儿童颅咽管瘤的治疗方法及其疗效现状分析

Analysis of Treatment Methods and Curative Effect of Craniopharyngioma in Children

摘要: 目的：通过文献复习归纳儿童颅咽管瘤的不同治疗方法及其疗效，寻求目前最佳的治疗选择，探索未来儿童颅咽管瘤治疗方法研究的方向。方法：阅读近5年国内外具有代表性的颅咽管瘤文献，归纳颅咽管瘤分型及其对儿童的适用性和对手术入路的指导意义；分类整理儿童颅咽管瘤现阶段的治疗方法及疗效，分析得出现阶段最佳治疗方案和未来治疗可能研究的方向。结果：外科手术治疗仍为现阶段儿童颅咽管瘤主流的治疗方法，颅咽管瘤分型能协助指导手术评估；显微手术创伤性大、术后恢复周期长、治疗相关性下丘脑及垂体功能障碍发生率高，影响患儿生活质量、生长发育；近年经鼻内镜手术备受推崇，镜下直视操作、术野清晰、下丘脑干扰小，中线中央区域尤为适用；调强适形放疗、伽玛刀能有效控制原发和复发颅咽管瘤肿瘤细胞增长，延长手术介入的时限，但放射性组织粘连增加完全切除难度，同时会产生放射性损伤，质子治疗能规避/减小放射性副损伤，但目前处于研究阶段；放射性同位素间质内放疗为儿童囊性颅咽管瘤理想的治疗选择，但肿瘤控制率有限，复发率高，存在泄露导致严重并发症的风险，理论和实际相差甚远；干扰素毒副反应小，限于数据量小，有效性、安全性尚待进一步研究；基因水平研究的深入，提升了对颅咽管瘤发病机制分子水平的认识，为分子靶向治疗提供坚实的理论支撑，现有报道已证实靶向药物的有效性。结论与展望：颅咽管瘤分型指导下外科手术仍为现阶段儿童颅咽管瘤主要的治疗方法，放射能协助控制术后残留病灶和复发肿瘤；囊性颅咽管瘤间质内放疗和药物化疗制剂的选择及潜在的安全性、有效性尚待进一步前瞻性研究；儿童颅咽管瘤质子刀治疗前景可观；靶向治疗初现成效，分子致病机制的深入探索将为儿童颅咽管瘤开起新的治疗选择。

Abstract: Objective: To summarize the different treatment methods of children’s craniopharyngioma and their efficacy through literature review, seek the best treatment options at present, and explore the research directions of craniopharyngioma treatment methods for children in the future. Methods: Read the representative craniopharyngioma literature at home and abroad in the past 5 years, summarize the craniopharyngioma classification and its applicability to children and the guiding significance of surgical approach; classify and sort out the current treatment methods and curative effects of craniopharyngioma in children, and analyze the best treatment options and possible future research directions. Results: Surgical treatment is still the mainstream treatment method for craniopharyngioma in children. The craniopharyngioma’s classification can help guide surgical evaluation; microsurgical trauma, long postoperative recovery period, high incidence of treaty-related hypothalamus and pituitary dysfunction, affect the quality of life, growth and de-velopment of children. In recent years, endoscopic endonasal surgery, with the advantages of di-rect-view operation, clear surgical field, and low hypothalamus interference, has been highly re-spected, especially suitable for midline central tumors; IMRT and gamma knife can effectively control the recurrent craniopharyngioma tumor cells growth and prolong the duration of surgical intervention. However, the adhesion of radioactive tissue increases the difficulty of gross total re-section, and radioactive damage will occur. Proton therapy can avoid/reduce the radioactive side effects, but it is currently in the research stage. Radioisotope interstitial radiotherapy is an ideal treatment option for cystic craniopharyngioma, but the rate of tumor control is limited, meanwhile with a high rate of recurrence and serious complications caused by leakage. The side effect of toxicity of interferon is small, but the effectiveness and safety need to be further studied due to the limited data. Genetic research is gradually deepening, which promotes us to understand the molecular pathogenesis of craniopharyngioma and provides a solid theoretical support for molec-ular targeted therapy. And case reports have confirmed the effectiveness of targeted drugs. Con-clusions and Outlook: Surgery based on the guidance of craniopharyngioma classification is still the main treatment method for children’s craniopharyngioma. And radiation can effectively help control residual lesions and recurrence tumors after surgery. Potential safety and efficacy of cystic craniopharyngioma interstitial radiotherapy and drug therapy need further prospective research. Proton therapy for children craniopharyngioma has promising prospects. Targeted drug therapy has shown initial efficacy, and in-depth study of molecular mechanisms will provide new treatment options for children craniopharyngioma.