Paediatric, teenage and young adult cancers

Published on 09/04/2015 by admin

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Last modified 09/04/2015

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22 Paediatric, teenage and young adult cancers

HM. Hatcher

Introduction

Cancers in this group of patients present specific challenges in diagnosis, treatment, recruitment to clinical trials and survival. Childhood cancers have seen a significant improvement in survival in the last 30 years from less than 30% to greater than 70% long-term survival. Unfortunately the same is not the case in the teenage and young adult cancers (TYA) with very little improvement seen over the same time period.

In addition they have particular social, educational, developmental and psychological needs which, if not addressed, have long-term consequences for the patient and their family. Those who survive cancer at a young age may also be left with significant medical late effects which need to be recognized and managed (p. 58).

Incidence/epidemiology

The incidence of malignancy in the UK is:

• 12.4 per 100,000 up to 15 years.
• 14.4 per 100,000 in those 15–19.
• 22.6 per 100,000 in those 20–24.

Those aged 14–24 account for 0.5% of all cancer registrations in the UK. In a study from the north-west of England the incidence was 174 cases per million with a male : female ratio of 1.22 : 1. In the same study the incidence was found to be increasing, particularly for bone tumours, testicular tumours, thyroid cancer and malignant melanoma.

Cancer is the most frequent natural cause of death in this age group, second only to accidents.

• Male : female ratio is 1.2 : 1.

Aetiology

The majority of older adult cancers are related to specific risk factors such as smoking, whereas the majority of paediatric malignancies are thought to be developmental in origin. A number of syndromes are associated with childhood cancer (e.g. Down’s syndrome associated with leukaemia) but the majority arise without an underlying genetic predisposition (e.g. Li–Fraumeni and sarcomas). TYA malignancies fall between the two age extremes and may represent a late developmental malignancy or an early adult malignancy due to other factors, e.g. familial adenomatous polyposis. In most cases the aetiology is not known.

Types of cancer

The types of cancer vary not only between children and adults but as the age increases in certain age group bandings (Table 22.1).

Table 22.1 Distribution of cancer types according to age

Age 0–14 years Age 15–19 years Age 20–24 years
Leukaemia 35%
Brain tumours 24%
Lymphomas 12%
Wilms’ tumour 7%
Neuroblastoma 7%
Sarcomas 11%
Retinoblastoma 3%
Others 1%

Lymphoma 27%
Sarcomas 16%
Leukaemia 15%
Carcinomas 11%
Brain tumours 11%
Germ cell tumours 10%
Melanoma 6%
Others 4%
Lymphomas 24%
Carcinomas 21%
Germ cell tumours 17%
Melanoma 10%
Sarcomas 8%
Leukaemia 8%
Brain tumours 8%
Others 4%

In children leukaemias represent the most common malignancy with acute lymphoblastic leukaemia (ALL) being most frequent. Brain tumours also make up a significant group, but the incidence falls as a proportion of cases from childhood towards early adulthood. In the 15 to 19-year-olds, the most frequent malignancies are lymphomas, leukaemias and carcinomas (especially thyroid and nasopharyngeal). For the 20 to 24-year-olds, lymphomas remain the most common malignancy but with carcinomas (especially cervix, thyroid and breast) and germ cell tumours as next common. Osteosarcomas and germ cell tumours peak in late teenage and early adulthood.

Clinical features

The clinical features in children, teenagers and young adults are similar to those seen in other age groups with the same malignancies, but are often not recognized as such due to the rarity of malignancy in these age groups. In addition for children who are unable to explain symptoms clearly, the disease has usually progressed so that symptoms are obvious to their family. Delays in diagnosis are very common in the adolescent and young adult and can contribute to reduced survival at all ages.

In children warning signs may be due to localized disease, or the manifestations of disseminated disease are listed in Box 22.1.

Box 22.1
Warning signs of cancer in children

• Obvious lump or swelling around the head, neck, abdomen or elsewhere.
• Increasing and persistent pain in bone or joints, often leading to less use of the limb or limping.
• Pallor, especially if associated with unexplained bruising or bleeding.
• Worsening headaches, especially if associated with vomiting in the morning.

In the older child and young adult, the same warning features apply but also include additional signs specific to the different malignancies.

Diagnosis and delays

Diagnosis of malignancy currently follows the pathways for either paediatric or adult cancers. Paediatric cancers are diagnosed and treated in specialist principal treatment centres with some care shared with local hospitals. For TYA cancers, currently they may be seen in a paediatric or adult setting and treated according to those protocols. In the UK TYA cancer will soon be treated in specialized centres. This may mean referral to a specialist TYA centre or to an adult cancer specialist in a particular site of the body in a designated regional hospital with appropriate TYA facilities (Figure 22.1).

image

Figure 22.1 Referral for treatment of childhood and TYA malignancy.

Delays in diagnosis can occur in children if symptoms are occult, especially in very young children but are more common in the TYA group.

Specific childhood cancers

Some cancers are specific to children and young adults, or their treatment is different to that in adults. These tumours will be highlighted below. For other malignancies, covered by other chapters, only specific changes in management for children and young adults will be made.

Leukaemias

In childhood acute lymphoblastic leukaemia (ALL) is by far the most frequently occurring leukaemia representing over 75% cases. (p. 308).

Presentation is usually with symptoms and signs of bone marrow infiltration and these can appear to occur quite rapidly, although are often preceded by several weeks of general malaise or recurrent infections and fevers. Usual features at the time of diagnosis are pallor, abnormal bruising or bleeding, bone pain (which may manifest as limping in young children) and on clinical examination lymphadenopathy and hepatomegaly in some children.

Diagnosis is suspected on the differential blood count but bone marrow examination is essential to confirm the diagnosis (p. 308), to perform immunocytochemical stains and cytogenetics which are of diagnostic and prognostic significance (Box 22.2).

Box 22.2

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Cancer in pregnancy Cancers of unknown primary Clinical trials in cancer Cancers of the genitourinary system Cancers of the musculoskeletal system Cancers of the haematopoietic system