Federal State Autonomous Institution (N. N. Burdenko National Medical Research Center of Neurosurgery) of the Ministry of Health of the Russian Federation, Moscow, Russia.
Moscow Gamma-Knife Center, Moscow, Russia.
Adv Tech Stand Neurosurg. 2022;45:97-137. doi: 10.1007/978-3-030-99166-1_3.
Taking into account the benign nature of craniopharyngiomas, the main method of treatment is the resection of the tumor. However, the tendency of these tumors to invade critical structures (such as optic pathways, the hypothalamic-pituitary system, the Willis circle vessels) often limits the possibility of a radical surgery.Craniopharyngiomas of the third ventricle represent the greatest challenge for surgery. After radical surgery, hypothalamic disorders often occur, including not only obesity but also cognitive, emotional, mental, and metabolic disturbances. Metabolic disorders associated with damage to the hypothalamus progress after surgery and lead to impaired functions of the internal organs. This process is irreversible and, in many cases, becomes the direct cause of mortality. The life expectancy of patients with the surgically affected hypothalamus is significantly shorter than in patients with preserved diencephalic function. The incidence of hypothalamic disorders after surgery can reach 40%.Even with macroscopically total resection, craniopharyngiomas can recur in 10-30% of cases, and in the presence of tumor remnants and with no further radiation treatment, the risk of recurrence significantly increases to up to 50-85% according to various studies. For this reason, the observation of patients with residual tumors after surgery is an incorrect strategy.Radiation therapy significantly improves progression-free survival (PFS), and the use of stereotactic irradiation techniques ensures conformity of irradiation of tumor remnants with a complicated shape and location (Iwata H et al., J Neurooncol 106(3):571-577, 2012; Aggarwal et al., Pituitary 16(1):26-33, 2013; Savateev et al., Zh Vopr Neirokhir Im N N Burdenko 81(3):94-106; 2017), which potentially reduces the risk of undesirable postradiation effects. Therefore, the quality of life in patients with craniopharyngiomas infiltrating the hypothalamus is significantly higher after non-radical operations with subsequent stereotactic radiation than after a total or subtotal removal.
考虑到颅咽管瘤的良性性质，主要的治疗方法是肿瘤切除术。然而，这些肿瘤侵袭关键结构的倾向（如视路、下丘脑-垂体系统、Willis 环血管）常常限制了根治性手术的可能性。第三脑室颅咽管瘤对手术来说是最大的挑战。根治性手术后，下丘脑功能障碍常发生，不仅有肥胖，还有认知、情绪、精神和代谢紊乱。与下丘脑损伤相关的代谢紊乱在手术后进展，导致内脏器官功能受损。这个过程是不可逆转的，在许多情况下，成为死亡的直接原因。接受下丘脑受影响手术的患者的预期寿命明显短于保留间脑功能的患者。手术后下丘脑疾病的发生率可达 40%。即使进行了大体全切除，颅咽管瘤在 10-30%的病例中仍可复发，在肿瘤残留且无进一步放疗的情况下，根据各种研究，复发风险显著增加至 50-85%。因此，对手术后有残留肿瘤的患者进行观察是一种不正确的策略。放疗显著改善无进展生存期（PFS），立体定向放疗技术的应用确保了复杂形状和位置的肿瘤残留的照射一致性（Iwata H 等人，J Neurooncol 106(3):571-577, 2012; Aggarwal 等人，Pituitary 16(1):26-33, 2013; Savateev 等人，Zh Vopr Neirokhir Im N N Burdenko 81(3):94-106; 2017），这潜在地降低了不良放疗后效应的风险。因此，对于侵袭下丘脑的颅咽管瘤患者，非根治性手术联合立体定向放疗后的生活质量明显高于完全或次全切除后的生活质量。
