OBJECTIVE: To assess the outcome of craniopharyngiomas depending on their relation to the hypothalamus location.
METHODS: We conducted a retrospective analysis of 79 patients with a craniopharyngioma who underwent surgery from 2007 to 2022. Craniopharyngiomas were classified in 3 groups, depending on the type of hypothalamus involvement assessed by preoperative magnetic resonance imaging: infra-hypothalamic (type A, n = 33); perforating the hypothalamus (type B, n = 40); and supra-hypothalamic (type C, n = 6). Surgical strategy was guided by the type of hypothalamic involvement, favoring endonasal approaches for type A and type B, and transcranial approaches for type C.
RESULTS: Long-term disease control was achieved in 33/33 (100%), 37/40 (92%), and 5/6 (83%) patients in type A, B, and C, respectively. In type B, vision was improved in 32/36 (89%) patients, while hypothalamic function was improved, stable, or worsened in 6/40 (15%), 32/40 (80%), and 2/40 (5%) patients, respectively. Papillary craniopharyngiomas were found in 5/33 (15%), 9/40 (22%), and 3/6 (50%) patients in types A, B, and C, respectively. In 4 patients, BRAF/MEK inhibitors were used, with significant tumor shrinkage in all cases.
CONCLUSIONS: Craniopharyngiomas located below the hypothalamus or perforating it can be safely treated by transsphenoidal surgery. For supra-hypothalamic craniopharyngiomas, postoperative results are less favorable, and documenting a BRAF mutation may improve outcome, if targeted therapy was efficient enough to replace surgical debulking.
目的:根据颅咽管瘤与下丘脑位置的关系来评估颅咽管瘤的预后。
方法:我们对2007年至2022年接受手术的79例颅咽管瘤患者进行了回顾性分析。颅咽管瘤分为三组,根据术前MRI评估的下丘脑受累类型:下丘脑下(A型,n=33);下丘脑穿孔(B型,n=40);下丘脑上(C型,n=6)。手术策略以下丘脑受累的类型为指导,有利于A型和B型的鼻内途径,和经颅方法治疗C型。
结果:在33/33(100%)中实现了长期疾病控制,37/40(92%)和5/6(83%)的A型患者,分别为B和C。在B型中,32/36(89%)患者的视力得到改善,下丘脑功能得到改善,在6/40(15%)稳定或恶化,32/40(80%)和2/40(5%)患者。乳头状颅咽管瘤见于5/33(15%),A型9/40(22%)和3/6(50%)患者,分别为B和C。在四名患者中,使用BRAF/MEK抑制剂,在所有病例中都有明显的肿瘤缩小。
结论:位于下丘脑以下或穿孔的颅咽管瘤可以通过经蝶窦手术安全治疗。对于下丘脑上颅咽管瘤,术后效果较差,记录BRAF突变可能会改善结果,如果靶向治疗足够有效,可以取代手术切除。