VHL patients are predisposed to certain kinds of cancer. The most feared is Renal Cell Carcinoma. Kidney Cancer.
Once you have symptoms...it's too late. Early detection is critical. You may develope cysts and/or tumors...it's important to check regularly.
My Uncle Bill had RCC (Renal Cell Carcinoma). He passed away in 1981.
What is Cancer?
Cancer can be a frightening word. Families need to know that cancer canoccur with VHL. However, with careful early monitoring and treatment, the worst possibilities of cancer may never occur.
Cancer is not one thing, it is a group of more than 100 different diseases. While each disease differs from the others in many ways, every cancer is a disease of some of the body’s cells. Cancer associated with VHL is limited to specific types.
Healthy cells that make up the body’s tissues grow, divide, and replace themselves in an orderly way. This process keeps the body in good repair. Sometimes, however, normal cells lose their ability to limit and direct their growth. They divide too rapidly and grow without any order. Too much tissue is produced, and tumors begin to form. Tumors can be benign or malignant.
• Benign tumors, such as VHL tumors of the brain, spine, and retina, are not cancerous and do not spread.
• Malignant tumors, like those which may occur in the kidney, are cancerous. They can invade and destroy nearby healthy tissues and organs. Cancer cells also can spread, or metastasize, to other parts of the body and form new tumors.
Because VHL can cause malignant tumors in the visceral organ systems, it is considered one of a group of familial cancer risk factors, which are transmitted genetically. The objective is to find tumors early, watch for signs that a tumor is becoming aggressive in its behavior, and to remove the tumor before it invades other tissues. Since these tumors are inside the body, you need medical imaging techniques to find and watch them.
Not all tumors require surgery when they are found. Research is going on to learn more about how to tell when a tumor is getting worrisome and requires action. You and your family can help researchers learn more about how long we can safely watch tumors by sharing your family’s own experiences. Please contact the VHL Family Alliance for more information on researching your family tree.
I am not a doctor or an expert by any means. Please visit VHL Family Alliance website for more information. http://www.vhl.org/