Napieralska Aleksandra, Mandera Marek, Sordyl Ryszard, Antosz Aleksandra, Bekman Barbara, Blamek Sławomir
Radiotherapy Department, MSC National Research Institute of Oncology Gliwice Branch, 44-101 Gliwice, Poland.
Department of Pediatric Neurosurgery, Medical University of Silesia, 40-752 Katowice, Poland.
Children (Basel). 2023 Jul 24;10(7):1272. doi: 10.3390/children10071272.
An analysis of patients below 21 years old treated due to craniopharyngioma in the years 1979-2022 was performed with the aim of evaluating the long-term outcome and treatment side-effects. The standard statistical tests were used, and 56 patients with a median age of 11 years were evaluated. Surgery was the primary treatment in 55 patients; however, in only 29 it was the only neurosurgical intervention. Eighteen children were treated with radiotherapy (RTH) in primary treatment. The most common neurosurgical side effects observed were visual and endocrine deficits and obesity, which were diagnosed in 27 (49%), 50 (91%), and 25 (52%) patients, respectively. Complications after RTH were diagnosed in 14 cases (32%). During the median follow-up of 8.4 years (range: 0.4-39.8 years), six patients died and the 5- and 10-year overall survival was 97% and 93%, respectively. Five-year progression-free survival for gross total resection, resection with adjuvant RTH, and non-radical resection alone was 83%, 68%, and 23%, respectively ( = 0.0006). Surgery combined with RTH provides comparable results to gross tumor resection in terms of oncologic outcome in craniopharyngioma patients. Adjuvant irradiation applied in primary or salvage treatment improves disease control. The rate of complications is high irrespective of improved surgical and radiotherapeutic management.
对1979年至2022年期间因颅咽管瘤接受治疗的21岁以下患者进行了分析,目的是评估长期疗效和治疗副作用。使用了标准的统计测试,评估了56名中位年龄为11岁的患者。55名患者的主要治疗方法是手术;然而,其中只有29名患者接受了唯一的神经外科干预。18名儿童在初始治疗中接受了放射治疗(RTH)。观察到的最常见的神经外科副作用是视力和内分泌缺陷以及肥胖,分别在27名(49%)、50名(91%)和25名(52%)患者中被诊断出。RTH后并发症在14例(32%)中被诊断出。在中位随访8.4年(范围:0.4 - 39.8年)期间,6名患者死亡,5年和10年总生存率分别为97%和93%。大体全切除、辅助RTH切除和单纯非根治性切除的5年无进展生存率分别为83%、68%和23%( = 0.0006)。在颅咽管瘤患者的肿瘤学结局方面,手术联合RTH与肿瘤大体切除提供了相当的结果。在初始或挽救治疗中应用辅助放疗可改善疾病控制。无论手术和放疗管理有所改善,并发症发生率仍然很高。