Paediatric oncology
The treatment of children with cancer requires the same general principles of approach to diagnosis, treatment and management as those utilised in adult oncology. However, many of the tumours respond more favourably to chemotherapy than adult cancers do and the goal of treatment is always survival. Chemotherapy and radiotherapy can have devastating long-term consequences in children; increasing their risk of further cancers in later life and limiting bone growth and intellect due to exposure of growing bone and developing brain to radiation. Such issues require careful explanation and consideration when planning treatment in patients of young age.
There are practical considerations when dealing with children, as they are generally not good historians and reliance is placed on the vigilance of parents and carers, which can result in a delayed presentation and hinder the progress of treatment. Children are often scared of hospitals, doctors and medical procedures, so painful procedures such as lumbar puncture or bone marrow aspirates are often carried out under general anaesthetic to minimise the distress. Sedation is often required for imaging such as CT or MRI.
The multidisciplinary approach is of great importance as it is not only the medical and psychological wellbeing of the child that must be addressed, but the ripple effect that a diagnosis of childhood cancer has on the whole family.
Teenager and young adult oncology
National standards for cancer services in the UK recognise that teenagers and young adults (TYA) require specific provision of care. More specifically the improvements in outcomes for TYA lag behind those seen in cancer treatment for the very young and very old. This is due to the differing medical and psychological needs of this group, under-representation of TYA in clinical trials, unique aetiologies and the challenge of coping at a time when they are facing difficult physical, emotional and social challenges of adolescence. This group of patients are often subject to late diagnosis and straddle the crossover of children’s (generic) and adult (site-specific) cancer services. As a result TYA units have been commissioned to address this shortfall in service provision.
Specific educational goals include smoking cessation, safe sun exposure, breast and testicular examination, healthy eating and drinking, advice regarding HPV infection and a general promotion of self-awareness of their own health.
Referral pathways are agreed in all cancer networks in the UK, where a primary treatment centre can provide a full range of sustainable services by appropriately trained staff. All care must be provided in age-appropriate facilities and patients must have unhindered access to facilities and support.
Age-appropriate care provides access to cancer expertise that is relevant to young people, where staff work with young people throughout the whole pathway, from bench to bedside. There should be access to peers, with at least some of their treatment happening with patients of a similar age. The care needs to be delivered in an environment that is sensitive to the needs of young people.
Patients aged 19 and over who decide to receive all of their treatment in local adult services should have an individualised treatment plan agreed by the local site-specific MDT and the TYA MDT at the designated primary treatment centre.
Aetiology
Childhood cancers are rare in the UK, with 1500 children diagnosed with cancer each year, representing only 0.5% of all cancers. Cancer still represents the second leading cause of death in children after accidental injury. The incidence of cancer under age 15 years is approximately 140 cases per million children, giving a 1 in 500 risk of developing cancer in childhood. Childhood cancer survival has improved for all types of tumour over the past 50 years, and almost eight out of every 10 children (78%) diagnosed with cancer now survive for 5 years or more.
The types, prevalence and categorisation of cancers affecting children vary noticeably from those seen in adults. Childhood cancers are classified by histology to identify their tissue of origin, rather than by primary tumour site. Table 53.1 outlines the common childhood cancers and their overall prevalence and demonstrates a difference in the prevalence of tumour type by sex; overall, boys are slightly more likely to develop cancer than girls by a ratio of approximately 6:5. The distribution of cancer in children also varies with age, as shown by Figure 53.1.
Haematological cancers
Leukaemia
Leukaemia accounts for almost a third of all childhood cancers and is predominantly acute leukaemia (ALL, 79%; AML, 15%). Acute lymphoblastic leukaemia (ALL) shows a peak incidence at around 2–3 years of age, while acute myeloid leukaemia (AML) is most common in infancy. Presentation is similar to that in adults, with common symptoms of general malaise, easy bruising, bleeding, fever, bone or muscular pain, headache, irritability, shortness of breath and increased incidence of infections. Diagnosis is made on FBC, blood film and bone marrow aspiration. Staging requires imaging and lumbar puncture (see Chapter 49).
Treatment requires intensive combination chemotherapy for all forms of acute leukaemia. Girls with ALL typically receive treatment for 2 years, while boys need 3 years of treatment as their risk of relapse is higher. The 5-year survival for ALL is 88% and AML 64%.
Lymphoma
These cancers are categorised into Hodgkin’s lymphoma and non-Hodgkin’s lymphoma (NHL). Hodgkin’s lymphoma constitutes about two-fifths of all childhood lymphoma and NHL can be subdivided into three main types in children: lymphoblastic, small non-cleaved-cell (Burkitt’s lymphoma) and large-cell lymphoma.
In children, the prevalence in boys is twice that of girls and the incidence increases with age (Figure 53.1). Children tend to present with painless swollen lymph nodes noticed as a lump, or by compression of structures such as the airways or bowel, presenting with breathlessness, persistent cough or bowel obstruction. Systemic features include fever, weight loss, diminished appetite, hepatosplenomegaly and night sweats. Diagnosis requires an excision biopsy of an affected lymph node, with bone marrow aspirate and trephine and CT imaging to determine the extent of disease and stage.
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