While cancer in children, teenagers and young adults (CTYA) is rare, it is a significant cause of mortality and morbidity and early detection is important. Approximately 120 children aged less than 15 years, and 180 young persons aged between 15 and 24 years, are diagnosed with cancer in Scotland each year. The youngest age group (0-4 years) accounts for 48% of all childhood cancers. The distribution of cancers in teenagers and young adults is different from that in both young children and adults. The two most commonly occurring cancers in childhood are leukaemia (acute lymphoblastic leukaemia) and central nervous system (mostly brain) tumours. The two most commonly occurring cancers in male teenagers and young adults are lymphoma and testicular cancer, and malignant melanoma and lymphoma in females in this age group.
No risk factor or familial susceptibility can be identified in most cases. However, genetic susceptibility is apparent in some cases with associated conditions (such as Down syndrome, familial adenomatous polyposis coli, neurofibromatosis, aniridia and Li Fraumeni syndrome). The absence of a family history should not delay further investigations.
Cancers in children, teenagers and young adults are frequently diagnosed late due to the non-specific nature of many of the symptoms. A useful resource for parents is the awareness card identifying warning symptoms and signs of childhood cancer produced by the Grace Kelly Ladybird Trust11. Many of the cancer specific guidelines in this document are relevant to all ages e.g. melanoma, brain & CNS, sarcoma, etc.
Some conditions specific to this age group need special mention:
| Lymphomas |
- Hodgkin’s lymphoma: approximately 30 new cases are diagnosed in children and young adults less than 24 years, in Scotland every year. Approximately 83% of these cases are aged between 15 and 24 years. Hodgkin’s lymphoma rarely occurs under the age of five. Its natural history may be long (months), and about a third of patients have systemic symptoms.
- Non-Hodgkin’s lymphoma: approximately 20 new cases are diagnosed in children and young adults less than 24 years, in Scotland every year. Approximately 70% of these cases are aged between 15 and 24 years. Non-Hodgkin’s lymphoma has a more rapid progression of symptoms than Hodgkin’s lymphoma.
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| Leukaemia |
- Approximately 55 new cases are diagnosed in children and young adults less than 24 years in Scotland every year (about 70% are aged under 15 and about 38% under five). This accounts for about 8% of people diagnosed with leukaemia, every year.
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| Neuroblastoma |
- The majority of children present with abdominal distension (with or without a palpable mass) and symptoms of bone marrow failure. Infants under one year of age may have localised abdominal or thoracic masses; very young infants, less than six months old, may have massive hepatomegaly and skin lesions.
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| Brain tumours |
- Brain tumours remain one of the most common causes of cancer-related death in children and people under 40 years. Approximately two fifths of deaths among children age less than 15 dying from cancer are due to CNS tumours.
- Headsmart12 is an online resource which presents evidence based detailed guidance in different age groups.
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| Wilms’ tumour (nephroblastoma) |
- Common presenting features include unexplained visible haematuria, or unilateral abdominal mass, with or without pain, in a well-child.
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| Bone tumours |
- Osteosarcoma: can occur at any age although approximately 60% present in the second decade of life. Most common sites are femur, tibia and humerus.
- Ewing’s sarcoma: peak incidence is between 10 and 15 years. Ewing’s sarcoma rarely occurs under the age of five years or over the age of 30 years. Most commonly affected sites are the pelvis, femur, tibia, fibula, rib and humerus.
- Sarcomas of the long bones are usually excluded by normal X-ray but further investigation may be required for spine, pelvis, ribs or scapula.
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| Retinoblastoma |
- Common symptoms of retinoblastoma include white or absent pupillary red reflex and squint.
- There is a family history in approximately 15% of cases.ary red reflex and squint. There is a family history in approximately 15% of cases.
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| Gonadal tumours |
- Testicular cancer is one of the most common cancers in male teenagers and young adults.
- Testicular or other scrotal masses can be difficult to differentiate – any non transilluminable mass associated with the testis is significant.
- Ovarian tumours can be associated with precocious puberty.
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11 Grace Kelly Ladybird https://www.gp-update.co.uk/SM4/Mutable/Uploads/pdf_file/Cancer-presenting-symptoms-of-children-with-cancer-December-2021.pdf.
12 Headsmart https://www.headsmart.org.uk/
General considerations
- Consider referral for any patient with repeat presentations (three or more times) of any symptoms which do not appear to be resolving or following an expected pattern, taking into account parental or carer and patient concern
- In a child where symptoms and signs do not clearly fit with these guidelines, but nevertheless lead to concern about excluding cancer, the referrer should consider discussing the case with a senior paediatric colleague at their earliest convenience.
Urgent suspicion of cancer referral
- Unexplained petechiae or purpura is always an indication for emergency referral
- Unexplained fatigue, persistent pallor, failure to thrive or weight loss
- Any new persistent unexplained pain, particularly back pain or nocturnal pain
- Unexplained abdominal mass or distension
- Unexplained visible haematuria
| Bone pain, especially if: |
- diffuse or involves the back
- persistently localised at any site
- nocturnal pain
- limping
- requiring analgesia, or
- limiting activity
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| Lymphadenopathy, if: |
- non-tender, firm/hard and greater than 2cms in maximum diameter
- progressively enlarging
- associated with other signs of general ill health, fever or weight loss
- involves axillary nodes (no local infection or dermatitis) or any supraclavicular lymphadenopathy
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| Headache, if increasing in severity or frequency and: |
- worse in the morning or causing early wakening or
- associated with vomiting or any new neurological signs
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| Any new neurological signs, (such as weakness, loss of balance, etc.) especially if: |
- associated with behavioural change or deterioration in normal daily or school performance
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| Other possible signs of brain tumours: |
- increasing head circumference
- failure of fontanelle closure
- abnormal head position such as wry neck, head tilt or stiff neck
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| Soft tissue mass, if: |
- shows rapid or progressive growth
- size greater than 2cm maximum diameter
- deep to fascia, fixed or immobile, regardless of size
- recurrence after previous excision of sarcoma
- associated with regional lymph node enlargement
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| Eyes: |
- any new squint, if associated with headache or other neurological signs (otherwise consider optometrist and ophthalmology assessment)
- change in pupillary red reflex to absent or white
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Primary care management
- X-ray if there is unexplained bone pain of:
- increasing severity
- persistent
- tender
- non-mechanical bone pain particularly if disturbing rest or sleep
- If symptoms persist but X-ray is normal, repeat X-ray (after discussion with a radiologist) and consider referral, especially if the patient presents 3 or more times.
- Spontaneous or minor trauma fracture should raise suspicion of bone cancer.