Von Hippel-Lindau Disease (VHL) and Hereditary Renal Cell Carcinoma

Genetic syndromes related to kidney cancer can have effects beyond the kidneys. People with these conditions experience other non-cancer-related illnesses. They may also notice symptoms at younger ages.

Our nationally recognized Translational Medicine and Human Genetics program includes experts in kidney cancer genetics. These doctors closely collaborate with your cancer specialists to provide care that considers every part of you.

What is Von Hippel-Lindau Disease?

Von Hippel Lindau Disease (VHL) is a rare genetic disorder that occurs when there is a growth of tumors and cysts in certain organs of the body.

Is Kidney Cancer Hereditary?

Certain factors increase your risk for developing renal cell carcinoma, or kidney cancer. Rarely, kidney cancer occurs because parents pass down certain gene changes to their children. Doctors call this kind of kidney cancer hereditary renal cell cancer.

Some familial (hereditary) syndromes can increase your risk for these hereditary renal cell cancers. These syndromes are known as familial renal cell carcinoma syndromes or genetic kidney cancer syndromes.

Familial Renal Cell Carcinoma Syndromes: Why Choose the Abramson Cancer Center?

We take a team approach to familial kidney cancer care that includes genetic counseling. Our genetic counselors use their expertise to help you:

  • Understand how hereditary syndromes affect you and your family
  • Understand results from genetic testing
  • Make informed decisions about your health

Your genetic counselor is like your own private investigator for your genes. They make sure we get the information and tests needed to provide you with complete care.

Familial Renal Cell Carcinoma Syndromes We Treat

We offer expertise in genetic kidney cancer syndromes. These syndromes include:

Birt-Hogg-Dubé Syndrome (BHD)

Kidney cancers associated with Birt-Hogg-Dubé syndrome tend to grow slowly. Birt-Hogg-Dubé syndrome can also lead to:

  • Skin lesions (fibrofolliculomas)
  • Collapsed lung (spontaneous pneumothorax)
  • Lung cysts

Von Hippel-Lindau Disease (VHL)

Kidney cancers associated with VHL tend to be slow growing. VHL can also lead to cancerous and noncancerous tumors in the:

  • Adrenal glands
  • Central nervous system
  • Ears
  • Eyes
  • Pancreas
  • Sex organs

Our Von Hippel-Lindau (VHL) Syndrome Center is dedicated to the care of adults and children with VHL. It offers multidisciplinary care including:

  • Diagnosis
  • Medical management
  • Genetic counseling and testing
  • Education
  • Support

Hereditary Papillary Renal Cell Cancer

Hereditary papillary renal cell cancers include:

  • Hereditary papillary renal cell carcinoma (type 1 papillary)
  • Hereditary leiomyomatosis and renal cell cancer, or HLRCC (type 2 papillary)

Kidney cancers associated with hereditary papillary renal cell carcinoma tend to grow slowly.

Kidney cancers associated with HLRCC tend to grow quickly. HLRCC can also lead to:

Our Hereditary Renal Cell Carcinoma Treatments

Our kidney cancer treatments include:

Active Surveillance (Watchful Waiting)

Because doctors find many of these cancers early, when they are at low risk of spreading, active surveillance can be effective. During active surveillance, our skilled advanced practice providers (APPs) regularly check tumors for growth and spread. Patients can often avoid more invasive treatments and side effects.

Our APPs specialize in the care of urologic cancers like renal cell carcinoma. Their special training in this area helps them notice any concerning changes early — when treatment can have the most impact.

Kidney Cancer Surgery (Nephrectomy)

When you have hereditary kidney cancer, you are at higher risk of developing multiple tumors during your life. Kidney removal surgery (nephrectomy) can be an effective way to treat these tumors, but it can have long-term effects on kidney function. You may also need multiple surgeries over time. Learn more about kidney cancer surgery.

Renal Ablation

We perform renal ablation for people who can’t or don’t want to have kidney cancer surgery. Ablation is a minimally invasive treatment that kills tumors with energy sources. These sources include electrical currents (radiofrequency ablation) and extreme cold (cryotherapy).

Advanced imaging technology with ablation helps doctors precisely target tumors and spare healthy kidney tissue. Other benefits of tumor ablation include:

  • Lower risk for complications
  • Ability to treat multiple tumors at the same time
  • Shorter hospital stays

At the Abramson Cancer Center, an interventional radiologist with expertise in tumor ablation is part of our kidney cancer team. This setup allows our entire team to weigh in on your renal ablation — and not just your ablation specialist.

Make an Appointment

Please call 800-789-7366 or request a callback.