Von Hippel-Lindau Syndrome: Pancreatic Cancer

Von Hippel-Lindau: Pancreas

Having Von Hippel-Lindau syndrome (VHL) can increase your risk of getting pancreatic cancer from 9% to 17%. Patients with VHL-associated pancreatic tumors can be found in 3 types, these include the following:

• Pancreatic cysts: these happen in 75% of VHL patients and typically present with no symptoms unless they start pressing on surrounding organs in which case, they need immediate intervention.
• Pancreatic tumors: these account for 17% of VHL patients and don't need to be removed unless they start affecting enzyme function and fluid flow.
• Pancreatic neuroendocrine tumors (pNET): also known as islet cell tumors, these are the most commonly occurring in VHL. They account for only 2% of pancreatic cancers but have a better outlook for patients.
• Other types of pancreatic cancers include the following:
• Pancreatic adenocarcinoma (accounts for 95% of all pancreatic cancers)
• Ampullary cancer or carcinoma of the ampulla of Vater
• Exocrine cancers

What are the Symptoms of Pancreatic Cancer?

Signs and symptoms of pancreatic cancer may not appear in the early stages, and they may also resemble those of other conditions, making for a challenging diagnosis. Make sure to check with your doctor if you have any of the following symptoms:

• Jaundice (yellowing of the skin and the eyes)
• Pain in the abdominal and back area
• Fatigue and tiredness
• Loss of appetite
• Unexplained weight loss
• Dark urine
• Nausea
• Vomiting
• Light stool color
• Lump in your right ribs

Can Pancreatic Cancer be Detected Early?

Since Von Hippel-Lindau disease is a risk factor for developing pancreatic cancer, you have a chance of preventing this disease from progressing to the advanced late stages (metastatic stage), where the cancer spreads to other parts of the body, decreasing your chance for recovery.

This can be done by performing routine screening and following up with your healthcare provider regularly about any symptoms, and undergoing screening tests to find out whether you've developed any tumors or not. By detecting tumors early, you're significantly increasing your chances of treatment response and your disease outlook.

Below are some of the diagnostic tests for pancreatic cancer that are prescribed by your doctor if they suspect you have pancreatic cancer:

• Physical examination and medical history
• Blood tests (to check liver enzymes and bilirubin levels in the blood, also to check CA19-9 which is also called carbohydrate antigen and is considered a tumor marker)
• Imaging tests (CT scan, PET scan, ERCP, ultrasound, and MRI)
• Biopsy (taking a sample from the tumor and examining it in the lab under the microscope)

What Complications Are Involved with Pancreatic Cancer?

Left untreated, pancreatic cancer can bring about subsequent complications specific to the location and size of the tumor; these include the following:

• Diabetes mellitus
• Bowel obstruction
• Blood clots
• Pancreatic insufficiency (when the function of enzymes produced by the pancreas is disrupted, resulting in malabsorption, malnutrition, and cramps in the abdomen)

How Can I Prevent Pancreatic Cancer?

As a Von Hippel-Lindau patient, the best strategy for you to reduce your risk of pancreatic cancer is to undergo routine screening checkups to improve your chances of early detection. When found early, pancreatic cancers have a good chance of responding to treatment.

Since there's no guaranteed way to prevent pancreatic cancer, improving your lifestyle and quitting bad habits have been recommended by the American Cancer Society to manage your risk better, these include the following:

• Quitting smoking
• Reducing alcohol intake
• Maintaining a healthy diet
• Watch your weight
• Preventing exposure to chemicals

What Treatments are Available for Pancreatic Cancer?

There are various treatment options for pancreatic cancer, and they depend on many factors, including the following:

• Stage of pancreatic cancer
• Tumor size
• Tumor number
• Tumor location
• Previous treatments
• Overall health of the patient
• If the patient has any underlying medical conditions

After considering these factors, your doctor will present you with your treatment choices. This is your chance to ask any questions to understand more and share any concerns you might have about the next step in your treatment.

Make sure to ask your doctor about the goal of the recommended treatment and the risks or side effects associated with this option. If you're interested in participating in a clinical trial, they can guide you about your next steps and the eligibility criteria, and whether you qualify or not.

Below are the treatment options available for pancreatic cancer:

Surgery. This aims to remove tumors from the pancreas; many types of surgeries are available, depending on your case. However, the tumor needs to be accessible for this to work. Only 20% of patients with pancreatic cancer undergo surgery because, by the time they're diagnosed, they have reached late stages where the tumor has grown significantly or metastasized to other areas of the body.

Radiation therapy. This aims to eliminate tumors by projecting high-energy x-rays onto the specific site of the tumor. Many types of radiotherapy are available; your doctor will recommend the most suitable one for your case.

Chemotherapy. This treatment aims to inhibit the growth of tumors or destroy it using chemotherapeutic drugs that have a toxic inhibitory effect on the tumor. There are many types of chemotherapy drugs available; your doctor will prescribe the best suitable one for your case.

Targeted therapy. This treatment aims to target specific proteins or genes in the cancer cell to block its growth and minimize damage to the surrounding healthy cells. Recently, belzutifan a new targeted therapy drug that was approved by the FDA in August 2021, has been used to treat patients with pancreatic neuroendocrine tumors as well as those who have clear renal cell carcinoma and central nervous system hemangioblastomas.

Immunotherapy. This treatment aims to stimulate the functions of the immune system to better identify and eliminate cancer cells. Immune checkpoint inhibitors are a type of immunotherapeutic drug that has been approved in the treatment of several types of cancers, including pancreatic cancer. There are many kinds of these drugs; your doctor will decide according to the type of cancer you have.

Your doctor may prescribe one treatment or pair up two or more treatments together according to your condition.

Questions to Ask Your Doctor

• Which type of pancreatic cancer do I have?
• What is the stage of my cancer?
• What are my treatment options to fight off this disease?
• What are the side effects of the recommended treatment?
• Should I consider enrolling in a clinical trial?
• What are my chances of recovery?
• Is there anything I should do to improve my quality of life with this disease?

The Bottom Line

As a Von Hippel-Lindau patient, you should look out for any of the previous symptoms that indicate you have pancreatic cancer. Remember to follow up regularly with your doctor and perform your screening tests. Catching pancreatic cancer early goes a long way in improving your chances of survival and prolonging your recovery.

Especially now, since there have been many recent advancements in treating VHL-associated cancers like the approval of belzutifan. Although this medication needs further investigations when it comes to learning its long-term safety and effectiveness, it holds great promise as a candidate for patients with VHL-associated pancreatic cancer.

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