The following information is about second primary tumours. Reading the section on genetics may help in understanding some of the terms on this page.
Visit this section for more information about the genetics of retinoblastoma
This section relates to long-term effects of retinoblastoma. If you are a parent of a child currently in treatment you may not feel it is appropriate for you to read this until your child's treatment is concluded. Please do try and read this page as soon as you feel ready to do so as it is important.
Second primary tumours
The great majority of patients with retinoblastoma (Rb) are successfully treated. Some survivors have an increased risk of developing other types of cancer. Such further tumours of a different type are referred to as second primary tumours or second primary cancers.
The increased risk applies mainly to those with the heritable form of the disease (in which all cells of the body have an altered copy of the Rb gene). This group includes cases of Rb where the tumour is bilateral or multifocal or where there is a family history. It has been known for many years that patients surviving this form of Rb have an increased risk of developing sarcomas, i.e. tumours of the bones and soft tissues, within about 5-25 years of being treated for retinoblastoma. More recently it has become clear that they are also at an increased risk of developing other forms of cancer and the risk continues into later life. The level of risk depends also on the treatment given for the Rb. For a patient who has had no radiation treatment, or only a small dose, the increase in risk will be less.
Patients with unilateral Rb and no family history of Rb (most of whom have the non-heritable form of the disease) have a very much smaller chance of a further cancer developing. There is, however, still a risk among people affected with unilateral Rb; the reasons for this are partly that a small number of people affected by unilateral retinoblastoma actually have the heritable form and partly that some people who had unilateral Rb were treated with radiation, which can also increase the risk of developing a second primary cancer.
Patients in this group (unilateral Rb, no family history) will usually be examined using laboratory investigations (genetic testing) to see whether they do in fact have the heritable form. The main reason for doing this will be to determine whether their children are at risk of Rb but it is also important in determining whether they have an increased risk of further cancers.
Looking after yourself
Be aware of the signs of cancer
Seek medical advice from a GP for any unexplained or persistent pain, lumps or new moles or changes to an existing mole
Wear sunscreen, avoid over exposure to the sun and don't use sunbeds.
Do not smoke.
Make sure you are in adult oncology (cancer) follow-up.
This advice is intended mainly for patients with the heritable form of Rb, but should be followed also by those with the non-heritable form.
It is important for people who have had Rb to be aware of the possibility of a second tumour occurring.
The risks are for various types of cancer, anywhere on or in the body. If you have an increased risk of developing cancers you should be aware of the signs of the common cancers as well as knowing how to check yourself. The link below takes you to the Cancer Research UK website to a page about the signs and symptoms of cancer. You can refresh your awareness of the signs here.
Though the increased risk of developing a second primary tumour could mean various types of cancer, certain tumours are worth noting in more detail.
Osteosarcomas can arise in any bone in the body, but most commonly in one of the leg bones or in the bones of the orbit around the eye. Soft tissue sarcomas can occur in the muscles of the face and scalp, or in the abdomen. Both these tumours tend to affect people relatively early in life.
One of the other tumour types for which there is an increased risk is melanoma, a cancer of the skin. As melanomas can develop as a consequence of sun exposure, it is important for everyone who has Rb to use sunscreens, avoid over-exposure to the sun and they are advised not to use sun beds. Looking out for warning signs such as the appearance of a new skin mole or a change in the appearance of an existing mole is also very important. Those with visual impairment should make sure someone else looks at their skin regularly.
People with heritable Rb are also more susceptible to other risk factors for cancer, and so should be strongly advised not to smoke.
Medical advice should be sought for any worrying or persistent problems such as unexplained lumps or pains, new skin moles or changes to an existing mole. It may be worth reminding your GP that you have a higher risk of getting second primary tumours as you had Rb as a child.
Anyone who has had Rb should be monitored to detect and manage any long-term problems caused by the disease or treatment in order to ensure the best possible quality of life. It is especially important for those with the heritable form of Rb to be seen in an adult oncology (cancer) follow-up clinic for the reasons mentioned above. If you are not currently seen in a follow-up clinic you need a referral letter from your GP to be seen either by Dr Judith Kingston (London) or Dr Helen Jenkinson (Birmingham) The referral letter should detail the treatment you had as a child if this is information the GP has available. In many cases one of these doctors will see you for the first appointment and then refer you to a more local adult oncology follow up clinic.
If you want to get in contact with either of the doctors above you can contact them via the retinoblastoma team co-ordinators.
The Retinoblastoma Service, The Royal London Hospital, 0203 594 1419
The Retinoblastoma Service, Birmingham Children's Hospital, 0121 333 9465
If you would like to talk about this issue with us please contact a support worker.