Hermann L Müller1. 1. Department of Pediatrics, Klinikum Oldenburg, Rahel-Straus-Strasse 10, 26133 Oldenburg, Germany. mueller.hermann@klinikum-oldenburg.de
Abstract
CONTEXT: Childhood craniopharyngioma, a rare embryogenic tumorous malformation of the sellar region, is characterized by survival rates ranging from 91 to 98%. However, quality of survival is frequently impaired due to proximity to optical, pituitary, and hypothalamic structures. Long-term sequelae substantially reduce the quality of life of approximately 50% of long-term survivors, notably extreme obesity owing to hypothalamic involvement and/or surgical- or radiation-induced lesions. EVIDENCE ACQUISITION AND SYNTHESIS: This report reviews the current understanding of diagnostic and treatment options and their consequences on the prognosis and quality of life in patients with childhood craniopharyngioma based on publications from PubMed, Science Citation Index Expanded, EMBASE, and Scopus from the year 1980 onward. CONCLUSIONS: Total resection is the treatment of choice in patients with favorable tumor localization, with extreme care taken to preserve hypothalamic-pituitary and optical nerve functions. When tumor localization is unfavorable, i.e. involvement of hypothalamic or optic structures, a limited resection followed by local irradiation is recommended. Optimal timing of recurrence-inhibiting irradiation after incomplete resection is currently under investigation in an international trial. The rarity of the disease, coupled with limited surgical options, dictates that treatment and long-term monitoring of consequences should be confined to experienced multidisciplinary teams.
CONTEXT: Childhood craniopharyngioma, a rare embryogenic tumorous malformation of the sellar region, is characterized by survival rates ranging from 91 to 98%. However, quality of survival is frequently impaired due to proximity to optical, pituitary, and hypothalamic structures. Long-term sequelae substantially reduce the quality of life of approximately 50% of long-term survivors, notably extreme obesity owing to hypothalamic involvement and/or surgical- or radiation-induced lesions. EVIDENCE ACQUISITION AND SYNTHESIS: This report reviews the current understanding of diagnostic and treatment options and their consequences on the prognosis and quality of life in patients with childhood craniopharyngioma based on publications from PubMed, Science Citation Index Expanded, EMBASE, and Scopus from the year 1980 onward. CONCLUSIONS: Total resection is the treatment of choice in patients with favorable tumor localization, with extreme care taken to preserve hypothalamic-pituitary and optical nerve functions. When tumor localization is unfavorable, i.e. involvement of hypothalamic or optic structures, a limited resection followed by local irradiation is recommended. Optimal timing of recurrence-inhibiting irradiation after incomplete resection is currently under investigation in an international trial. The rarity of the disease, coupled with limited surgical options, dictates that treatment and long-term monitoring of consequences should be confined to experienced multidisciplinary teams.
Authors: Anthe S Sterkenburg; Anika Hoffmann; Ursel Gebhardt; Monika Warmuth-Metz; Anna M M Daubenbüchel; Hermann L Müller Journal: Neuro Oncol Date: 2015-04-02 Impact factor: 12.300
Authors: Chiaho Hua; Barry L Shulkin; Daniel J Indelicato; Yimei Li; Xingyu Li; Frederick A Boop; Thomas E Merchant Journal: J Neurosurg Pediatr Date: 2015-08-21 Impact factor: 2.375
Authors: Anna M M Daubenbüchel; Anika Hoffmann; Maria Eveslage; Jale Özyurt; Kristin Lohle; Julia Reichel; Christiane M Thiel; Henri Martens; Vincent Geenen; Hermann L Müller Journal: Endocrine Date: 2016-09-01 Impact factor: 3.633
Authors: Alessia Pica; Sarah Abbeel; Nicolas Von der Weid; Ali Sajadi; Laura Negretti; Franziska Phan-Hug; Michael Hauschild; Daniel Schmidhalter; Valerie Schwitzgebel; Damien Weber Journal: J Radiosurg SBRT Date: 2013