Abstract：
BACKGROUND: Outcome of craniopharyngioma is related to its locoregional extension, which impacts resectability and the risk of surgical complications. To maximize resection and minimize complications, optic tract localization, temporal lobe extension, and hypothalamic involvement are essential factors for surgical management.
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.
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.
摘要：
背景：颅咽管瘤的结局与其局部扩展有关，影响可切除性和手术并发症的风险。为了最大限度地切除和减少并发症，视神经束定位,颞叶伸展和下丘脑受累对手术治疗至关重要.
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。
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。
