512 Neuroendocrinology 2020;110:753–766
                © 2019 S. Karger AG, Basel

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                Page count
                Figures: 4, Tables: 3, Pages: 14
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