希佩尔-林道病

为什么选择UCLA健康中心进行VHL疾病护理?

皇冠hga025大学洛杉矶分校健康's Von Hippel-Lindau (VHL) Disease Program is the only VHL clinical care center in Southern California recognized by the VHL Alliance 和 one of only a few in the western United States. 这个称号意味着你将接受一个协调的, multispecialty team of VHL 专家 that you can trust to provide you with comprehensive care.

我们课程的亮点包括:

Multispecialty护理: 我们的团队与 皇冠hga025大学洛杉矶分校健康约翰逊综合癌症中心 专家和其他人. 我们与专家合作 泌尿外科, 眼科学, 神经外科外科肿瘤学; 胃肠病学临床遗传学; 神经学, 放射学 和 核医学 提供协调的、全面的护理.  

高容量: 我们每年在皇冠hga025大学洛杉矶分校医疗中心治疗100多名VHL患者, 使我们成为美国西部最大的VHL项目之一. 我们的医生在VHL治疗方面有丰富的经验.

Physician-leaders: 皇冠hga025大学洛杉矶分校健康 physicians are active researchers that focus on VHL 和 related conditions, as well as clinicians who continually bring you the latest therapies in their areas of specialization. 我们的几个团队成员担任VHL联盟的董事会成员, where they contribute to establishing VHL treatment guidelines 和 setting the priority for research in this area.

基因检测及咨询: A team of genetic counselors provides individualized testing 和 counseling for VHL disease. 甚至在出生前， 母胎医学 和 生殖健康 specialists can offer genetic testing 和 discuss family planning options with you.

获得医疗服务: 远程医疗预约扩大了获得我们专家护理的机会. Family members who live far away may receive specialized VHL care from various providers.

什么是Von Hippel-Lindau (VHL)病?

VHL疾病是一种罕见的遗传病(1 / 30),000 births) characterized by the growth of tumors 和 cysts throughout the body. 这些肿瘤可能是非癌性的(良性)或癌性的(恶性)。. The symptoms of VHL disease usually appear in young adulthood but can affect people of all ages. 几乎所有的VHL患者在60岁之前都有肿瘤.

VHL会发生哪些类型的肿瘤?

People with VHL disease have a higher risk of developing tumors or cysts in organs throughout their body. VHL disease is commonly associated with specific types of tumors 和 cysts, including:

肾上腺肿瘤: 嗜铬细胞瘤是一种起源于肾上腺的肿瘤, 哪一种负责产生各种激素. 它影响了大约五分之一的VHL患者. 这些肿瘤可以分泌肾上腺素，并可能导致高血压, 心率过快(心动过速), 心跳过快(心悸), 头痛, 出汗或恶心.

内淋巴囊肿瘤: 这些罕见的肿瘤生长在内耳. 它们会引起耳胀感, 部分或完全听力丧失，耳鸣(耳鸣).

脑和脊髓肿瘤: 许多VHL患者会发展成血管母细胞瘤, 由大脑或脊髓的新血管形成的肿瘤. 血管母细胞瘤可引起神经系统症状. 取决于肿瘤的位置, 症状可能包括肌肉无力, 失去协调性, 呕吐或头痛.

胰腺肿瘤和囊肿: 许多患有VHL疾病的人在胰腺上长有囊肿, 消化器官帮助消化食物和调节血糖的器官. Less commonly, people with VHL disease develop 胰腺神经内分泌肿瘤 (pNET). Pancreatic cysts 和 tumors rarely cause symptoms but may compress nearby organs if they grow large 和 spread to distant sites.

肾囊肿及肿瘤: 更多的 than half of people with VHL disease develop cysts or tumors on their kidneys, the organs that produce urine to remove waste 和 excess fluid from your blood. 小肿瘤可能没有任何症状, 而大的会导致疼痛, 腹部肿块或尿中带血. 大的肿瘤也有可能扩散到远处.

生殖损伤: A noncancerous tumor called a cystadenoma develops in about half of all men with VHL disease. A similar rare tumor has been described in women with VHL disease in the pelvis (within the broad ligament). Cystadenomas often cause no symptoms but may lead to pain 和 pressure in the scrotum or pelvic area.

视网膜肿瘤: A retinal hemangioblastoma forms in the tissue lining the back of the eye (retina). 这些肿瘤可能导致视力丧失，严重时甚至失明.

我们提供的VHL疾病治疗

With appropriate treatment, people with VHL disease can live a long 和 healthy life. But patients often may need multiple surgeries to ensure that tumors or cysts don't cause long-term organ damage. 我们的 specialists monitor you regularly to look for signs of new tumors or cysts or check the growth of an existing tumor or cyst.

如果你有肿瘤或囊肿，治疗可能包括:

积极监测: Your doctor monitors the tumor or cyst without any formal medical treatment to see if it grows or if you develop new symptoms. We may not need to intervene immediately if a tumor or cyst isn't causing symptoms.

口服药物治疗: 你可以服用belzutifan, an oral medication that can help shrink a tumor or stop it from growing further. Belzutifan是由美国批准的.S. 美国食品和药物管理局(FDA)针对患有VHL疾病的肾脏患者, 胰腺, brain or spinal cord tumors that will require treatment but do not need immediate surgery.

激光治疗: 你的医生使用激光能量来缩小或摧毁视网膜肿瘤. They aim the laser through your pupil 和 target the blood vessels around the tumor. 

光动力治疗: 你需要注射一种光敏剂，一种由光激活的药物. Then your doctor directs laser energy through your pupil to the back of your eye. The laser activates the photosensitizer to destroy a retinal tumor 和 the blood vessels that supply it.

消融: 一些vhl相关的肾脏肿瘤可以用针治疗. 使用极端温度(高温或低温)可能会摧毁癌症. 对一些人来说, this approach may provide outcomes similar to surgery while reducing complication rates.

栓塞: 在这个微创手术中, 我们使用长, 空心管(导管)进入供应肿瘤的血管. Your doctor injects a substance into the blood vessels to block blood supply to the tumor. We may use embolization to lower the risk of bleeding before surgically removing the tumor.  

立体定向放射治疗: This external radiation therapy targets brain or spinal cord tumors with precisely focused radiation beams. 你戴上一个头框或面罩，帮助稳定你的头部. Your doctor uses imaging scans 和 specialized software to plan 和 deliver focused radiation therapy to the tumor. 

开放、机器人或腹腔镜手术切除: 我们的医生通过手术切除肿瘤. 医生可能会采用开放式方法，通过一个大切口进行手术. Or they may be able to perform a minimally invasive technique using small instruments 和 cameras through tiny incisions. 我们的 doctors frequently perform open 和 minimally invasive surgery for VHL-associated central nervous system (CNS) 和 spinal hemangioblastomas, 胰腺神经内分泌肿瘤, 嗜铬细胞瘤和肾细胞癌.

玻璃体切除术: In some cases, you may need a vitrectomy to treat a VHL-associated hemangioblastoma. 在这个过程中, 眼科医生切除肿瘤和部分或全部玻璃体, 你眼睛中间的凝胶状液体. They replace the vitreous with an oil or gas bubble or a saline solution to help your retina stay in place. As you heal, your eye replaces the liquid or bubble with a natural fluid called aqueous humor.

我们的专家团队

我们的 专家 are active researchers 和 clinical leaders who bring you the most advanced treatment options. 我们的 multispecialty approach allows us to offer comprehensive care for patients with VHL disease.

皇冠hga025

呼叫 310-794-2858 要求预约皇冠hga025大学洛杉矶分校健康中心的VHL疾病专家.