Summary of recommendations and practice points

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Early detection of cancer in AYAs > Summary of recommendations and practice points

The working group modified relevant recommendations from existing guidelines[1][2] to suit the purpose of this guidance (including the Australian context) and assigned NHMRC grades of recommendation to assist users to distinguish between those based on strong evidence, and those based on weak evidence. The grade does not indicate the importance of the recommendation, but reflects the strength of the evidence supporting it.

Given the low volume and/or quality of evidence relating to some of the questions this guidance addresses, the working group identified several important practice recommendations for which there is not, nor is there likely to be, any research evidence. These are identified as Practice Points (PP).

Communicating effectively with AYAs

Practice point(s)
  • GPs play an important role in cancer management. GPs should engage, guide and support adolescents and young adults (AYAs) with cancer throughout the entire journey and bring together recommendations and advice from other health professionals and specialists and make sense of it to the young person and their family.
  • The GPs role can be most effectively achieved through excellent communication skills which take into account the developmental stage of the young person, their cultural background and which include using appropriate language.
  • Youth-friendly care principles GPs can apply include:
    • Treating the whole person with the problem and avoiding a problem focus alone - this means exploring how the patient is fairing in all aspects of their life
    • Maintaining youth-friendly principles across the whole practice - with reception and nursing staff and including waiting room atmosphere
    • Ensuring that reception staff explain “Medicare” and the obtaining of “Medicare cards” at age 15 to all AYAs
    • Outlining confidentiality (and its exceptions) at the beginning of the consultation
    • Explaining what is happening and why, and what to expect when accessing cancer services to which they might be referred
    • Finding time to talk with the young person on their own and balancing this with involving family support as appropriate
    • Understanding the implications of embarrassment for AYAs
    • Providing honest feedback about concerns
    • Involving the young person in the management plan
    • Being flexible, open, honest and non-judgmental - this includes creating space for questions and providing answers to commonly asked questions
    • Providing youth-friendly resources and resources to support families
    • Offering information about support groups.

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Referral of AYAs with suspected cancer

Recommendation Grade
Once the decision to refer has been made, GPs should make sure that the referral is made within 1 working day.
D
Recommendation Grade
GPs should include all appropriate information in referral correspondence, including whether the referral is urgent or non-urgent.
D


Practice point(s)

GPs should consider telephone contact with the specialist when there is a high suspicion of cancer.

  • GPs should ensure that patients are informed regarding expected referral time frames, for example:
  • Receiving an acknowledgment of the referral
  • Being seen by a specialist.

Patients should contact their GP or specialist services if there appears to be a delay in accessing specialist appointments.

  • GPs should be aware of patient and practitioner factors that may delay referral.

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Symptoms and signs of AYA cancer

Recommendation Grade
Diagnosis of cancer on clinical grounds alone can be difficult. GPs should be familiar with common symptoms and signs of typical AYA cancers, and be able to readily identify these features when AYAs consult with them.
D
Recommendation Grade
AYAs who present with symptoms and/or signs of cancer should be referred urgently to a paediatric oncologist or Youth Cancer Service, depending on local arrangements.
D
Recommendation Grade
AYA cancers may present initially with symptoms and/or signs associated with common conditions. AYAs presenting several times (three or more) with the same problem, who are clinically unwell, but with no clear diagnosis, should be considered for urgent referral to a specialist.
D


Practice point(s)

The need to consider action increases as the number of presenting symptoms and/or signs increases. Consider that combinations of symptoms and/or signs can have a higher predictive value than a single sign or symptom in isolation. However, a single symptom and/or sign should not be overlooked when considering a cancer diagnosis.

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Clinical assessment of AYAs with suspected cancer

Recommendation Grade
Investigations by GPs should not delay referral. In patients with less typical symptoms and/or signs initial investigations may be necessary, but should be undertaken straightaway to avoid delay. If specific investigations are not readily available locally, an urgent specialist referral should be made.
D
Recommendation Grade
The presence of any of the following symptoms and signs requires consideration of investigation with full blood count (FBC):
  • Pallor
  • Fatigue
  • Irritability
  • Unexplained fever
  • Persistent or recurrent upper respiratory tract infections
  • Generalised lymphadenopathy
  • Persistent or unexplained bone pain (and X-ray)
  • Persistent back pain
  • Unexplained bruising.


C


Practice point(s)

AYAs presenting with symptoms and/or signs suggestive of cancer should be assessed through a detailed history and examination, irrespective of the presence of risk factors.

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Individual AYA cancers

Leukaemia

Recommendation Grade
AYAs should be referred immediately to a specialist if they have unexplained petechiae or hepatosplenomegaly.
C
Recommendation Grade
The presence of one or more of the following symptoms and signs requires consideration of investigation with FBC and blood film:
  • Pallor
  • Fatigue
  • Unexplained irritability
  • Unexplained fever
  • Persistent or recurrent upper respiratory tract infections
  • Generalised lymphadenopathy
  • Persistent or unexplained bone pain
  • Unexplained bleeding or bruising.


If the FBC or blood film indicates leukaemia then an urgent specialist referral should be made.

C

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Lymphoma

Recommendation Grade
AYAs presenting with hepatosplenomegaly should be referred immediately to a specialist.
C
Recommendation Grade
AYAs with a mediastinal or hilar mass on chest x-ray should be referred immediately to a specialist.
C
Recommendation Grade
AYAs should be referred urgently to a specialist if they have lymphadenopathy with one or more of the following, particularly if there is no evidence of local infection:
  • Non-tender, firm or hard lymph nodes
  • Lymph nodes greater than 2 cm in size
  • Progressively enlarging lymph nodes
  • Other features of general ill-health, fever or weight loss
  • Axillary node involvement (in the absence of local infection or dermatitis)
  • Supraclavicular node involvement.

AYAs should be referred immediately to a specialist if they have shortness of breath in association with the above signs; particularly if the shortness of breath is not responding to bronchodilators.

C

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Brain tumours

Recommendation Grade
Persistent headache in AYAs requires a neurological examination by the GP.
D
Recommendation Grade
AYAs presenting with recurrent headache and vomiting that cause early morning waking or occur on waking (signs of raised intracranial pressure) should be referred immediately to a specialist.
C
Recommendation Grade
The presence of any of the following neurological symptoms and signs in AYAs should prompt urgent or immediate referral:
  • New-onset seizures or fits
  • Cranial nerve abnormalities
  • Visual disturbances
  • Gait abnormalities
  • Motor or sensory signs
  • Deterioration in intellectual capacity
  • Unexplained behavioural and/or mood changes.


D
Recommendation Grade
An AYA presenting with a reduced level of consciousness requires emergency admission.
C

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Bone tumours

Recommendation Grade
AYAs presenting with symptoms suggestive of bone cancer should be referred to a sarcoma specialist affiliated with a Youth Cancer Service where available.
D
Recommendation Grade
AYAs with increasing, unexplained or persistent bone pain, tenderness or swelling, particularly pain at rest (and especially if not in the joint), persistent back pain or an unexplained limp should be investigated urgently by the GP including x-ray, and referred to a specialist if considered concerning.
C
Recommendation Grade
GPs should consider the possibility of a bone sarcoma in AYAs irrespective of history of an injury.
C
Recommendation Grade
GPs should consider primary or secondary bone cancer in AYAs with suspected spontaneous fracture and refer patients for an immediate x-ray (completed and reported within 5 days).
C
Recommendation Grade
If an x-ray indicates that bone cancer is a possibility, an urgent specialist referral should be made.
C
Recommendation Grade
If the x-ray is normal but the patient remains symptomatic, the x-ray should be repeated and the patient referred to a specialist. Other investigations may be ordered as appropriate.
C

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Soft tissue sarcoma

Recommendation Grade
AYAs presenting with symptoms suggestive of soft tissue sarcoma should be referred to a sarcoma specialist affiliated with a Youth Cancer Service where available.
D
Recommendation Grade
An urgent specialist referral should be made in AYAs presenting with an unexplained mass (at almost any site) that has one or more of the following features:
  • Deep to the fascia
  • Non-tender
  • Progressively enlarging
  • Associated with a regional lymph node that is enlarging greater than 2 cm in diameter.


C

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Testicular cancer

Recommendation Grade
AYAs with an unexplained swelling or mass in the body of the testis should be referred urgently to a specialist.
C
Recommendation Grade
An urgent ultrasound should be considered in AYAs with a scrotal mass that does not transilluminate and/or when the body of the testis cannot be distinguished.
D

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Thyroid cancer

Recommendation Grade
AYAs presenting with symptoms of tracheal compression including stridor due to thyroid swelling require immediate emergency department admission.
D
Recommendation Grade
AYAs presenting with a thyroid swelling and one or more of the following should be urgently referred to a specialist:
  • A solitary nodule increasing in size
  • A history of neck irradiation
  • A family history of an endocrine tumour
  • Unexplained hoarseness or voice changes
  • Cervical lymphadenopathy


D
Recommendation Grade
AYAs presenting with a thyroid swelling without stridor or any of the features indicated above should be referred by GPs for thyroid function tests. Patients with hyper- or hypothyroidism and an associated goitre are very unlikely to have thyroid cancer and could be referred to an endocrinologist. Those with goitre and normal thyroid function tests should be referred to an endocrinologist.
D
Recommendation Grade
In AYAs presenting with symptoms and/or signs suggestive of thyroid cancer, a referral for an ultrasound investigation may be made, but this should not delay referral to a specialist. Referral for isotope scanning is likely to result in unnecessary delay and is not recommended.
D

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Melanoma

Recommendation Grade
In the context of a high suspicion of melanoma, any one feature from the seven point checklist (see practice point below) is adequate to prompt urgent referral to a regional melanoma service; excision in primary care should be avoided.
C
Recommendation Grade
In the context of a low suspicion of melanoma, GPs should use the seven point checklist (see practice point below) to carefully monitor for change at the initial examination to be repeated at eight weeks.
D


Practice point(s)

GPs should use the seven point checklist (Table 1) in the assessment of pigmented lesions to determine referral. Suspicion is greater for lesions scoring 3 points or more (based on major features scoring 2 points each and minor features scoring 1 point each).

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References

  1. National Collaborating Centre for Primary Care. Referral Guidelines for Suspected Cancer. Clinical guideline 27. London: National Institute for Health and Clinical Excellence; 2005.
  2. New Zealand Guidelines Group. Suspected cancer in primary care: guidelines for investigation, referral and reducing ethnic disparities. Wellington: New Zealand Guidelines Group; 2009.

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