Suspected cancer (part 1—children and young adults): visual overview of updated NICE guidance
BMJ 2015; 350 doi: https://doi.org/10.1136/bmj.h3036 (Published 23 June 2015) Cite this as: BMJ 2015;350:h3036
Childhood cancers infographic
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Updated NICE referral guidelines for suspected childhood cancer: absence of appropriate stakeholder input has resulted in gaps in the guidance.
Murray MJ1, Joseph RV2, Dommett R3,4, Gamble A5, Bennison J6,7, Nicholson JC1,8.
Author affiliations:
1 Consultant Paediatric Oncologist, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK
2 Consultant Paediatrician, Luton and Dunstable Hospital, UK
3 Consultant Paediatrician in Haematology/Oncology, Bristol Royal Hospital for Children, Bristol, UK
4 Honorary Lecturer, University of Bristol, Bristol, UK
5 Chief Executive, Children's Cancer and Leukaemia Group (CCLG), University of Leicester, Leicester Royal Infirmary, Leicester, UK
6 General Practitioner, Edinburgh
7 Vice-Chair, Scottish Intercollegiate Guidelines Network (SIGN)
8 Chairman, Children's Cancer and Leukaemia Group (CCLG)
Correspondence: Dr James Nicholson; james.nicholson@addenbrookes.nhs.uk
Conflicts of interest: None
We read with interest the article by Hamilton et al [1], which provides an overview of the updated National Institute for Health and Care Excellence (NICE) recognition and referral guidelines NG12 for suspected cancer in children. The full guidance is available at http://www.nice.org.uk/guidance/ng12 [2]. Readily accessible practical guidance that may assist in the early recognition of signs and symptoms suggestive of possible malignancy, and which outlines appropriate referral pathways and timelines from primary care, is important, because cancer in children is rare [3]. Only ~1600 cases occur in patients <15 years of age in the UK per year, equating to just one child in 500 developing cancer [4]. The average general practitioner is therefore likely to see no more than a handful of such cases in their entire career [5]. Guidance for the referral of children with suspected cancer from primary care is therefore to be welcomed.
The original 2005 NICE referral document [6; now no longer available] provided pragmatic guidance specifically concerning children, based on individual tumour types. It included, for example, detail about when lymphadenopathy (a very frequent reason for referral) should represent cause for concern, advice regarding presentations of mediastinal masses, and what symptoms should alert physicians to the possibility of a childhood brain or spinal tumour. Moreover, it underlined the need to be mindful of children presenting repeatedly with the same symptoms and to make urgent contact with appropriate specialists in the event of concern. By implication, the two week wait process, acknowledged as a vital safety net for timely management of adults referred with suspected cancer, had little relevance for children - general practitioners and paediatricians alike would expect much more rapid responses to referrals where there was a suspicion of malignancy.
The authors of the 2015 revision have endeavoured to improve on the previous guidance, focusing on new evidence derived from primary care and using a symptom-based approach [2]. Unfortunately however, much of the crucial detail relating to some of the conditions with which children’s cancer specialists are familiar has been lost, resulting in significant gaps in the revised guidance. On the one hand, due to loss of clarity regarding signs and symptoms of the most aggressive presentations, those with life-threatening malignancies requiring urgent or emergency referral and management could be delayed; on the other, this lack of detail may lead to unnecessary referrals, due to higher levels of physician uncertainty, in turn causing unnecessary patient and parental anxiety. Some authors of this article (MJM, RVJ, JCN) have received anecdotal reports from one UK region that the latter situation is occurring already.
The revised referral guidelines reflect what appears to be a concerning trend within NICE to produce guidance without appropriate expert input at an early stage to their Guideline Development Groups (GDGs), particularly relating to children. In this case, NICE have relied on invitations for stakeholder comments during a short window and at the pre-final draft stage. Another recent example was the NICE quality standard for sarcoma (QS78) [7], which included young children in its remit but again included no paediatric input on its GDG. We believe that this approach, without the involvement of experts in the relevant fields, could adversely affect patient outcomes, completely counter to the very premise the guidelines were commissioned for in the first place.
The key tension in current guideline development methodology is the balance between rigour and pragmatism, specifically referring to the interface between methodologists and appropriate stakeholder involvement, respectively [8]. Recently, methodologists have been the main drivers of continuously increasing rigour in GDGs, despite the burden of this rigour falling upon stakeholders [8]. The authors contend that GDGs need to address the needs and concerns of all stakeholders affected by the guidelines, and argue for greater collaboration [8]. Unfortunately, in this critical regard, the current NICE guidelines for suspected cancer in children fall well short. They take a backwards step from the 2005 publication, in terms of the confidence general practitioners can have in their decisions to refer. The document is large and unwieldy; as a result, the relevant information for children’s cancer is difficult to find amongst the narrative. A separate, more concise document for children would have been infinitely more useful. In addition, the considerable knowledge and experience that lies within professional bodies and charities, such as the UK-based Children’s Cancer and Leukaemia Group (CCLG) and Teenage Cancer Trust (TCT), have not been recognised within the NICE framework until late consultation. Rather than review and extend the process in light of the extensive concerns and feedback received from key stakeholders at this pre-final draft stage, NICE proceeded to publish a document which did not adequately address the many issues raised. These recent examples highlight a potential need for a review of NICE processes and constitution of its GDGs to ensure the publication of documents that health professionals may have trust and confidence in.
In conclusion, we urge our general practitioner colleagues to continue to pick up the telephone and discuss with a paediatrician any cases where they have concerns about suspected cancer in children. This approach will both ensure appropriate and timely referral of those warranting emergency or urgent investigation, and avoid unnecessary referral of cases that should continue to be managed in primary care.
References
[1] Suspected cancer (part 1-children and young adults): visual overview of updated NICE guidance. Hamilton W, Hajioff S, Graham J, Schmidt-Hansen M. BMJ 2015;350:h3036.
[2] National Health Service. National Guidelines NG12. Recognition and referral guidelines for suspected cancer: http://www.nice.org.uk/guidance/ng12. June 2015. National Institute for Health and Care Excellence (NICE). Accessed 26th June 2015.
[3] Signs and symptoms of childhood cancer: a guide for early recognition. Fragkandrea I, Nixon JA, Panagopoulou P. Am Fam Physician. 2013;88:185-92.
[4] Web content: Cancer Research UK statistics. http://www.cancerresearchuk.org/health-professional/cancer-statistics/ch.... Accessed 26th June 2015.
[5] Diagnosing children in primary care – a realistic expectation? Feltbower RG, Lewis IJ, Picton S, Richards M, Glaser AW, Kinsey SE et al. Br J Cancer 2004;90:1882-4.
[6] National Health Service. Clinical guidelines CG27. Referral guidelines for suspected cancer. June 2005. http://www.nice.org.uk/CG027. National Institute for Health and Clinical Excellence (NICE). Website accessed 26th June 2015 – guidelines no longer available.
[7] National Health Service. Quality standard QS78 for sarcoma: http://www.nice.org.uk/guidance/qs78. January 2015. National Institute for Health and Care Excellence (NICE). Accessed 26th June 2015.
[8] When is good, good enough? Methodological pragmatism for sustainable guideline development. Browman GP, Somerfield MR, Lyman GH, Brouwers MC. Implement Sci. 2015;10:28.
Competing interests: No competing interests
Re: Suspected cancer (part 1—children and young adults): too much emphasis on positive predictive value resulting in a lack of clarity
We read the editorial by Hamilton et al (1) summarising the NICE guidance for suspected cancer in children and young adults with interest, but remain sceptical as to whether this is indeed an improvement on the 2005 guidelines. The entire document relies on positive predictive values (PPV) from a single nested case control study. Despite this being an important study and perhaps the only one addressing the risk of childhood cancer, it is surprising that the bulk of the guidance is based on this evidence alone. For instance, there is no mention of the RCPCH endorsed brain pathways guideline for children who may have a brain tumour (2). Also, in clinical practice, patients often have more than one symptom which helps the clinician estimate risk of cancer in a better way than by using PPV for individual symptoms. This has not been emphasised in the guideline.
The guideline has for the most part included paediatric cancers within adult cancer groups. We know that the type and severity of symptoms and the manner of presentation differ significantly between adults and children, particularly in children aged 0-4 years. The guideline lacks clarity regarding ‘red flag’ symptoms and pathways of referral, particularly regarding who the child is referred to (paediatrician or oncologist) and whether the patient and their families understand that the referral is being made for suspected cancer.
As one of the largest tertiary oncology centres in the UK, we have reviewed our referrals for suspected cancer over the last 4 years. We had 105 patients referred with suspected cancer with 7 being
diagnosed with cancer. All patients referred to our hospital are seen by an oncologist or haematologist, unlike in many other hospitals where they may be seen by a paediatrician. This in itself causes a lot of anxiety to the vast majority of patients who will have benign diagnoses but have to endure a visit to the oncology clinic. Disappointingly we found that the majority patients /parents were unclear as to why they had been referred to an oncologist, and the possibility of an underlying cancer had rarely been discussed at the point of referral.
The most significant symptoms included lymphadenopathy, pallor and bruising with cytopenias on blood count and unexplained abdominal mass. Referrals of clinically insignificant lymphadenopathy probably exemplify the deficiency of the current guidelines. In our series lymphadenopathy was the commonest cause of referral (44%), but only two patients were diagnosed with haematological malignancy and both had lymph node masses of over 5 cms.
In summary, as paediatric cancers are rare and their presentation can be varied, it is very important that referrals are discussed with the paediatrician/oncologist to improve the process of referral and the patient/family experience.
1. Hamilton W, Hajioff S, Graham J, Schmidt-Hansen M. Suspected cancer (part 1—children and young adults): visual overview of updated NICE guidance. BMJ. 2015;350. http://www.bmj.com/content/350/bmj.h3036.abstract.
2. Wilne S, Koller K, Collier J, Kennedy C, Grundy R, Walker D. The diagnosis of brain tumours in children: a guideline to assist healthcare professionals in the assessment of children who may have a brain tumour. Arch Dis Child . 2010. doi:10.1136/adc.2009.162057 .
Competing interests: No competing interests