A prospective evaluation of the fourth national Be Clear on Cancer 'Blood in Pee' campaign in England.

OBJECTIVE: To assess the impact of the fourth Be Clear on Cancer (BCoC) 'Blood in Pee' (BiP) campaign (July to September 2018) on bladder and kidney cancer symptom awareness and outcomes in England. METHODS: In this uncontrolled before and after study, symptom awareness and reported barriers to GP attendance were assessed using panel and one-to-one interviews. The Health Improvement Network (THIN), National Cancer Registration and Analysis Service (NCRAS) and NHS Cancer Waiting Times (CWT) data were analysed to assess the impact on GP attendances, urgent cancer referrals, cancer diagnoses and 1-year survival. Analyses used Poisson, negative binomial and Cox regression. RESULTS: Symptom awareness and intention to consult a GP after one episode of haematuria increased following the campaign. GP attendance with haematuria (rate ratio (RR) 1.17, 95% confidence interval (CI): 1.07-1.28) and urgent cancer referrals (RR 1.18 95% CI: 1.08-1.28) increased following the campaign. Early-stage diagnoses increased for bladder cancer (difference in percentage 2.8%, 95% CI: -0.2%-5.8%), but not for kidney cancer (difference -0.6%, 95% CI: -3.2%-2.1%). CONCLUSIONS: The fourth BCoC BiP campaign appears to have been effective in increasing bladder cancer symptom awareness and GP attendances, although long-term impacts are unclear.

Impact of the third national 'Be Clear on Cancer' Breast Cancer in Women over 70 Campaign on general practitioner attendance and referral, diagnosis rates and prevalence awareness.

OBJECTIVE: More than a third of women diagnosed with breast cancer in England, and over half of those who die from it, are over 70. The Be Clear on Cancer Breast Cancer in Women over 70 Campaign, running three times, 2014-2018, aimed to promote early diagnosis of breast cancer in England by raising symptom awareness and encouraging women to see their general practitioner (GP) without delay. We sought to establish whether the third campaign had successfully met its aims. METHODS: Metrics covering the patient pathway, including symptom awareness, attending a GP practice with symptoms, urgent GP referral, diagnosis and stage of cancer, were assessed using national cancer databases and two household surveys. RESULTS: The third campaign was associated with an increase in urgent cancer referrals, and therefore mammograms and ultrasounds performed. This was associated with an increase in breast cancers diagnosed. There was a delayed effect on GP attendances. Awareness of breast cancer prevalence for the 70-and-over age group improved. Impact on these metrics diminished across successive campaigns. CONCLUSIONS: Future campaigns should focus on harder-to-reach women and include GPs as targets as this campaign showed a potential to affect referral behaviour.

An evaluation of a national mass media campaign to raise public awareness of possible lung cancer symptoms in England in 2016 and 2017.

BACKGROUND: A two-phase 'respiratory symptoms' mass media campaign was conducted in 2016 and 2017 in England raising awareness of cough and worsening shortness of breath as symptoms warranting a general practitioner (GP) visit. METHOD: A prospectively planned pre-post evaluation was done using routinely collected data on 15 metrics, including GP attendance, GP referral, emergency presentations, cancers diagnosed (five metrics), cancer stage, investigations (two metrics), outpatient attendances, inpatient admissions, major lung resections and 1-year survival. The primary analysis compared 2015 with 2017. Trends in metrics over the whole period were also considered. The effects of the campaign on awareness of lung cancer symptoms were evaluated using bespoke surveys. RESULTS: There were small favourable statistically significant and clinically important changes over 2 years in 11 of the 15 metrics measured, including a 2.11% (95% confidence interval 1.02-3.20, p < 0.001) improvement in the percentage of lung cancers diagnosed at an early stage. However, these changes were not accompanied by increases in GP attendances. Furthermore, the time trends showed a gradual change in the metrics rather than steep changes occurring during or after the campaigns. CONCLUSION: There were small positive changes in most metrics relating to lung cancer diagnosis after this campaign. However, the pattern over time challenges whether the improvements are wholly attributable to the campaign. Given the importance of education on cancer in its own right, raising awareness of symptoms should remain important. However further research is needed to maximise the effect on health outcomes.

Abdominal cancer symptoms: Evaluation of the impact of a regional public awareness campaign.

OBJECTIVE: A regional 'Be Clear on Cancer' (BCoC) campaign developed by Public Health England aimed to promote public awareness of key abdominal cancer symptoms in people aged 50 years and over. METHODS: Data were analysed for metrics at different stages in the patient care pathway including public awareness, GP attendance and referrals, to cancer diagnosis. RESULTS: There was significantly higher recognition of the BCoC abdominal campaign in the campaign region compared to the control area (Post Campaign/Control, n = 401/406; 35% vs. 24%, p < 0.05). The campaign significantly improved knowledge of 'bloating' as a symptom (p = 0.03) compared to pre-campaign levels. GP attendances for abdominal symptoms increased significantly by 5.8% (p = 0. 03), although the actual increase per practice was small (average 16.8 visits per week in 2016 to 17.7 in 2017). Urgent GP referrals for suspected abdominal cancer increased by 7.6%, compared to a non-significant change (0.05%) in the control area. For specific abdominal cancers, the number diagnosed were similar to or higher than the median in the campaign area but not in the control area in people aged 50 and over: colorectal (additional n = 61 cancers), pancreatic (additional n = 102) and stomach cancers (additional n = 17). CONCLUSIONS: This campaign had a modest impact on public awareness of abdominal cancer symptoms, GP attendances and cancers diagnosed.

Relationships between hope and mental health among women in prison.

BACKGROUND: Previous research with non-offenders has linked a higher dispositional cognition of hope to lower levels of psychological symptoms and demonstrated mediating effects of attentional biases on the relationship between hope and psychological symptoms, but this has not been explored among offenders. AIMS: Our aim was to investigate associations between a dispositional cognition of hope and habitual attentional processing styles and distress among women in prison. We hypothesised that higher levels of hope would be associated with more attention to positive information and less to negative information in the surroundings and, in turn, lower levels of depression, anxiety and stress symptoms in women in prison. METHOD: In a cross-sectional study, we recruited consenting women serving a prison sentence who had been referred to psychological services. Participants completed a set of self-rating inventories individually, which scaled their levels of hope, attention to positive and negative information and symptoms of anxiety, stress and depression. RESULTS: Two hundred and three women participated. Their average age was 35.68 years (range 21-67 years). Over half were recidivists (170, 58%). Overall, the higher the level of hope they had, the lower were the ratings of their psychological symptoms. Positive attentional bias was associated with higher hope and lower psychological distress. In contrast, negative attentional bias was related to lower hope and higher psychological distress ratings. In statistical models, both attentional biases appeared to be partial mediators of the relationship between hope and psychological distress. CONCLUSIONS: Our findings among women in prison were consistent with those in non-forensic populations and not previously studied among prisoners. They suggest that it would be worth evaluating interventions to modify attentional styles as they may have value in increasing hope and reducing psychological symptoms and perhaps also harmful behaviours in this vulnerable population.

Reviewing the impact of 11 national Be Clear on Cancer public awareness campaigns, England, 2012 to 2016: A synthesis of published evaluation results.

The Be Clear on Cancer (BCoC) campaigns have run in England since 2010. They aim to raise awareness of possible cancer symptoms, encouraging people to consult a general practice with these symptoms. Our study provides an overview of the impact of 11 national campaigns, for bowel, lung, bladder and kidney, breast and oesophago-gastric cancers. We synthesised existing results for each campaign covering seven clinical metrics across the patient pathway from primary care attendances to one-year net survival. For each metric, "before" and "after" periods were compared to assess change potentially related to the campaign. Results show that primary care attendances for campaign-related symptoms increased for 9 of 10 campaigns and relevant urgent referrals for suspected cancer increased above general trends for 9 of 11 campaigns. Diagnostic tests increased for 6 of 11 campaigns. For 7 of 11 campaigns, there were increases in cancer diagnoses resulting from an urgent referral for suspected cancer. There were sustained periods where more cancers were diagnosed than expected for 8 of 10 campaigns, with higher than expected proportions diagnosed at an early stage for sustained periods for 4 of 10 campaigns. There was no impact on survival. In summary, there is evidence that the BCoC campaigns impact help-seeking by patients and referral patterns by general practitioners, with some impact on diagnosis (incidence and stage). There was no clear evidence of impact on survival.

When Does Stand-Alone Software Qualify as a Medical Device in the European Union?-The Cjeu's Decision in Snitem and What it Implies for the Next Generation of Medical Devices.

This contribution analyses the first decision by the Court of Justice of the European Union (CJEU) on the qualification and regulation of stand-alone software as medical devices. Referring to the facts of the case and the applicable European Union (EU) regulatory framework, the Court specifically found that prescription support software may constitute a medical device. This would even be the case where the software does not act directly in or on the human body. Yet, according to the CJEU, it is necessary that the intended purpose falls within one or more of the 'medical purpose' categories of the regulatory definition of 'medical device'. The case has important implications, not only for specific legal debates, but it also signifies a paradigm shift with a rapidly increasing digitalisation of the health and life sciences. This highlights the demand for continuous debates over the necessary evolution of the regulatory framework applying to the interface of medical artificial intelligence (AI) and Big Data.

Extended-Release Oral Milrinone for the Treatment of Heart Failure With Preserved Ejection Fraction.

Background Heart failure with preserved ejection fraction (HFpEF) is an increasingly prevalent form of heart failure, representing half of the total burden of heart failure. We hypothesised that modulation of the phosphodiesterase type 3/cyclic AMP using a novel oral formulation of milrinone might exert favorable effects HFpEF via pulmonary and systemic vasodilation and enhancement of ventricular relaxation. We assessed the safety and efficacy of oral milrinone on quality of life and functional outcomes in patients with HFpEF. Methods and Results The MilHFPEF (Extended Release Oral Milrinone for the Treatment of Heart Failure With Preserved Ejection Fraction) study was a randomized, double-blind, placebo-controlled pilot study in 23 patients with symptomatic HFpEF. Efficacy end points included changes from baseline in Kansas City Cardiomyopathy Questionnaire summary score and 6-minute walk distance. The primary safety end point was the development of clinically significant arrhythmia. The Kansas City Cardiomyopathy Questionnaire score improved significantly in milrinone-treated patients compared with placebo (+10±13 versus -3±15; P=0.046). Six-minute walk distance also tended to improve in the treatment group compared with placebo (+22 [-8 to 49] versus -47 [-97 to 12]; P=0.092). Heart rate (-1±5 versus -2±9 bpm; P=0.9) and systolic blood pressure (-3±18 versus +1±12 mm Hg; P=0.57) were unchanged. Early filling velocity/early mitral annular velocity (-0.3±3.0 versus -1.9±4.8; P=0.38) was unchanged. One patient in the placebo arm was hospitalized for heart failure. Holter monitoring did not demonstrate evidence of a proarrhythmic effect of milrinone. Conclusions In this novel pilot study, extended release oral milrinone was well tolerated and associated with improved quality of life in patients with HFpEF. Further longer-term studies are warranted to establish the role of this therapeutic approach in HFpEF. Registration URL: https://www.anzctr.org.au/; Unique identifier: ACTRN12616000619448.

Optimal Mechanical Unloading in Left Ventricular Assist Device Recipients Relates to Progressive Up-Titration in Pump Speed.

BACKGROUND: Left ventricular (LV) assist devices (LVADs) are known to elicit reverse remodeling by mechanically unloading the left ventricle. Current guidelines target a reduction in LV end-diastolic diameter (LVEDD) of 15% compared with pre-LVAD dimensions; however, there is significant heterogeneity in the degree of unloading achieved. We sought to investigate factors associated with mechanical unloading at 6 months of LVAD support. METHODS: Data were retrospectively collected for 75 LVAD recipients at five time points: pre-LVAD, within 14 days post-LVAD, and at 1, 3, and 6 months post-LVAD. The percentage change in LVEDD between the pre-LVAD and 6 months post-LVAD time points was termed ΔLVEDD. Optimal LV unloading was defined as ΔLVEDD of ≥15% at 6 months. Patients who achieved optimal unloading (group A, n = 30) were compared with patients who did not (group B, n = 45). RESULTS: At 6 months, optimally unloaded patients (group A) demonstrated higher fractional shortening (15% ± 10% vs 10% ± 7%, P = .007), lower rates of moderate or severe mitral regurgitation (10% vs 33%, P = .02), and lower pulmonary capillary wedge pressure (9 ± 4 vs 16 ± 7 mm Hg, P = .02). Right ventricular dysfunction was more prevalent at 6 months in poorly unloaded (group B) patients (73% vs 43%, P = .008). Between hospital discharge and 6 months, the percentage increase in pump speed (Δ revolutions per minute) was higher in group A patients (4.4% ± 3.7% vs 0.1% ± 2.6%, P < .001). In a multivariate analysis, Δ revolutions per minute and tricuspid annular systolic velocity (S') at 6 months were independently associated with 6-month ΔLVEDD. CONCLUSIONS: Recipients of LVADs who undergo progressive pump speed up-titration during outpatient follow-up are more likely to sustain optimal LV unloading. Progressive LVAD-related right ventricular failure is prevalent in suboptimally unloaded patients.

Extended Release Oral Milrinone, CRD-102, for Advanced Heart Failure.

Although heart transplantation and mechanical circulatory support are effective therapies for patients with advanced heart failure (HF), many patients are ineligible due to co-morbidities. Continuous home intravenous with positive inotropes such as milrinone are used in these patients to improve quality of life. We hypothesized that, unlike previous studies with oral milrinone, a slow-release formulation that provides stable lower plasma levels may be better tolerated and provide symptomatic benefit. Accordingly, we developed an extended release milrinone formulation (CRD-102) and evaluated its effects in 26 patients with no-option Stage D HF. One month after open-label therapy there were significant improvements in NYHA class, Minnesota Living with Heart Failure score and 6-minute walk distance. There was no evidence of hypotension or increased arrhythmic burden. In conclusion, the present study demonstrates evidence of beneficial actions of extended release milrinone in advanced HF. Longer-term randomized clinical trial data are required.

Enhancing self-care strategies in heart failure through patient-reported outcome measures.

Heart failure (HF) is a major cause of morbidity and mortality, requiring collaborative patient-centred care. Patient engagement is fundamental to long-term management; patient-reported outcome measures are an increasingly recognised method of assessing medical interventions. A qualitative study of 31 patients with HF found they were twice as likely to use their own management strategies including electronic platforms rather than existing resources. Barriers to self-care included patient education, timely recognition of signs and symptoms of HF with an appropriate escalation plan, non-adherence and polypharmacy.

Effects of cognitive-behavioural therapy (CBT) and positive psychological intervention (PPI) on female offenders with psychological distress in Hong Kong.

BACKGROUND: Despite rapid growth in the female prison population, there is little research on effectiveness of psychological interventions for them. AIMS: To test the hypotheses that (1) each of two psychological interventions administered separately - cognitive behavioural therapy (CBT) or positive psychology intervention (PPI) - would be more effective than 'treatment-as-usual' alone in reducing psychological distress and enhancing psychological well-being; (2) outcomes would differ according to intervention; and (3) combining the interventions would be more effective than delivering either alone. METHODS: We recruited 40 women in a special Hong Kong prison unit for female offenders with psychological distress. Half of them received eight sessions of CBT followed by eight sessions of PPI; the other half received the same interventions in the reverse order. We recruited another 35 women who received only 'treatment as usual' (TAU) in the same unit. We used various clinical scales to assess the women's psychological distress or well-being before and after the interventions or at similar time points for the comparison women. RESULTS: All intervention group women showed a significant reduction in psychological distress and enhancement in psychological well-being after each intervention alone compared to the TAU women. There were no significant differences between CBT and PPI in this respect. Receiving both treatments, however, did yield significantly more improvement than either intervention alone in reducing depressive thoughts and enhancing global judgement of life satisfaction, self-perceived strengths and hopeful thinking style. CONCLUSIONS AND IMPLICATION FOR PRACTICE: Our findings provide preliminary empirical support for the effectiveness of psychological interventions with psychologically distressed women in prison. It would be important now to conduct a full, randomised trial to determine optimal length and combinations of treatment. Copyright © 2017 John Wiley & Sons, Ltd.

A Gender-Responsive Treatment Facility in Correctional Services: The Development of Psychological Gymnasium for Women Offenders.

With increasing evidence suggesting a disparity in female and male offenders in terms of rehabilitative needs, growing concern has been placed on the development of gender-specific services for female offenders. As such, a prison-based psychological gymnasium (PSY GYM), with distinctive features in integrating cognitive-behavioral and positive-psychology concepts to address female offenders' needs, was set up in 2011. The present study aims to introduce the concept as well as present findings to evaluate the overall effectiveness of PSY GYM. Nineteen Chinese female offenders participated in a 6- to 8-month intensive training program at the Lo Wu Correctional Institution (LWCI) of the Hong Kong Correctional Services Department in Hong Kong. Psychometric inventories were used to assess their psychological distress and positive growth following the program. Participants showed significant reduction in their depression, anxiety, and stress symptoms, and in their tendency to pay attention to negative information. Furthermore, their sense of hope, gratitude, and tendency to pay attention to positive information had remarkably increased. This indicated the effectiveness of PSY GYM in facilitating successful rehabilitation for female offenders.

Effectiveness of online cognitive behavioral therapy on family caregivers of people with dementia.

PURPOSE: Family caregivers of persons with dementia (PWD) may receive caregiver training because of logistical constraints and privacy concerns. This study evaluated the effectiveness of an online intervention for family caregivers of PWD in improving their self-efficacy in managing behavioral and psychological symptoms of dementia (BPSD), and their emotion well-being. SUBJECTS AND METHODS: A total of 36 family caregivers of people with dementia participated in a 9-week online intervention based on the cognitive behavioral therapy model. Outcomes of the intervention were measured by the Chinese version of the Neuropsychiatric Inventory Questionnaire and two domains of the Revised Scale for Caregiving Self-Efficacy. Wilcoxon signed rank tests were used to compare the change in outcome variables. RESULTS: The severity of BPSD of PWD and BPSD-related distress in family caregivers showed a statistically significant reduction after the intervention. Subgroup analysis showed self-efficacy in controlling upsetting thoughts significantly improved in caregivers of PWD at moderate to severe stages. CONCLUSION: Online cognitive behavioral therapy for family caregivers reduced BPSD of PWD and the related distress in their caregivers.

Reducing iodinated contrast volume by manipulating injection pressure during coronary angiography.

OBJECTIVE: To evaluate the impact of a device-mediated modification of the injection pressure profile on iodinated contrast volumes during coronary angiography and percutaneous coronary intervention (PCI). BACKGROUND: The development of iodinated contrast-induced acute kidney injury (AKI) is associated with significant morbidity and mortality in patients undergoing coronary angiography and intervention, particularly in the setting of ST-elevation myocardial infarction. Iodinated contrast volume is a recognized risk factor for iodinated contrast-induced AKI. Mitigation of iodinated contrast loss due to coronary iodinated contrast reflux represents a potential means to reduce iodinated contrast volume. METHOD AND RESULTS: We assessed the effectiveness of injection pressure modulation using an iodinated contrast conservation system (CCS), in 21 patients undergoing diagnostic coronary angiography or PCI. System activation was associated with a marked reduction in iodinated contrast injection compared with standard imaging (3.5 ± 1.1 vs. 5.4 ± 1.7 mL, P < 0.001). Paired images were obtained in 59 instances with significantly less iodinated contrast use with CCS use (3.3 ± 1.1 vs. 5.5 ± 1.6 mL, P < 0.001), with comparable image quality in 98% of cases. CONCLUSIONS: Modulation of iodinated contrast injection pressure provides a novel potential means to reduce iodinated contrast volumes by attenuating inefficient losses due to ostial reflux. Future studies are required to define the ability of the CCS to reduce total iodinated contrast volume and to impact upon the risk of iodinated contrast-induced AKI.

Lung cancer incidence and survival in England: an analysis by socioeconomic deprivation and urbanization.

INTRODUCTION: Most previous studies have investigated either socioeconomic deprivation or urbanization in relationship to lung cancer incidence or survival. We investigated the association between socioeconomic deprivation, urbanization, and lung cancer incidence and survival in England. METHODS: We extracted data on patients diagnosed with lung cancer (ICD-10 C33-C34) between 2003 and 2007 and who were resident in England. We assigned each patient to an urbanization score and to a socioeconomic quintile based on their postcode of residence. We calculated age-specific and age-standardized incidence rates (per 100,000 European standard population) by urbanization, sex, and socioeconomic deprivation group. We used Kaplan-Meier survival analysis to compare the survival of patients from urban and rural areas by socioeconomic deprivation. RESULTS: A high proportion of urban areas in England were classified as deprived and rural areas were mostly affluent. The incidence of lung cancer was higher in urban areas than in rural areas. In the more affluent areas, the incidence of lung cancer in urban and rural areas was very similar. Survival from lung cancer was slightly higher in affluent areas than in deprived areas. Survival from lung cancer in urban and rural areas was similar across all socioeconomic deprivation quintiles. CONCLUSIONS: The difference in incidence between urban and rural areas can be explained by the differences in the distribution of socioeconomic deprivation quintiles in the two urbanization categories. When socioeconomic deprivation is taken into account, little difference is seen between both the incidence and survival of lung cancer in urban and rural areas.

Survival from twenty adult cancers in the UK and Republic of Ireland in the late twentieth century.

BACKGROUND: International studies have shown that cancer survival was generally low in the UK and the Republic of Ireland compared to western and northern European countries, but no systematic comparative analysis has been performed between the UK countries and the Republic of Ireland. METHODS: Population-based survival for 20 adult malignancies was estimated for the UK and the Republic of Ireland. Data on adults (15-99 years) diagnosed between 1991 and 1999 in England, Scotland, Wales, Northern Ireland (1993-99) and the Republic of Ireland (1994-99) were analysed. All cases were followed up until the end of 2001. Relative survival was estimated by sex, period of diagnosis and country, and for the nine regions of England. Predicted survival was estimated using the hybrid approach. RESULTS: Overall, cancer survival in UK and Republic of Ireland improved during the 1990s, but there was geographic variation in survival across the UK and Republic of Ireland. Survival was generally highest in Ireland and Northern Ireland and lowest in England and Wales. Survival tended to be higher in Scotland for cancers for which early detection methods were in place. In England, survival tended to be lower in the north and higher in the south. CONCLUSIONS: The geographic variations in survival seen across the UK and Republic of Ireland are narrower than between these countries and comparable European countries. Artefact is likely to explain some, but not all of the differences across the UK and Republic of Ireland. Geographic differences in stage at diagnosis, co-morbidity and other clinical factors may also be relevant.

Socio-economic disparities in access to treatment and their impact on colorectal cancer survival.

BACKGROUND: Significant socio-economic disparities have been reported in survival from colorectal cancer in a number of countries, which remain largely unexplained. We assessed whether possible differences in access to treatment among socio-economic groups may contribute to those disparities, using a population-based approach. METHODS: We retrospectively studied 71 917 records of colorectal cancer patients, diagnosed between 1997 and 2000, linked to area-level socio-economic information (Townsend index), from three cancer registries in UK. Access to treatment was measured as a function of delay in receipt of treatment. We assessed socio-economic differences in access through logistic regression models. Based on relative survival < or =3 years after diagnosis, we estimated excess hazard ratios (EHRs) of death for different socio-economic groups. RESULTS: Compared with more affluent patients, deprived patients had poorer survival [EHR = 1.20; 95% confidence interval (CI) 1.16-1.25], were less likely to receive any treatment within 6 months [odds ratio (OR) = 0.87, 95% CI 0.82-0.92] and, if treated, were more likely to receive late treatment. No disparities in survival were detected among patients receiving treatment within 1 month from diagnosis. Disparities existed among patients receiving later or no treatment (EHR = 1.30; 95% CI 1.22-1.39), and persisted after adjustment for age and stage at diagnosis (EHR = 1.15; 95% CI 1.08-1.24). CONCLUSIONS: Tumour stage helped explain socio-economic disparities in colorectal cancer survival. Disparities were also greatly attenuated among patients receiving early treatment. Aspects other than those captured by our measure of access, such as quality of care and patient preferences in relation to treatment, might contribute to a fuller explanation.

The epidemiology and survival of extrapulmonary small cell carcinoma in South East England, 1970-2004.

BACKGROUND: Extrapulmonary small cell carcinoma (EPSCC) is a rare cancer and few studies describe its epidemiology. Our objectives were to compare the incidence and survival of EPSCC in South East England with small cell carcinoma of the lung (SCLC), to determine the most common anatomical presenting sites for EPSCC and to compare survival in EPSCC by disease stage and site of diagnosis. METHODS: We used data from the Thames Cancer Registry database for South East England between 1970 and 2004 to determine the incidence, most common anatomical sites, and survival by site, and stage of EPSCC. 1618 patients registered with EPSCC were identified. We calculated the age-standardised incidence rate for EPSCC using the European standard population and compared this to that for SCLC. We calculated survival using the Kaplan-Meier method for EPSCC and SCLC, and reported 3-year survival for different EPSCC anatomical sites and disease stages. RESULTS: The incidence of EPSCC was much lower than for SCLC, similar in males and females, and stable throughout the study period, with incidence rates of 0.45 per 100,000 in males and 0.37 in females during 2000-2004. In general, patients with EPSCC had a better 3-year survival (19%) than SCLC (5%). The most common anatomical sites for EPSCC were oesophagus (18%), other gastrointestinal (15%), genitourinary (20%), head and neck (11%), and breast (10%). Breast EPSCC had the best 3-year survival (60%) and gastrointestinal EPSCC the worst (7%). CONCLUSION: This study suggests that EPSCC has a stable incidence and confirms that it presents widely, but most commonly in the oesophagus and breast. Site and extent of disease influence survival, with breast EPSCC having the best prognosis. Further studies using standardised diagnosis, prospective case registers for uncommon diseases and European cancer registries are needed to understand this disease.