When you hear the word cancer used in relation to you or your family, you may have any one of many normal reactions, such as fear, confusion, panic, anger and anxiety. You may feel like your life is turning upside down. Others who have not experienced this may not understand what you are going through.
It is important to find the help you need to get you through this. On a personal level, you may want to talk to a family member, friend, religious advisor, counsellor, or join a cancer support group. On a medical level, we recommend finding a doctor who will listen and who should refer you to a group of specialists in different areas (such as an oncologist, urologist, dermatologist, pulmonologist depending on your symptoms), as well as a genetic counsellor and/or geneticist.
Because BHD syndrome is so rare, you may have trouble finding a doctor who knows about BHD-associated kidney cysts and tumours. A well-trained urologist, urologic oncologist, or oncologist may not be aware that BHD-associated kidney cancer is not necessarily treated the same way as ‘normal’, or sporadic kidney cancer. You may be the only person, or family, with BHD syndrome in your area. Even a doctor who sees many people with kidney cancer may never have met anyone with BHD syndrome. That is why it is vital that you know as much as possible, so that you can make the best decisions with your doctors as a team. You may wish to download a Clinical Introduction information pamphlet from our website to give to your doctors.
It is good to know that the BHD-associated kidney cancer in most cases is manageable and does not spread (metastasise). To date, there are very few reported cases of death due to BHD related kidney cancer. The very small number of fatalities that have been associated with BHD have been clear cell, chromophobe or papillary cases. The metastatic kidney cancer types that led to BHD-related deaths were a form of clear cell or papillary RCC. Given these statistics, a clear cell or papillary kidney tumour diagnosis may seem especially frightening. However, regular scanning should enable your doctors to catch any cancers early and arrange an appropriate treatment.
Though BHD tumours are considered to be slow-growing, both tumours and cysts may continue to ‘pop up’ after earlier cysts and/or tumours have been treated and removed; currently, it is not known how to stop this growth. Consequently, it is very important that you are regularly monitored by a team of doctors to catch any tumours early on.
Publication date: September 2012
Review date: September 2014