Pediatric Neurosurgery, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy.
Università Cattolica del Sacro Cuore-Rome, Rome, Italy.
Adv Tech Stand Neurosurg. 2022;45:139-176. doi: 10.1007/978-3-030-99166-1_4.
In spite of the significant technical and technological progress in neurosurgery and the continuous discoveries by the basic research, adamantinomatous craniopharyngioma remains a significant clinical challenge. Actually, the huge size of the tumor, its multiple cystic components, the encasement of Willis' circle and optic pathways, and the invasion of the hypothalamus often prevent its safe surgical resection. Moreover, the local aggressiveness of the tumor accounts for a high risk of recurrence even after a gross total resection. For these reasons, more and more efforts are being dedicated to enhance the knowledge about AC and improve the tools for its treatment.This paper is dedicated to the most recent advances concerning the AC management. Promising, new insights come for the basic research, thanks to the updates on the role of the WNT-β-catenin pathway (important for the tumor genesis and progression, not yet developed enough for a safe target therapy in children but useful for determining the prognosis) and the inflammatory mediators (widely overexpressed, especially by the cyst of the tumor, and for which target therapies are being developed). Moreover, further factors and pathways are under investigation.Also the development of new treatment strategies accounts for the improvement of the prognosis and the quality of life of AC patients. The enhancement of the experience with the endoscopic techniques (both transsphenoidal and transventricular approaches) actually allows to perform a less invasive but effective surgery that can be coupled with new modalities of radiation therapy aiming at obtaining a reliable control of the disease and protecting the endocrinological, ophthalmological, and neurological functions. A special mention is finally deserved by the techniques specifically designed for the intracystic therapy (as cyst fenestration alone or in combination with administration of radionuclides or bleomycin or interferon-α) that are here analyzed together with the aforementioned advances.
尽管神经外科在技术和技术方面取得了重大进展，基础研究也不断有新发现，但造釉细胞瘤仍然是一个重大的临床挑战。实际上，肿瘤的巨大体积、其多个囊性成分、Willis 环和视路的包绕以及下丘脑的侵犯常常妨碍其安全的手术切除。此外，即使在大体全切除后，肿瘤的局部侵袭性也会导致很高的复发风险。出于这些原因，人们越来越致力于增强对 AC 的认识并改进其治疗方法。本文致力于 AC 管理的最新进展。由于 WNT-β-catenin 通路（对肿瘤发生和进展很重要，尚未在儿童中发展为安全的靶向治疗，但对确定预后有用）和炎症介质（广泛过表达，尤其是肿瘤的囊）的作用，基础研究带来了有希望的新见解，针对这些介质的靶向治疗正在开发中。此外，还有其他因素和途径正在研究中。
新的治疗策略的发展也提高了 AC 患者的预后和生活质量。内镜技术（经蝶窦和经脑室途径）经验的提高实际上允许进行侵袭性更小但有效的手术，该手术可与旨在获得可靠疾病控制并保护内分泌、眼科和神经功能的新放射治疗方式相结合。最后，值得特别提及的是专门为囊内治疗设计的技术（如单纯囊腔开窗或联合放射性核素、博来霉素或干扰素-α给药），本文将与上述进展一起对其进行分析。
