诊断
颅咽管瘤的诊断通常从病史回顾和症状讨论开始。用于诊断颅咽管瘤的检测项目包括：
- 神经学检查。在这项检查中，医疗护理专业人员会测试视力、听力、平衡性、协调性、反射以及生长发育。以确定大脑的哪个部分可能受到肿瘤的影响。
- 血液检查。血液检查可能显示激素水平的变化，表明肿瘤正在累及垂体。
- 影像学检查。影像学检查可捕获大脑图像。图像可显示出肿瘤的大小及位置。影像学检查包括 X 线、CT 和 MRI。在某些情况下，可能需要其他检测。
治疗
颅咽管瘤的治疗通常从手术开始。外科医生会尽可能切除整个肿瘤。有些情况下，可能无法完全切除颅咽管瘤。外科医生可能会在保证安全的前提下尽可能多地切除颅咽管瘤。
可以在手术之后利用放射疗法来治疗任何残余的肿瘤细胞。联合使用手术和放射疗法通常可以很好地控制肿瘤。这种方法还有助于降低手术后并发症的风险。
在某些情况下可能需要其他疗法，例如化疗和靶向治疗。
外科手术
对于颅咽管瘤患者，最常推荐通过手术切除全部或大部分肿瘤。进行哪种手术取决于肿瘤的位置和大小。
- 开放性颅咽管瘤手术。也称开颅术，是指将颅骨打开以接近肿瘤。
- 微创颅咽管瘤手术。也称经蝶窦手术，通过鼻子将特殊的手术工具插入，工具穿过天然通道到达肿瘤。这种方法对大脑的影响最小。
如果可能，外科医生会切除整个肿瘤。但是，由于肿瘤周围经常有许多精细而重要的结构，外科医生有时为了确保术后良好的生活质量不会切除整个肿瘤。在这些情况下，手术后可以使用其他疗法。
在进行手术时，外科医生会避免伤害肿瘤附近的结构，注意降低视力出现问题的风险，尽量减少对下丘脑的损伤。下丘脑有许多功能，包括控制食欲和警觉性。
有时采用手术来缓解大脑中液体积聚的问题（脑积水）。可以插入一根导管来排出液体。一般使用临时性导管，有时也需要使用永久性导管。永久性导管（分流管）可以将脑内积液引流到腹部。
放射疗法
放射疗法使用高能射束来控制肿瘤细胞。能量可以来自 X 线、质子或其他来源。
放射疗法通常用于手术后治疗任何残余的肿瘤细胞。
颅咽管瘤的各类放射疗法包括：
化疗
化疗是使用药物杀死肿瘤细胞。化疗药物可以直接注射到肿瘤中，使治疗药物到达靶细胞。这不太可能损害附近的健康组织。
乳头型颅咽管瘤的治疗
靶向治疗药物可能可用于治疗一种称为乳头型颅咽管瘤的颅咽管瘤。这类病症并不常见。成人患者中，约三分之一的颅咽管瘤患者所患的就是这种颅咽管瘤。
靶向治疗是使用药物来攻击肿瘤细胞中的特定化学物质。通过阻断这些化学物质，靶向治疗可杀死肿瘤细胞。
几乎所有乳头型颅咽管瘤细胞都含有一种称为 BRAF 基因的 DNA 变化。靶向治疗可能是针对这种变化的一种疗法。实验室检查可以显示颅咽管瘤是否含有乳头状细胞。检测还可以判断这些细胞是否具有 BRAF 基因突变。
临床试验
临床试验是对新疗法进行研究。这些研究让患者有机会尝试最新的治疗方法。副作用带来的风险可能未知。询问您的医疗护理团队您是否能够参加临床试验。
临床试验
探索 Mayo Clinic 的研究 测试新的治疗、干预与检查方法，旨在预防、检测、治疗或控制这种疾病。
准备您的预约
Make an appointment with a doctor or other healthcare professional if you have any symptoms that worry you.
If your healthcare professional thinks that you might have a craniopharyngioma, you may be referred to a doctor who specializes in diseases of the nervous system, called a neurologist.
Because appointments can be brief, it's a good idea to be prepared. Here's some information to help you get ready.
What you can do
- Be aware of anything you need to do ahead of time. At the time you make the appointment, be sure to ask if there's anything you need to do in advance, such as restrict your diet.
- Write down symptoms you have, including any that may not seem related to the reason for which you scheduled the appointment.
- Write down important personal information, including major stresses or recent life changes.
- Make a list of all medicines, vitamins or supplements you're taking and the doses.
- Take a family member or friend along. Sometimes it can be very hard to remember all the information provided during an appointment. Someone who goes with you may remember something that you missed or forgot.
- Write down questions to ask your healthcare team.
Your time with your healthcare team is limited, so preparing a list of questions can help you make the most of your time together. List your questions from most important to least important in case time runs out. For craniopharyngioma, some basic questions to ask include:
- Do I have a craniopharyngioma?
- Will I need more tests?
- What are the treatment options?
- How much does each treatment increase my chances of a cure or prolong my life?
- What are the potential side effects of each treatment?
- How will each treatment affect my daily life?
- Is there one treatment option you believe is the best?
- What would you recommend to a friend or family member in my situation?
- Should I see a specialist?
- Are there any brochures or other printed material that I can take with me? What websites do you recommend?
- What will determine whether I should plan for a follow-up visit?
Don't hesitate to ask other questions.
What to expect from your doctor
Be prepared to answer questions, such as:
- When did your symptoms begin?
- Have your symptoms been continuous or occasional?
- How severe are your symptoms?
- What, if anything, seems to improve your symptoms?
- What, if anything, appears to worsen your symptoms?