Gaillard Stephan, Benichi Sandro, Villa Chiara, Jouinot Anne, Vatier Camille, Christin-Maitre Sophie, Raffin-Sanson Marie-Laure, Jacob Julian, Chanson Philippe, Courtillot Carine, Bachelot Anne, Bertherat Jérôme, Assié Guillaume, Baussart Bertrand
Department of Neurosurgery, Assistance Publique-Hôpitaux de Paris, La Pitié-Salpêtrière University Hospital, 75013 Paris, France.
Department of Neurosurgery, Assistance Publique-Hôpitaux de Paris, Necker University Hospital, 75015 Paris, France.
J Clin Endocrinol Metab. 2024 Jul 12;109(8):2083-2096. doi: 10.1210/clinem/dgae049.
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.
To assess the outcome of craniopharyngiomas depending on their relation to the hypothalamus location.
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.
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.
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.
颅咽管瘤的预后与其局部区域扩展有关,这会影响肿瘤的可切除性和手术并发症风险。为了实现最大程度的切除并将并发症降至最低,视束定位、颞叶扩展和下丘脑受累情况是手术治疗的关键因素。
根据颅咽管瘤与下丘脑位置的关系评估其预后。
我们对2007年至2022年期间接受手术的79例颅咽管瘤患者进行了回顾性分析。根据术前磁共振成像评估的下丘脑受累类型,将颅咽管瘤分为3组:下丘脑下方(A 型,n = 33);穿透下丘脑(B 型,n = 40);下丘脑上方(C 型,n = 6)。手术策略以下丘脑受累类型为指导,A 型和 B 型倾向于采用鼻内镜入路,C 型采用经颅入路。
A 型、B 型和 C 型患者分别有33/33(100%)、37/40(92%)和5/6(83%)实现了长期疾病控制。在 B 型中,32/36(89%)的患者视力得到改善,而下丘脑功能改善、稳定或恶化的患者分别为6/40(15%)、32/40(80%)和2/40(5%)。A 型、B 型和 C 型患者中分别有5/33(15%)、9/40(22%)和3/6(50%)发现乳头状颅咽管瘤。4例患者使用了BRAF/MEK抑制剂,所有病例肿瘤均显著缩小。
位于下丘脑下方或穿透下丘脑的颅咽管瘤可通过经蝶窦手术安全治疗。对于下丘脑上方的颅咽管瘤,术后结果较差,如果靶向治疗足够有效以替代手术减瘤,记录BRAF突变可能会改善预后。