Clinical presentation of childhood soft tissue sarcomas: a systematic review and meta-analysis.

BACKGROUND: Time to diagnosis (TTD) of childhood soft tissue sarcoma (STS) is significantly associated with survival. This review aims to identify pre-diagnostic symptoms/signs to inform earlier diagnosis interventions. METHODS: Medline, Embase, Cochrane and Web-of-Science were searched between January 2010 and February 2021 for studies including children (<18 years) diagnosed with STS, with no language restrictions. Pooled proportions of symptoms/signs were calculated and subanalysed by tumour location and age. RESULTS: Fifty-nine eligible studies were identified, totalling 2462 cases. The most frequent symptoms were lump/swelling (38%, 95% CI 27% to 51%), pain (6%, 95% CI 3% to 10%), cutaneous changes (4%, 95% CI 0 to 9%), localised eye swelling (3%, 95% CI 0 to 7%), cranial nerve deficits (2%, 95% CI 0 to 5%) and constitutional symptoms (2%, 95% CI 0 to 5%).Symptoms varied by location and age. Localised eye swelling (20%, 95% CI 3% to 45%), cranial nerve deficits (14%, 95% CI 4% to 28%) and impaired visual function (6%, 95% CI 0 to 17%) were frequent in head and neck tumours. For abdomen/pelvic tumours, urinary symptoms (24%, 95% CI 5% to 15%), abdominal distension/discomfort (22%, 95% CI 4% to 47%), genital lump/swelling (16%, 95% CI 1% to 42%), constitutional symptoms (9%, 95% CI 0%] to 23%), vaginal bleeding (7%, 95%C I 0 to 21%) and bowel habit changes (6%, 95% CI 0 to 17%) were frequent.In <5 years, consumptive coagulopathy (16%, 95% CI 0 to 48%), cutaneous changes (5%, 95% CI 0 to 40%), genital lump/swelling (4%, 95% CI 0 to 14%), reduced mobility (3%, 95% CI 0 to 11%), vaginal bleeding (2%, 95% CI 0 to 11%) and bleeding/bruising/petechiae (2%, 95% CI 0 to 20%) were frequent compared with lump/swelling, constitutional symptoms, pain and headaches which were frequent among >11 years. CONCLUSIONS: For STS, pre-diagnostic symptoms differ by age and location, highlighting the need to tailor early diagnosis interventions.

Assessing and investigating children with suspected bone and abdominal tumours: an e-Delphi consensus process.

BACKGROUND: The incidence of childhood cancer has risen by 15% since the 1990s. Early diagnosis is key to optimising outcomes, however diagnostic delays are widely reported. Presenting symptoms are often non-specific causing a diagnostic dilemma for clinicians. This Delphi consensus process was conducted to develop a new clinical guideline for children and young people presenting with signs/symptoms suggestive of a bone or abdominal tumour. METHODS: Invitation emails were sent to primary and secondary healthcare professionals to join the Delphi panel. 65 statements were derived from evidence review by a multidisciplinary team. Participants were asked to rank their level of agreement with each statement on a 9-point Likert scale (1=strongly disagree, 9=strongly agree), with responses ≥7 taken to indicate agreement. Statements not reaching consensus were rewritten and reissued in a subsequent round. RESULTS: All statements achieved consensus after two rounds. 96/133 (72%) participants responded to round 1 (R1) and 69/96 (72%) completed round 2 (R2). 62/65 (94%) statements achieved consensus in R1 with 29/65 (47%) gaining more than 90% consensus. Three statements did not reach consensus scoring between 61% and 69%. All reached numerical consensus at the end of R2. Strong consensus was reached on best practice of conducting the consultation, acknowledging parental instinct and obtaining telephone advice from a paediatrician to decide the timing and place of review, rather than adult cancer urgent referral pathways. Dissensus in statements was due to unachievable targets within primary care and valid concerns over a potential overinvestigation of abdominal pain. CONCLUSIONS: This consensus process has consolidated statements that will be included in a new clinical guideline for suspected bone and abdominal tumours for use in both primary and secondary care. This evidence base will be translated into awareness tools for the public as part of the Child Cancer Smart national awareness campaign.

Assessing Healthcare Professionals' Identification of Paediatric Dermatological Conditions in Darker Skin Tones.

The impacts of the lack of skin tone diversity in medical education images on healthcare professionals (HCPs) and patients are not well studied. The aim of this study was to assess the diagnostic knowledge of HCPs and correlate this with confidence and training resources used. An online multiple choice quiz was developed. The participants' demographics, training resources and self-confidence in diagnosing skin conditions were collected. The differences in the results between the subgroups and the correlations between the respondents' experience, self-reported confidence and quiz results were assessed. The mean score of 432 international participants was 5.37 (SD 1.75) out of a maximum of 10 (highest score). Eleven percent (n = 47) reached the 80% pass mark. Subanalysis showed no difference by the continent (p = 0.270), ethnicity (p = 0.397), profession (p = 0.599), training resources (p = 0.198) or confidence (p = 0.400). A significance was observed in the specialty (p = 0.01). A weak correlation between experience and confidence (Spearman's ρ = 0.286), but no correlation between scores and confidence or experience (ρ = 0.087 and 0.076), was observed. Of diagnoses, eczema was recognised in 40% and meningococcal rash in 61%. This is the first study assessing the identification of paediatric skin conditions in different skin tones internationally. The correct identification of common/important paediatric conditions was poor, suggesting a possible difference in knowledge across skin tones. There is an urgent need to improve the representation of all skin tones to ensure equity in patient care.

Impact of Diversity in Training Resources on Self-Confidence in Diagnosing Skin Conditions Across a Range of Skin Tones: An International Survey.

Background: Medical images are invaluable in facilitating recognition of clinical signs. Recent studies highlight a lack of diversity of skin tone images used within medical education. However, there is a paucity of data on the impact of this on patient care. Aims: To investigate diversity in training resources used by users of an International online teaching platform and self-confidence in diagnosing skin conditions in all skin tones. Methods: Users of an online teaching platform (www.dftbskindeep.com) were invited to participate in a survey evaluating key points including geographical location, ethnicity, profession, specialty, years of experience, training resources and confidence in diagnosing skin conditions. Data analyses were performed using SPSS. Categorical variables were presented as proportions. Chi-squared or Fisher's exact tests were used to compare the distribution between groups as appropriate. Results: Of 600 participants, 74% reported training resources featuring predominantly white skin. Participants were "generally uncertain" in 43% cases, "sometimes uncertain but clinically safe" (52%), and "confident across a range of skin tones" in a minority (5%). Self-confidence was associated with location [higher in Africa (29%) and Latin America (11%), (p < 0.001)]; diversity of training resources [higher with a mix (10%) or darker tones (20%) (p < 0.001)]; clinical experience [6-10 (5%) or >10 years of practice (11%) (p < 0.001)] and specialty [highest in dermatologists (53%, p < 0.001)]. Self-confidence was lowest among pediatricians, emergency medicine and pediatric emergency medicine specialists (<5%). Conclusions: These data provide preliminary evidence that training resources used by healthcare professionals on a global scale may lack enough diversity on representation of skin images, and a lack of self-confidence in diagnosing pediatric skin conditions. Further work is needed to understand the impact on knowledge and patient care to ensure equitable healthcare for all.

The Childhood Cancer Diagnosis (CCD) Study: a UK observational study to describe referral pathways and quantify diagnostic intervals in children and young people with cancer.

INTRODUCTION: Childhood cancer is diagnosed in 400 000 children and young people (CYP) aged 0-19 years worldwide annually. In the UK, a child's cumulative cancer risk increases from 1 in 4690 from birth to aged 1, to 1 in 470 by age 15. Once diagnosed, access to treatments offers survival to adulthood for over 80%. Tumour diagnoses are at a later stage and mortality is higher when compared with those in other parts of Europe. This means higher risk, more intensive therapies for a cure. Some CYPs are known to experience delays to diagnosis which may further contribute to poor outcomes. This study aims to understand the current pathway of childhood cancer referrals and diagnosis and quantify diagnostic intervals in the UK. METHODS AND ANALYSIS: This is a prospective multicentre observational study including all tertiary childhood cancer treatment centres in the UK. CYP (0-18 years) with a new diagnosis of cancer over the study period will be invited to participate. Data will be collected at initial diagnosis and 5 years after diagnosis. Data will include demographic details, clinical symptoms, tumour location, stage and clinical risk group. In addition, key diagnostic dates and referral routes will be collected to calculate the diagnostic intervals. At 5 years' follow-up, data will be collected on refractory disease, relapse and 1-year and 5-year survival. Population characteristics will be presented with descriptive analyses with further analyses stratified by age, geographical region and cancer type. Associations between diagnostic intervals/delay and risk factors will be explored using multiple regression and logistic regression. ETHICS: The study has favourable opinion from the York and Humber, Leeds West REC (19/YH/0416). DISSEMINATION: Results will be presented at academic conferences, published in peer-reviewed journals and disseminated through public messaging in collaboration with our charity partners through a national awareness campaign (ChildCancerSmart). STUDY REGISTRATION: researchregistry.com (researchregistry5313).

Importance and impact of diversity in paediatric medical education.

Despite the increasing diversity of the UK population, we continue to see inequalities in health outcomes in patients of ethnic minorities. Unfortunately, this increasing diversity is not reflected in educational resources, which often continue to feature few images of patients with darker skin tones. Given the lack of diversity in these resources, it is unsurprising that healthcare professionals find it challenging recognising, diagnosing and managing presentations of clinical conditions in darker skinned patients. The development of new online, accessible image banks of medical conditions in different skin tones, such as Skin Deep, aims to facilitate increasing diversity in educational materials and hence improve delivery of equal standards of care to all patients.

Multicentre service evaluation of presentation of newly diagnosed cancers and type 1 diabetes in children in the UK during the COVID-19 pandemic.

Background: The COVID-19 pandemic led to changes in patterns of presentation to emergency departments. Child health professionals were concerned that this could contribute to the delayed diagnosis of life-threatening conditions, including childhood cancer (CC) and type 1 diabetes (T1DM). Our multicentre, UK-based service evaluation assessed diagnostic intervals and disease severity for these conditions. Methods: We collected presentation route, timing and disease severity for children with newly diagnosed CC in three principal treatment centres and T1DM in four centres between 1 January and 31 July 2020 and the corresponding period in 2019. Total diagnostic interval (TDI), patient interval (PI), system interval (SI) and disease severity across different time periods were compared. Results: For CCs and T1DM, the route to diagnosis and severity of illness at presentation were unchanged across all time periods. Diagnostic intervals for CCs during lockdown were comparable to that in 2019 (TDI 4.6, PI 1.1 and SI 2.1 weeks), except for an increased PI in January-March 2020 (median 2.7 weeks). Diagnostic intervals for T1DM during lockdown were similar to that in 2019 (TDI 16 vs 15 and PI 14 vs 14 days), except for an increased PI in January-March 2020 (median 21 days). Conclusions: There is no evidence of diagnostic delay or increased illness severity for CC or T1DM, during the first phase of the pandemic across the participating centres. This provides reassuring data for children and families with these life-changing conditions.

Changing patterns of emergency paediatric presentations during the first wave of COVID-19: learning for the second wave from a UK tertiary emergency department.

Background: The SARS-CoV-2 pandemic and initial public health response led to significant changes in health service delivery, access and utilisation. However, SARS-CoV-2 illness burden in children and young people (CYP) is low. To inform effective child public health interventions, we aimed to compare patterns of paediatric emergency department presentation during the initial pandemic response with a previous non-pandemic period. Methods: Retrospective review of attendances (0-18 years) over the initial pandemic (2 March 2020-3 May 2020) compared with 2019. Outcome measures included number of attendances, referral source, presenting complaint, discharge diagnosis and disposal. Descriptive statistics with subgroup analysis by age/sex/ethnicity and pandemic time periods (pre-lockdown, lockdown weeks 1-3 and lockdown weeks 4-6) was performed. Results: 4417 attendances (57% illness and 43% injuries) occurred, compared with 8813 (57% illness and 43% injuries), a reduction of 50%, maximal in lockdown week 2 (-73%). Ranking of top three illness presentations changed across the pandemic weeks. Breathing difficulty dropped from first (300, 25%) to second (117, 21%) to third (59, 11%) (p<0.001). Abdominal pain rose from the third pre-lockdown (87, 7%) and lockdown weeks 1-3 (37, 7%) to second in weeks 4-6 (62, 12%; p=0.004). Fever ranked second (235, 19%) in pre-lockdown and first in weeks 1-3 (134, 24%) and weeks 4-6 (94, 18%; p=0.035). Conclusions: Despite a 50% reduction, there was no significant change in acuity of illness. Rank of illness presentations changed, with abdominal pain ranking second and fever first, an important change from previous, which should prompt further research into causes. CYP-specific public health messaging and guidance for primary care are required in this second wave to ensure access to appropriate emergency services.

Delphi method to identify expert opinion to support children's cancer referral guidelines.

BACKGROUND: The National Institute for Health and Care Excellence (NICE) guidance for referral of children with suspected cancer was first published in 2005 and updated in 2015. The updated version relied on sparse primary care evidence and published without input from key stakeholders, for example, acute general paediatricians and paediatric haematologists/oncologists. This led to a document that fell short as a practical guide for referring physicians managing children with potentially life-threatening conditions. Following discussions between the Children's Cancer and Leukaemia Group (CCLG, the UK multidisciplinary professional body for healthcare professionals caring for children with cancer) and NICE, it was agreed that a practical supplement should be produced for the 2015 guidance. A prerequisite was evidence gathering from tertiary care to balance the existing primary care evidence, and a Delphi consensus method was therefore convened. METHODS: A CCLG NICE Guidance Committee formulated 25 draft statements for review. The CCLG emailed its paediatric haematologist/oncologist membership (n=179) and 88 responded (49%). To achieve consensus, statements required ≥70% agreement from ≥60% of actual respondents, from the denominator (n=88). RESULTS: Fifteen of 25 original statements were accepted at the first round of voting. Three of 25 statements where >50% did not support were rejected outright. One statement could not be revised without replicating a previously accepted statement. The six remaining statements were revised and a second round of voting undertaken; all six revised statements were accepted. Overall, 21 of 25 statements (84%) met consensus criteria. CONCLUSIONS: This expert opinion should help streamline suspected cancer referral in children and help optimise subsequent outcomes.

Accelerating diagnosis for childhood brain tumours: an analysis of the HeadSmart UK population data.

BACKGROUND: HeadSmart, a public and professional awareness campaign, was launched to enhance awareness of brain tumour symptomatology identified in the Royal College of Paediatrics and Child Health, National Institute for Health and Care Excellence-accredited guideline. Quality improvement data showed a reduction in diagnostic interval nationally. To reach the government target of 4 weeks, we need to identify subgroups with ongoing delays. METHODS: Incident cases of brain tumours (0-18) diagnosed between January 2011 and May 2013 across 18 UK centres were included. Anonymised data including demographics, diagnosis and date of symptom onset/presentation were collected. Key outcome measures, total diagnostic interval (TDI), patient interval (PI) and system interval (SI) were calculated. Subanalysis by age, tumour grade and location was also performed. RESULTS: Young children (0-5 years) accounted for 38% of cases, with a peak age at diagnosis of 2 years. Central tumours experienced longest intervals with a median TDI of 10.5 weeks, PI of 3.2 weeks and SI of 2.9 weeks. Craniopharyngioma, low-grade glioma and optic pathway gliomas had the longest TDIs with a median of 15.1, 11.9 and 10.4 weeks, respectively. The greatest proportion of delay was in the SI. The 12-18 age group had a median TDI of 12.1 weeks, compared with 8 weeks for the 5-11 age group and 6 weeks for the 0-5 age group (p<0.001). CONCLUSIONS: Clear patterns of intervals for different age groups and anatomical locations have been demonstrated. Tailoring education and awareness strategies to ensure earlier diagnosis for central tumours and young people is crucial to minimise brain injury, subsequent disability and late effects of treatment for 70% of survivors.

Medical problems presenting to paediatric emergency departments: 10 years on.

OBJECTIVE: To describe the common medical presenting problems of children attending a paediatric emergency department (ED) compared with 10 years previously. DESIGN: A retrospective review of electronic patient record and comparison with previous cohort. SETTING: A UK university hospital ED. PATIENTS: A cohort of children and young people aged 0-15 years who attended the ED between 7 February 2007 and 6 February 2008 (n=39 394) compared with a historical cohort from 10 years earlier. MAIN OUTCOME MEASURES AND RESULTS: Information on presenting problem, demographic data and source of referral were collected. Presenting problems were ranked and comparisons made with previous data using the difference between proportions analysis and the significance test for a difference in two proportions. A total of 39 394 children (57% boys) were seen with 14 724 medical attendances compared with 10 369 attendances from the 1997 cohort, an increase of 42%. Most (85%) ED attendances can be accounted for by the 10 most common presenting problems, including breathing difficulty (2494, 20.1%), febrile illness (1752, 14.1%), diarrhoea with or without vomiting (1731, 14.0%), rash (1066, 8.6%) and cough (835, 6.7%). Similar proportions are described to a decade earlier; however, there were fewer patients attending with breathing difficulty (-10.9%, p<0.001). CONCLUSIONS: Over a 10-year period, there has been a rise in the number of people attending the ED with medical conditions. The 10 most common presenting problems account for 85% of medical attendees. These results suggest the increasing utilisation of ED services for children with common medical presenting problems and should inform further research exploring the pathways for attendance and the thresholds in seeking medical advice in order to inform the commissioning of paediatric emergency and urgent care services.