During a nephrectomy, it is possible to remove just part of the kidney (partial nephrectomy) or a whole kidney (radical nephrectomy).
In a partial nephrectomy, just tumours and any nearby kidney tissue is removed, leaving as much healthy kidney tissue as possible intact. For BHD patients, this is the preferred treatment compared with removing a whole kidney. Your surgeon will decide whether this is an option in your case.
Sometimes, depending on the size of your tumours, how many you have and where they are, you may need to have your whole kidney removed. If your surgeon plans to do a radical nephrectomy, it is very important you tell him that you have BHD and you may get tumours in the other kidney at a later date.
Nephrectomies can be performed as an open surgery, or by keyhole surgery (laparoscopy) where the surgeon only makes a few small incisions and uses specialist instruments and a small camera inserted through the incisions to operate. Recovery times are generally much shorter following keyhole surgery, and there is usually less pain, bleeding and scarring. Some surgeons use robotic equipment to improve the precision of laparoscopic partial nephrectomies.
An experimental technique called Natural Orifice Translumenal Endoscopic Surgery (NOTES) has been used to perform nephrectomies in a handful of cases. NOTES is a minimally invasive technique that uses existing orifices, such as the mouth, vagina, rectum or naval to access internal organs.
Ablation can be used to remove smaller tumours. There are several types of ablation – radiofrequency ablation, cryoablation, ultrasound ablation, and microwave ablation – which use radiowaves, extreme cold, ultrasound, and microwaves to kill off tumour cells.
However, BHD experts do not recommend ablation for BHD patients with kidney tumours. Ablation techniques are best suited to patients with a single, small tumour. As BHD patients often have more than one tumour, the ablation surgery required would be very extensive, making partial nephrectomy a better option. Additionally, ablation can make it difficult to treat any tumours that may grow in that kidney later.
Kidney transplant and dialysis
It is possible that the kidney cancer becomes so extensive that it causes kidney failure, meaning a patient requires a kidney transplant or dialysis.
Although we have not heard of any BHD patients who have had a kidney transplant, there have been reports of patients with another genetic kidney cancer syndrome (VHL) who have had successful kidney transplants.
There is a very long kidney transplant waiting list in many parts of the world and transplants using organs from compatible family members or friends are easier to arrange. However, if your family member also has BHD, they would not be a suitable donor as the transplanted kidney may develop tumours, and the donor may also develop cancer in their remaining kidney.
There are a few reports of people with Birt-Hogg-Dubé syndrome who are on dialysis.
However, if any tumours are discovered early and removed when they are only 3 cm, this will reduce the chances of needing a whole kidney removed. This will in turn reduce the chances of needing dialysis or a transplant.
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