Von Hippel-Lindau Disease

我们的专家团队领导着美国西部最大的VHL治疗项目之一. You receive comprehensive, multispecialty care.

Female nurse comforting older female patient

Why choose 皇冠hga025大学洛杉矶分校健康 for VHL disease care?

皇冠hga025大学洛杉矶分校健康中心的冯希佩尔-林道(VHL)疾病项目是南皇冠hga025唯一获得VHL联盟认可的VHL临床护理中心，也是美国西部为数不多的VHL临床护理中心之一. This designation means you receive treatment from a coordinated, 您可以信赖的VHL专家多专业团队为您提供全面的护理.

Highlights of our program include:

Multispecialty care: 我们的 team works closely with 皇冠hga025大学洛杉矶分校健康 Jonsson Comprehensive 癌症中心专家和其他人. We collaborate with specialists in 泌尿外科, 眼科学, 神经外科, surgical oncology, 胃肠病学, clinical genetics, 神经学, 放射学和核医学 to provide coordinated, comprehensive care.

高容量: We treat more than 100 patients at 皇冠hga025大学洛杉矶分校健康 with VHL each year, making us one of the largest VHL programs in the western United States. 我们的 physicians have extensive experience in VHL treatment.

Physician-leaders: 皇冠hga025大学洛杉矶分校的健康医生是专注于VHL和相关疾病的活跃研究人员, 以及不断为您带来专业领域最新疗法的临床医生. Several of our team members serve on boards of the VHL Alliance, 他们在制定VHL治疗指南和确定该领域研究重点方面做出了哪些贡献.

Genetic testing 和 counseling: 一组遗传咨询师为VHL疾病提供个性化的测试和咨询. 甚至在出生前， maternal-fetal medicine 和 reproductive health 专家可以提供基因测试，并与你讨论计划生育的选择.

获得医疗服务: Telehealth appointments exp和 access to our expert care. 居住较远的家庭成员可以从不同的提供者那里接受专门的VHL治疗.

What is Von Hippel-Lindau (VHL) disease?

VHL disease is a rare genetic condition (1 in 30,以全身肿瘤和囊肿生长为特征的一种疾病. These tumors may be noncancerous (benign) or cancerous (malignant). VHL疾病的症状通常出现在年轻的成年期，但可以影响所有年龄段的人. Nearly all patients with VHL have a tumor before age 60.

What types of tumors occur in VHL disease?

患有VHL疾病的人在全身器官中发生肿瘤或囊肿的风险更高. VHL疾病通常与特定类型的肿瘤和囊肿相关，包括:

肾上腺肿瘤: Pheochromocytoma is a tumor that arises from the adrenal gl和, which is responsible for producing various hormones. It affects around one-in-five patients with VHL disease. These tumors can secrete adrenaline 和 may cause high blood pressure, rapid heart rate (tachycardia), periods of too-fast heartbeats (palpitations), 头痛, 出汗或恶心.

Endolymphatic sac tumors: These rare tumors grow in the inner ear. They can cause sensations of ear fullness, partial or complete hearing loss 和 ringing or buzzing in the ears (tinnitus).

Brain 和 spinal cord tumors: Many people with VHL disease develop hemangioblastomas, tumors that form from new blood vessels in the brain or spinal cord. Hemangioblastomas may cause neurologic symptoms. Depending on the tumor location, symptoms may include muscle weakness, loss of coordination, vomiting or 头痛.

Pancreatic tumors 和 cysts: Many people with VHL disease have cysts that grow on their pancreas, an organ that helps digest food 和 regulate blood sugar. 不太常见的是，VHL患者会发展为胰腺神经内分泌肿瘤(pNET)。. 胰腺囊肿和肿瘤很少引起症状，但如果它们变大并扩散到远处，可能会压迫附近的器官.

Renal cysts 和 tumors: 超过一半的VHL患者的肾脏会出现囊肿或肿瘤, the organs that produce urine to remove waste 和 excess fluid from your blood. Small tumors may cause no symptoms, while large ones can lead to pain, an abdominal mass or blood in your urine. Large tumors also have the potential to spread to distant locations.

Reproductive lesions: 一种叫做囊腺瘤的非癌性肿瘤发生在大约一半的VHL患者身上. 在骨盆(宽韧带内)的女性VHL疾病中也有类似的罕见肿瘤。. 囊腺瘤通常没有症状，但可能导致阴囊或骨盆区域疼痛和压力.

视网膜肿瘤: 视网膜血管母细胞瘤在眼睛后部(视网膜)的组织中形成。. These tumors may cause vision loss or, in severe cases, blindness.

VHL disease treatments we offer

通过适当的治疗，VHL患者可以过上健康长寿的生活. 但患者通常可能需要多次手术，以确保肿瘤或囊肿不会造成长期的器官损害. 我们的专家定期监测您，寻找新肿瘤或囊肿的迹象，或检查现有肿瘤或囊肿的生长情况.

If you have a tumor or cyst, treatment may include:

Active surveillance: 你的医生在没有任何正式治疗的情况下监测肿瘤或囊肿，看看它是否生长或你是否出现新的症状. 如果肿瘤或囊肿没有引起症状，我们可能不需要立即干预.

口服药物治疗: You may take belzutifan, 一种帮助缩小肿瘤或阻止肿瘤进一步生长的口服药物. Belzutifan is approved by the U.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, 需要治疗但不需要立即手术的脑或脊髓肿瘤.

激光治疗: Your doctor uses laser energy to shrink or destroy a retinal tumor. 他们让激光穿过你的瞳孔，瞄准肿瘤周围的血管.

Photodynamic therapy: You receive an injection of a photosensitizer, a medication activated by light. 然后你的医生引导激光能量通过你的瞳孔到达你的眼睛后部. 激光激活光敏剂，摧毁视网膜肿瘤和供应肿瘤的血管.

消融: Some VHL-associated renal tumors can be treated with a needle. Using extreme temperatures (heat or cold) may destroy the cancer. 对一些人来说, 这种方法可以提供与手术相似的结果，同时减少并发症的发生率.

栓塞: In this minimally invasive procedure, 我们使用长, hollow tube (catheter) to access the blood vessels supplying the tumor. 你的医生会在血管中注射一种物质来阻断肿瘤的血液供应. 在手术切除肿瘤之前，我们可以使用栓塞术来降低出血的风险.

Stereotactic radiation therapy: 这种外部放射疗法用精确聚焦的放射束靶向脑或脊髓肿瘤. You wear a head frame or face mask that helps stabilize your head. 你的医生使用成像扫描和专门的软件来计划并向肿瘤提供集中的放射治疗.

Open, robotic or laparoscopic surgical resection: 我们的 doctors treat many tumors by surgically removing them. The doctor may use an open approach, operating through one large incision. 或者他们可以通过微小的切口使用小型仪器和相机进行微创技术. 我们的医生经常为vhl相关的中枢神经系统(CNS)和脊髓血管母细胞瘤进行开放和微创手术, 胰腺 neuroendocrine tumors, pheochromocytomas 和 renal cell carcinomas.

玻璃体切除术: 在某些情况下，你可能需要玻璃体切除术来治疗vhl相关的血管母细胞瘤. During this procedure, an ophthalmologist removes the tumor 和 part or all of the vitreous, the gel-like fluid in the middle of your eye. 他们用一个油或气泡或盐水溶液代替玻璃体，以帮助你的视网膜保持在原位. 当你痊愈时，你的眼睛会用一种叫做房水的天然液体代替液体或气泡.