Dr Helen Jenkinson, a Consultant Paediatric Oncologist at Birmingham Children’s Hospital, highlights what retinoblastoma survivors and their families can expect from their long term follow up team…

Every year in the UK around 40-50 children are diagnosed with retinoblastoma. Increasing public and professional awareness and advances in treatment, together with a centralised approach to care has resulted in excellent long term outcomes, with a 98% survival rate.

As a result, there is a growing population of over 2,000 survivors of retinoblastoma in the UK, whose care and support, along with other survivors of childhood cancer, has become a priority area within the NHS through the government’s cancer reform strategy.

On finishing treatment for retinoblastoma

After treatment, children with retinoblastoma will initially be followed up by their Rb multidisciplinary team. At the end of treatment, feelings of relief and pleasure can be mixed with uncertainty and worry. A detailed summary of the treatment received with a clear plan of the care to expect over the coming months and years can help lessen some of these worries.

The care plan will outline the purpose of follow up appointments, how frequently they will occur and who you can expect to see. If you are not offered an end-of-treatment summary and care plan then ask your team to provide one.

Immediately after treatment, the focus of follow up is to:

• check for retinoblastoma coming back in previously treated areas within the eye.
• check for signs of new tumours developing.
• ensure there is no evidence of retinoblastoma having spread outside the eye.

During these early months you will have access to your Rb key worker who should be available to answer queries and provide continuing support and guidance.

In the longer term

With time, the risk of the tumour recurring either within the eye or elsewhere in the body falls and there is a shift in the focus of long term follow up.

The emphasis moves towards monitoring for the late effects of treatment (chemotherapy and/or radiotherapy) and ensuring good health in the future, particularly in those patients with heritable retinoblastoma.

During this period, your care is likely to transfer from the Rb team in London or Birmingham to a late effects team, who have expertise in long term care and who are likely to be based in your local paediatric oncology centre.

This may happen from as early as one year from finishing treatment up to 16 years, depending on local arrangements. The focus of long term follow up is:

Monitoring for the side effects of chemotherapy

The chemotherapy drugs used to treat retinoblastoma have a range of side effects, some of which may occur many years after finishing treatment. These should have been discussed with you at the start of treatment.

There are national recommendations for investigating these long term side effects which include:

  • Carboplatin: possible effect on kidney function and hearing.
  • Etoposide: secondary leukaemia, which is a rare occurrence but generally within five years of completing treatment.
  • Vincristine: possible nerve damage.

Monitoring for the side effects of radiotherapy

Radiotherapy has a number of potentially challenging long term side effects and newer treatments are being developed in order to try and limit its use. However, it is an effective treatment for retinoblastoma and was the mainstay of treatment for many years – a significant number of people have retained their eyes and vision thanks to its use.

Long term complications can be cosmetic, hormonal or related to second cancers and the focus of follow up is to identify and treat these promptly.

Lifestyle advice and health promotion

One of the priorities in long term follow up care is to encourage and support young people to be aware of their own health risks and to minimise these through a healthy approach to life.

Patients who are known to have the heritable form of retinoblastoma carry an alteration in their Rb gene which predisposes them to other cancers later in life, this is not the case for patients with non-heritable or non-genetic Rb.

Although this can be a difficult and worrying discussion for many young people, it is important to remember that our current knowledge is based on patients treated many years ago and we hope that through patient education, alteration to treatment and healthy lifestyle choices it may be a very different story in another 40 years.

A recent paper published in the British Journal of Cancer studied second cancers in patients treated in Britain since 1951. This paper confirms the risk for patients with heritable retinoblastoma and identifies certain cancers which occur most frequently.

These are bone and soft tissue tumours, melanoma of the skin and cancers of the bladder, uterus and lung. Many of these cancers are associated with the damaging effects of UV-light or tobacco.

By raising awareness in young people, encouraging them to avoid smoking and limit their exposure to UV-light by careful use of high SPF sunscreen, these risks could be significantly reduced in the future.

For this reason, patients with heritable retinoblastoma should remain under the supervision of a late effects team lifelong. This does not always mean regular hospital appointments but patients should have access to a key worker, know how to contact the late effects team if needed and understand the risks in order to make their own lifestyle choices.

Adulthood after retinoblastoma

As young people become adults their care is likely to move to a late effects team within adult services. The process of transferring care from paediatric to adult services is called transition and will be offered to all patients who need it.

The need for ongoing care into adult life is tailored to each individual patient depending on their genetic status and the treatment they received.

These are the typical long term follow up outcomes following differing treatments for Rb:

Surgery alone and non-heritable retinoblastoma

• Follow-up during childhood.
• Discharge at 16-18 years to GP care.
• Access to late effects team if required in the future.

Chemotherapy and non-heritable retinoblastoma

• Follow-up during childhood.
• Transfer to adult late effects team at 16–18 years.
• Eventual discharge to GP led care and self-management.

Heritable retinoblastoma or treated with radiotherapy

• Follow up during childhood.
• Transfer to adult late effects team at 16–18 years.
• Remain under supervision of adult late effects team lifelong.

There has been a welcome shift in the provision of long term follow up care for retinoblastoma and providers have the challenge of ensuring that each survivor receives high quality, individualised aftercare, which will enable them to achieve their full potential in life. The speed and extent to which this is achieved will depend on time, resource and both professional knowledge and public empowerment.

This article first appeared in the summer 2014 edition of our InFocus newsletter. If you’d like to receive the newsletter you can become a member of CHECT (it’s free). You can also see some recent editions of InFocus on our publications page.