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      Advances in the Management of Craniopharyngioma in Children and Adults

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          Abstract

          Background

          Childhood and adult-onset craniopharyngioma is a rare embryogenic tumor of the sellar, suprasellar, and parasellar region. Survival rates are high; however, tumor location and treatment sequalae including endocrine deficits, visual impairment, metabolic complications, cognitive and psychosocial deficits can significantly impair patient’s quality of life. There is considerable controversy regarding the optimal management of craniopharyngiomas. Subtotal resection of the tumor followed by targeted irradiation to avoid further hypothalamic damage is currently indicated. Novel insights in the tumor’s molecular pathology present the possibility for targeted therapy possibly decreasing the rate and severity of treatment-associated morbidity.

          Conclusions

          Craniopharyngioma should be seen as a chronic disease. To achieve optimal outcomes a multidisciplinary team of specialized neurosurgeons, neuro-radiologists, neuro-oncologists, pathologists and endocrinologists should be involved in the diagnosis, planning of the surgery, irradiation and long-term follow-up.

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          Most cited references47

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          CBTRUS Statistical Report: Primary brain and other central nervous system tumors diagnosed in the United States in 2010-2014.

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            Association between premature mortality and hypopituitarism

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              Premature mortality due to cardiovascular disease in hypopituitarism

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                Author and article information

                Journal
                Radiol Oncol
                Radiol Oncol
                raon
                raon
                Radiology and Oncology
                Sciendo
                1318-2099
                1581-3207
                December 2019
                25 October 2019
                : 53
                : 4
                : 388-396
                Affiliations
                [1 ]Department of Endocrinology, Diabetes and Metabolism, University Medical Centre Ljubljana , Ljubljana, Slovenia
                [2 ]Medical Faculty, University of Ljubljana , Ljubljana, Slovenia
                [3 ]Department of Endocrinology, Diabetes and Metabolism, University Children’s Hospital, University Medical Centre Ljubljana , Ljubljana, Slovenia
                [4 ]Department of Neurosurgery, University Medical Centre Ljubljana , Ljubljana, Slovenia
                [5 ]Institute of Pathology, Medical Faculty, University of Ljubljana , Ljubljana, Slovenia
                [6 ]Department of Radiotherapy, Institute of Oncology Ljubljana , Ljubljana, Slovenia
                [7 ]Clinical Institute of Radiology, University Medical Centre Ljubljana , Ljubljana, Slovenia
                [8 ]Department of Hematology and Oncology, University Children’s Hospital, University Medical Centre Ljubljana , Ljubljana, Slovenia
                Author notes
                [* ] Asst. Prof., Primož Kotnik, M.D., Ph.D., Department of Endocrinology, Diabetes and Metabolism, University Children’s Hospital, University Medical Center Ljubljana, Bohoričeva 20, SI-1000 Ljubljana, Slovenia. Department of Pediatrics, Faculty of Medicine, University of Ljubljana, Bohoričeva 20, SI-1000 Ljubljana, Slovenia. primoz.kotnik@ 123456mf.uni-lj.si
                Article
                raon-2019-0036
                10.2478/raon-2019-0036
                6884937
                31652121
                7a70cbba-6cdc-4ca9-b6c3-703653f41aba
                © 2019 Mojca Jensterle, Soncka Jazbinsek, Roman Bosnjak, Mara Popovic, Lorna Zadravec Zaletel, Tina Vipotnik Vesnaver, Barbara Faganel Kotnik, Primoz Kotnik, published by Sciendo

                This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

                History
                : 21 June 2019
                : 11 July 2019
                Page count
                Pages: 9
                Categories
                Review

                Oncology & Radiotherapy
                craniopharyngioma,hypopituitarism,metabolic syndrome,proton beam therapy,ctnnb1 gene,mapk/erk pathway

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