All the treatment options listed below are used in the treatment of kidney cancer. There is currently no treatment that has been developed specifically for BHD-associated kidney cancer. Although these treatments may be used to treat patients with BHD syndrome, their effectiveness for this type of cancer is unclear.
Interferons are proteins which function as part of the body’s immune response. In cancer treatment, interferon is a form of immunotherapy which works in several ways:
- it can directly help to stop cancer cell growth;
- it may also boost the immune system to attack the cancer;
- it may restrict the blood supply to the cancer cells.
Interferon treatment can help to stabilise or shrink the tumour, rather than removing it altogether. Interferon treatment has been shown to help patients with advanced kidney cancer, particularly metastatic cancer which has spread to other organs. However, people who benefited most from this treatment were in good general health, with few cancer symptoms.
The type of interferon used for kidney cancer is interferon-2alpha (Roferon-A). It is usually taken three times a week as a small injection just under the skin.
Interleukin 2 (also known as IL-2 or aldesleukin) is produced naturally in the body as part of the immune system. It stimulates white blood cells (lymphocytes) to fight infection. IL-2 can now be made in laboratories and is most often used to treat advanced kidney cancer.
IL-2 can be used on its own or in combination with other treatments, such as interferon or a chemotherapy drug called 5FU, for the treatment of advanced kidney cancer. IL-2 is also being tried as a treatment after surgery for early kidney cancer. The aim of this treatment is to try to lower the chance of the cancer recurring. Doctors call this adjuvant therapy.
Adjuvant therapy is treatment that is given after surgery or initial treatment where all detectable disease has been removed, but where there is a risk that the cancer will return. In general, adjuvant therapy has not been an option for BHD syndrome, due to the multifocal (multiple tumours) and bilateral (both kidneys affected) nature of BHD-associated tumours. There is currently no treatment that can be given after the first surgery that can stop the tumour growth in the kidneys. However, this is an avenue that researchers are currently working on.
Sunitinib, also known as Sutent, is a tyrosine kinase inhibitor (TKI). Tyrosine kinases are enzymatic proteins that are important in signal transduction within a cell. Sunitinib blocks these tyrosine kinases, which prevents cancerous cells from growing. Consequently, sunitinib can stop or slow the growth of advanced kidney cancer.
In the UK, the National Institute for Health and Clinical Excellence (NICE) has issued guidance that sunitinib should be available as a treatment option for people with advanced kidney cancer, if they are suitable for immunotherapy (e.g. Interferon or IL-2 treatment), and reasonably fit (e.g. well enough to do light house work).