Neurosurgery Department, Kasr Al-Ainy School of Medicine, Cairo University, Cairo, Egypt.
Neurosurgery Department, Children's Cancer Hospital Egypt (CCHE, 57357), Cairo, Egypt.
Childs Nerv Syst. 2021 Feb;37(2):391-401. doi: 10.1007/s00381-020-04833-x. Epub 2020 Jul 26.
To report our experience and management strategies during 10 years for 137 childhood craniopharyngiomas treated at a single institution.
Medical records of children with craniopharyngioma treated at Children's Cancer Hospital Egypt (CCHE-57357) from July 2007 to December 2017 were retrospectively reviewed. Beta-catenin as an immunohistochemical marker was assessed also in available specimens.
Our registry included 137 patients. Headache (n = 122), visual failure (n = 118), and hypothyroidism(n = 78) were the most common findings on presentation. Three management protocols were identified; 65 patients were primarily followed up after surgery, 71 patients had radiotherapy after surgery, and one patient underwent surgery for Ommaya insertion with intracystic interferon injection. Overall, gross total resection/near total resection was achieved in 48 cases (35.04%), subtotal resection was achieved in 58 patients (42.33%), 29 (21.16%) had biopsy and Ommaya reservoir, and two patients with calcified lesions had no operations. Fifty-four patients showed recurrence/progression of their lesions. Allover, 5-year progression-free survival (PFS) was 52.3%, while it was 34.49% and 72.25% for the follow-up group and the radiotherapy group, respectively. Beta-catenin mutations were positive in 61/95 patients; 5-year PFS for beta-catenin negative and positive cases was 65.5% and 39.4% respectively (p = 0.087). Mortality was reported in eight patients. Intraoperative endoscopy-assisted assessment was the cornerstone of tailored decision-making.
The concepts of conservative surgery and multimodal management should be applied to reach the perfect balance between the quality of life and the best tumor control rates. Beta-catenin mutations more than 5% are associated with statistically trending aggressive clinical behavior. The CCHE-57357 algorithm of individualized management protocol was presented.
报告我们在一家机构 10 年间治疗 137 例儿童颅咽管瘤的经验和管理策略。
回顾 2007 年 7 月至 2017 年 12 月埃及儿童癌症医院（CCHE-57357）收治的颅咽管瘤患儿的病历。还评估了β-连环蛋白作为免疫组化标志物的情况。
我们的登记处包括 137 名患者。头痛（n=122）、视力障碍（n=118）和甲状腺功能减退症（n=78）是就诊时最常见的表现。确定了三种治疗方案；65 例患者术后主要接受随访，71 例患者术后接受放疗，1 例患者行 Ommaya 插入术并囊内注射干扰素。总体而言，48 例（35.04%）实现了大体全切除/近全切除，58 例（42.33%）实现了次全切除，29 例（21.16%）行活检和 Ommaya 储液器，2 例钙化病变患者未行手术。54 例患者显示病变复发/进展。总的来说，5 年无进展生存率（PFS）为 52.3%，而随访组和放疗组分别为 34.49%和 72.25%。95 例中有 61 例β-连环蛋白突变阳性；β-连环蛋白阴性和阳性病例的 5 年 PFS 分别为 65.5%和 39.4%（p=0.087）。报告了 8 例死亡。术中内镜辅助评估是制定个体化决策的基石。
应应用保守手术和多模式管理的理念，在生活质量和最佳肿瘤控制率之间达到完美平衡。β-连环蛋白突变超过 5%与统计学上呈侵袭性临床行为趋势相关。提出了 CCHE-57357 个体化管理方案的算法。
