推荐回答(1个)
颅咽管瘤是颅内罕见肿瘤,非常难治疗,难以治疗的原因主要有四个。
一、颅咽管瘤不容易早期发现
颅咽管瘤是罕见的颅内肿瘤,各个年龄段都可以发病,当病人开始有症状的时候常常被忽视,不能及时发现。颅咽管瘤病人常见的症状是
◇视力下降。
学龄儿童在出现视力下降时,家长首先想到的是近视,就去配眼镜,配眼镜后孩子的视力仍然下降,眼科检查发现视神经萎缩后才想到颅内有肿瘤,耽误了治疗时机;3岁以内的小儿在视力下降时,家长甚至难以察觉,有的直到完全失明后才发现。我科曾治疗一例2岁儿童,完全失明后才发现脑内有肿瘤,及时作手术后视力恢复很好。五十多岁的病人在出现视力下降后首先想的是老花眼,也去配眼镜矫正,结果视力越来越差,最后作头CT才发现颅咽管瘤。
◇头痛。
头痛是一种非特异性的症状。很多人都有头痛的体验,常常视为感冒或劳累或高血压,不会想到是颅内有肿瘤。当渐渐出现视力下降、精神萎靡不振、健忘等症状后才提示颅内有肿瘤。
◇内分泌症状。
儿童颅咽管瘤的内分泌症状表现为生长慢、食欲差、尿多、喝水多,身高明显低于正常儿童;进入青春期年龄却无第二性征发育;成人内分泌症状表现为女性月经稀少、停经、性欲下降、不孕不育;男性性功能减退、性欲下降。
成年人也会出现喝水多、尿多症状。
儿童生长慢常常被认为是营养差,家长一般不会想到颅内肿瘤,不能及时到医院检查。
成年女性出现不孕不育后常常到妇产科检查,忽略了颅内肿瘤;
男性出现性功能减退也常常想到“肾虚”,用中药“补肾”。
“补肾”治疗往往无效,当病人又出现视力下降、头痛等症状后才想到颅内可能有肿瘤,延误了确诊时间。
二、颅咽管瘤位置深,手术难度大
颅咽管瘤最常见的部位是在鞍区,属于颅脑的中心地带,位置深,并且周围有视神经、颈内动脉、大脑前动脉、垂体、下丘脑等主要结构,而且颅咽管瘤常常与下丘脑粘连。
在手术过程中如果视神经受损,可以导致视力下降、失明;颈内动脉或大脑前动脉受损,会造成大出血,危及生命;下丘脑、垂体受损会出现垂体功能低下、内分泌紊乱,轻者造成病人体质差、抵抗力差,严重者也会造成生命危险。颅咽管瘤的手术难点在于既要尽可能的完全切除肿瘤,防止肿瘤复发,又要避免手术对肿瘤周围的重要结构造成损害。
三、颅咽管瘤切除后的垂体功能低下是世界难题
颅咽管瘤完全切除或者积极切除后(即不是姑息性手术)几乎都有垂体功能低下,表现为多饮多尿、肾上腺皮质功能低下、甲状腺功能低下、性腺激素低下、代谢紊乱。手术后的激素替代治疗是确保病人生命安全的重要手段。
有的颅咽管瘤病人在切除肿瘤后的前2天精神好,但随后出现精神萎靡、电解质紊乱、内分泌紊乱,如果不能正确处理,则病人会出现生命危险。我科在长期的临床工作中,对颅咽管瘤手术后的内分泌紊乱和电解质紊乱的治疗方面积累了丰富的经验,使颅咽管瘤的手术死亡率下降到2%以下。
四、颅咽管瘤易复发
颅咽管瘤虽然是良性肿瘤,但它与普通的良性肿瘤不同,没有包膜,有侵润性生长的特点。虽然在显微镜下看达到完全切除肿瘤,但是仍然有极少部分肿瘤细胞残留,造成后期肿瘤复发。颅咽管瘤对放射治疗和化疗都不敏感,一旦复发了治疗的难度进一步增加。
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