- 1 Introduction
- 1.1 Purpose of this guidance
- 1.2 Population covered
- 1.3 The need for practice guidance
- 1.4 Statement of intent/disclaimer
- 1.5 Remit (Scope)
- 1.6 Development and review of the guidance
- 1.7 References
This guidance has been developed for health professionals involved in the management of adolescents and young adults (AYAs) diagnosed with cancer.
If you are an AYA cancer patient, survivor or family member seeking advice about the information discussed in this guidance, we have provided links to patient information and websites.
Purpose of this guidance
This guidance is intended to help general practitioners (GPs) and other primary health care providers in Australia:
- Recognise symptoms and signs that could be caused by cancer in their AYA patients
- Make decisions about appropriate investigations
- Make a timely and appropriate referral of AYAs with suspected cancer.
This guidance is applicable to all AYAs – aged between 15 and 24 years of age – presenting with symptoms and/or signs suggestive of cancer.
The guidance does not cover individual cancers in considerable detail as this would require a diagnosis to have been made and existing guidelines already include this information (see resources). When individual cancers are discussed, the guidance focuses on common cancers in young people. AYAs may present with paediatric tumours such as Neuroblastoma and Wilm’s tumour, and true adult tumours like breast; however these are very rare.
Common types of cancer diagnosed in AYAs include leukaemia, lymphoma, brain and central nervous system (CNS) tumours, bone tumours, soft tissue sarcoma, germ cell tumours (including testicular cancer), thyroid cancer and melanoma. Melanoma, gonadal germ cell cancer, Hodgkin lymphoma and thyroid cancer represent about half of all cancers diagnosed in Australians aged 15-29; however mortality in this age group is greatest from brain tumours.
Relative survival in AYAs is highest for thyroid cancer, followed by gonadal germ cell cancer, Hodgkin lymphoma and melanoma. Significant increases in survival from most cancers in AYAs have been seen recently, especially for leukaemia. However, the relative improvement in 5-year cancer survival has been greater among children (aged 0-14) and older adults (aged 30-39) than AYAs. AYAs with cancer have also benefited less from therapeutic advances than other age groups, likely due to low participation in clinical trials.
The need for practice guidance
- GPs are highly accessible and can facilitate patient access to health and support services. Most GPs will only encounter a few new patients with malignant cancer each year. Cancer is uncommon in AYAs and many GPs would not expect to consider cancer as a possible diagnosis in this age group. However, cancer has a high burden of disease and injury and is responsible for around 8.5 years of life lost in 15-24 year olds. Early detection and prompt referral to appropriate specialists can optimise outcomes and survival.
- Many AYA cancers present with vague or common symptoms and signs which may be attributed to fatigue, stress, growing pains or other causes. As many as three quarters of AYAs have reported a delay in a cancer diagnosis with 57% of AYA patients visiting their GP three or more times before they were referred to a hospital. Brain tumours and sarcomas appear to have the greatest delays in identification by GPs.
- AYAs often ignore symptoms or delay seeing a GP because they think it’s not serious or fear a cancer diagnosis. Embarrassment may also delay presentation in this age group. Young people may also be concerned about lack of privacy and confidentiality when seeing a GP. Many AYAs don’t see a GP regularly and may give an incomplete history because they are unaware of or are not asked about relevant personal or family history.
- Some GPs may not be skilled or confident in communicating with AYAs. It is important for GPs to be able to engage with young people and have an appreciation of how their age and stage of life may impact their distress levels, self-esteem, family dynamics, need for information and communication, peer relationships, self-identity, body image, sexuality and other components of the cancer experience. GPs are well placed to overcome barriers to care for AYAs by making their services and consultations youth friendly.
- Referral pathways for AYAs with suspected cancer are often not defined, or are unknown to GPs. GPs may be unsure whether to refer AYAs to paediatric or adult oncologists. Time to diagnosis is greater among adolescents compared to children, with delays more prominent among AYAs referred to adult cancer centres than among those referred to children’s hospitals.
|Youth Cancer Services provide a streamlined referral pathway for AYAs with cancer throughout Australia. See resources for further information.|
Statement of intent/disclaimer
This guidance is a general guide to appropriate practice and potential options for the early detection, investigation and referral of suspected cancers. It is understood that GPs will use this guidance along with their own clinical judgement. Patient management must be personalised in consultation with the patient and their caregivers.
Our aim is to provide guidance that is succinct, simple, practical and evidence-based. Recommendations are based on consensus of an expert panel and exhaustive review of the international literature, current best practice and/or existing guideline evidence.
The guidance has been developed for Australian practice and is applicable nationally, subject to the need for local protocols and referral pathways for all health professionals involved in AYA cancer management.
This guidance provides information and recommendations targeted to GPs about five key areas:
- Communicating effectively with AYAs
- Referral of AYAs with suspected cancer in an appropriate and timely manner
- Symptoms and signs of cancer and challenges in detecting cancer
- Clinical assessment of suspected cancer
- Individual cancer risk factors
The guidance is intended for GPs managing AYA patients that have presented with a symptom and/or sign suggestive of cancer. Cancer prevention and screening and management of the patient beyond referral to a specialist are outside the scope.
Development and review of the guidance
This guidance has been produced by the Clinical Oncological Society of Australia (COSA) as part of a national Youth Cancer Networks Program project funded by the Australian Government.
COSA convened a working group of multidisciplinary health professionals with expert knowledge of the areas covered and a patient representative with personal experience to develop the guidance.
Guidance working group members
Dr Marianne B Phillips MBCHB, FRCP, FRCPCH, FRACP (Chair)
Consultant Paediatric & Adolescent Oncologist and Palliative Care Specialist
Princess Margaret Hospital for Children
Lead Clinician Adolescent & Young Adult Collaborative, WA Cancer & Palliative Care Network
Dr Antoinette Anazodo MBBS, MRCPCH, MSc, Dip Adolescent Cancer
Sydney Children's Hospital
Director of AYA Cancer Services SESIAH
Prince of Wales Hospital
Ms Michelle Bosworth
Dr Melissa Jessop MBBS, FRACP, Post Grad Cert in Care of Teenagers and Young Adults with Cancer
Paediatric and Adolescent Oncologist
Queensland Children's Cancer Centre
Dr Carolyn Kefford MBBS, FAC Psych Med, DipEating Disorders (Shared Care)
Lecturer, Discipline of General Practice
Sydney Medical School
The University of Sydney
Dr Sofia Khan MBBS, FRACGP
GP Consultant and Family Physician
Dr Pandora Patterson BSocSc(Hons), PhD, MAPS
National Research and Evaluation Manager
Associate Professor Lena Sanci MBBS, PhD, FRACGP
Deputy Head, Department of General Practice
The University of Melbourne
Professor Susan Sawyer MBBS, MD, FRACP, FSAHM
Director, Centre for Adolescent Health
Royal Children's Hospital
Chair of Adolescent Health, Department of Paediatrics
The University of Melbourne
Guidance development team
Dr Hayley Griffin (Writer/editor)
Ms Alice Winter-Irving (Literature review)
Project Officer, Clinical Guidelines Network
Cancer Council Australia
The first draft was written by Dr Hayley Griffin, Dr Antoinette Anazodo, Dr Pandora Patterson and Dr Sofia Khan. Revisions were made by Dr Hayley Griffin.
Additional assistance from Ms Lisa Herron and Mrs Rhonda DeSouza is acknowledged.
The working group followed a process based on the National Health and Medical Research Council (NHMRC) recommendations for developing clinical practice guidelines, but modified to suit our purpose and format (succinct and web-based only).
The working group members considered the results of a literature search, and deemed existing guideline evidence (from comprehensive literature reviews) sufficient to develop recommendations in this guidance. Relevant recommendations from existing guidelines were modified to suit the purpose of this guidance (including the Australian context) and graded according to the NHMRC grades. A review of the current literature was also undertaken to ensure the information was up-to-date.
The draft guidance was widely disseminated and promoted to target users (including consumers), identified experts and other stakeholders for public consultation during a four week period. The working group reviewed the comments received and amended the guidance accordingly.
COSA will monitor for new evidence related to the areas covered by this guidance and update or amend it as required.
Process of developing AYA clinical guidance
- Working group established to confirm and advise on the purpose and scope of the guidelines, target audience, issues and interventions to be considered and desired outcomes.
- Systematic literature review and review of existing guidelines, resources and other relevant documents.
- Working group reviewed evidence and provided advice re content and structure of the guideline.
- Working group drafted the guideline content, with assistance of writer/editor.
- Working group members reviewed content and developed recommendations.
- Draft guidance posted on Cancer Council Australia's Wiki platform, that is hosting COSA's AYA guidances, and made available to a broader group of interested individuals and organisations for review and consultation.
- Working group and writer reviewed comments and amended guidance as required.
- Guidance submitted to Australian Government Department of Health and Ageing for review.
- Working group members provided advice regarding dissemination and promotion of the guideline and developed strategy for evaluation and revision.
- Guidance published (made live on COSA website).
A literature search was conducted in June 2011 for the following clinical questions (developed by the working group):
- In patients attending primary care services with symptoms indicative of (specified) cancer, which factors are associated with delayed referral? Which factors influence delay by patient and which delay by provider?
- Are there other barriers to referral of AYAs with suspected cancer and if so how can these be addressed?
- In patients presenting to primary care, what signs, symptoms and clinical features are predictive of cancer (general and specific cancers)?
- Given that the same initial symptoms and signs of cancer in AYAs may occur with other less serious conditions, how can primary health care professionals distinguish those who may have cancer with those who do not?
- What is the best way to clinically assess an AYA presenting with symptoms and/or signs that could be due to (specified) cancer?
- What are the major known risk factors for (specified) cancer?
Search inclusion criteria included:
- Cancer (general and specific: leukaemia, lymphoma, brain tumours, bone tumours, soft tissue sarcomas, testicular cancer, thyroid cancer and melanoma)
- Appropriate study design: meta-analyses, randomised controlled trials, nonrandomised clinical trials, systematic reviews, clinical trials phase I-IV, clinical practice guidelines, comparative studies, evaluation studies, cohort studies, retrospective analyses, case control studies, case series studies, non-experimental descriptive studies, qualitative surveys and interviews, expert opinion (i.e. consensus recommendations) and other evidence based documents
- Adolescents and young adults with cancer aged 15-24
- Published in English.
Databases searched included PubMed, Embase, Cochrane Library, National Guideline Clearinghouse, National Comprehensive Cancer Network, Canadian Medical Association Clinical Practice Guidelines and Scottish Intercollegiate Guidelines Network. In addition hand searching of journals, PubMed related citations and reference lists of review papers was performed.
Grading of recommendations
In developing the recommendations in this guidance, the working group has 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. It indicates to users the likelihood that, if that recommendation is implemented, the predicted outcome will be achieved. So, NHMRC grade A and B recommendations are generally based on a body of evidence that can be trusted to guide clinical practice, whereas Grade C and D recommendations must be applied carefully to individual clinical and organisational circumstances and should be followed with care.
Given the low volume and/or quality of evidence relating to some of the questions this guidance addresses, several important practice recommendations for which there is not, nor is there likely to be, any research evidence were identified. The working group considered these aspects of management to be ‘such sound clinical practice that nobody is likely to question it’ or ‘clinical common sense’. These are identified as Practice Points (PP).
|A||Body of evidence can be trusted to guide practice|
|B||Body of evidence can be trusted to guide practice in most situations|
|C||Body of evidence provides some support for recommendation(s) but care should be taken in its application|
|D||Body of evidence is weak and recommendation(s) must be applied with caution|
|PP||A ‘practice point’ – where there is no evidence, but it is considered ‘clinical common sense’.|
- Australian Institute of Health and Welfare (AIHW). Cancer in adolescents and young adults in Australia. Cancer series number 62. Canberra: AIHW; 2011.
- Bleyer A, Budd T, Montello M. Adolescents and young adults with cancer: the scope of the problem and criticality of clinical trials. Cancer 2006 Oct 1;107(7 Suppl):1645-55 Abstract available at http://www.ncbi.nlm.nih.gov/pubmed/16906507.
- Chown P, Kang M, Sanci L, Newnham V, Bennett DL. Adolescent Health: Enhancing the skills of General Practitioners in caring for young people from culturally diverse backgrounds, GP Resource Kit 2nd edition. Sydney: NSW Centre for the Advancement of Adolescent Health and Transcultural Mental Health Centre; 2008.
- McAvoy BR. General practitioners and cancer control. Med J Aust 2007 Jul 16;187(2):115-7 Abstract available at http://www.ncbi.nlm.nih.gov/pubmed/17635097.
- Begg S, Vos T, Barker B, Stevenson C, Stanley L, Lopez A. The burden of disease and injury in Australia 2003. Canberra: AIHW; 2007.
- Miedema BB, Easley J, Hamilton R. Young adults' experiences with cancer: comments from patients and survivors. Can Fam Physician 2006 Nov;52(11):1446-7 Abstract available at http://www.ncbi.nlm.nih.gov/pubmed/17279205.
- Smith S, Davies S, Wright D, Chapman C, Whiteson M. The experiences of teenagers and young adults with cancer--results of 2004 conference survey. Eur J Oncol Nurs 2007 Sep;11(4):362-8 Abstract available at http://www.ncbi.nlm.nih.gov/pubmed/17287143.
- Mavinkurve-Groothuis AM, Hoogerbrugge PM, Meijer-Vandenbergh EM, Brouwer HG, van Enk JG. Patient delay due to embarrassment and fear in two adolescents with a malignant tumor. Pediatr Blood Cancer 2008 May;50(5):1083 Abstract available at http://www.ncbi.nlm.nih.gov/pubmed/18240169.
- Adolescent and Young Adult Oncology Progress Review Group. Closing the Gap: Research and Care Imperatives for Adolescents and Young Adults with Cancer. Bethesda: USA National Cancer Institute; 2006.
- Palmer, S. Improving care for AYA patients treated within adult hospitals: what can be done right now? Cancer Forum 2009;33(1):22-25 Abstract available at http://www.cancerforum.org.au/Issues/2009/March/Forum/Improving_care_for_AYA_patients.htm.
- Klein-Geltink J, Shaw AK, Morrison HI, Barr RD, Greenberg ML. Use of paediatric versus adult oncology treatment centres by adolescents 15-19 years old: the Canadian Childhood Cancer Surveillance and Control Program. Eur J Cancer 2005 Feb;41(3):404-10 Abstract available at http://www.ncbi.nlm.nih.gov/pubmed/15691640.
- National Collaborating Centre for Primary Care. Referral Guidelines for Suspected Cancer. Clinical guideline 27. London: National Institute for Health and Clinical Excellence; 2005.
- New Zealand Guidelines Group. Suspected cancer in primary care: guidelines for investigation, referral and reducing ethnic disparities. Wellington: New Zealand Guidelines Group; 2009.