Clinical situation, therapy, and follow up of adult craniopharyngioma - report from the German craniopharyngioma registry

• Published in: The journal of clinical endocrinology and metabolism 2020-01, Vol.105 (1), p.252-265
• Main Authors: Mende, Klaus Christian, Kellner, Teresa, Petersenn, Stephan, Honegger, Juergen, Evangelista-Zamora, Rocío, Droste, Michael, Stalla, Guenter, Deutschbein, Timo, Wang, Yawen, Moskopp, Dag, Knappe, Ulrich, Schilbach, Katharina, Flitsch, Joerg
• Format: Article
• Language: English
• Subjects: Body mass index; Bone cancer; Central nervous system; Children; Data collection; Diabetes insipidus; Hypothalamus; Neoplasia; Patients; Pituitary; Quality of life; Surgery; Vision
• ISSN: 0021-972X; 1945-7197
• DOI: 10.1210/clinem/dgz043

Context Craniopharyngioma is a rare neoplastic entity of the central nervous system. Childhood-onset craniopharyngioma is the subject of frequent research whereas the information on adult-onset craniopharyngioma is scarce. Objective The objective of this study was to examine the level of daily impairment in adult patients suffering from craniopharyngioma. Design Noninterventional patient registry indexed as PV4842 with the local ethics committee. Setting The study is set in a hospitalized and ambulatory setting. Patients 148 patients with adult-onset craniopharyngioma were recruited from 8 centers, 22 prospectively and 126 retrospectively. Mean follow-up was 31 months. Interventions No interventions performed. Main Outcome Measures Complications, symptoms, body mass index (BMI), and quality of life (QoL; EORTC QLQ C30 and BN20) were recorded preoperatively and at follow-up. The hypotheses tested were generated after data collection. Results Complications were more frequent after transcranial than transsphenoidal approaches (31 % vs. 11%; P < 0.01). Preoperative obesity was present in 0% papillary and in 38% of all adamantinomatous craniopharyngiomas (P = 0.05), and diabetes insipidus was more frequent for papillary craniopharyngioma (36.8% vs. 16,7%; P < 0.05). Hormone deficits at follow-up were reduced in 16.9%, equal in 31.4%, and increased in 63.6% (P < 0.001). BMI increased from 28.7 ± 7.4 kg/m2 before surgery to 30.2 ± 7.4 kg/m2 at follow-up (P < 0.001). In QoL, a decrease of future uncertainty (62.5 vs. 36.8; P = 0.02) and visual disorders (38.9 vs. 12.0; P = 0.01) were observed in the prospective collective after surgery. Conclusions Adult craniopharyngioma is associated with a complex sociological and psychological burden and hypothalamic dysfunction, warranting further investigation and emphasizing the need for a wider treatment approach.