Incidence and survival of uveal melanoma in Northern Ireland: how incomplete data can skew results in rare cancers.

BACKGROUND: The majority of Northern Irish uveal melanoma (UM) patients are diagnosed in Sheffield. This study aims to present incidence and survival outcomes for UM patients from Northern Ireland (NI). METHODS: Collaborative retrospective study between Sheffield and Northern Ireland Cancer Registry (NICR). For UM cases not on both databases, outcomes and survival rates (via Kaplan-Meier analysis) were compared. Anonymised NICR data were used to calculate whole-population incidence of UM for NI. RESULTS: In total, 161 patients from NI were diagnosed in Sheffield, 90 of which were not registered with NICR at the start of this study. Data-omissions were not consistent across patient groups, leading to significant differences between those patients registered and those not. Registered patients had an all-cause 5-year survival rate of only 68.9% compared to 92.5% of those not registered (p < 0.01) and were >17x more likely to have systemic metastases than those not registered (p < 0·001). Following rectification of data-omissions, the European age-standardised incidence rate of UM for NI was 8·6 per million. CONCLUSIONS: This study illustrates the impact of incomplete population-wide data, serving as a real-world lesson in case-identification bias. Rare cancers are at higher risk of omission due to systemic failures as the small numbers involved are not detected by system-wide validation procedures. Following this study, data-transfer agreements between England and NI were actioned, preventing future data-omissions. We present survival and incidence data for UM in NI for the first time, showing the incidence is amongst the highest in Europe, with good survival rates.

Early-Onset Cancers in Adults: A Review of Epidemiology, Supportive Care Needs and Future Research Priorities.

Rising incidence of specific types of early-age onset cancers in adults aged 18-49 years has been reported in high-income countries. In this review, we summarise the epidemiology of early-onset cancers using exemplar data from a high-income UK region, discuss supportive care needs for young patients and outline future research directions. The incidence rate of early-onset cancers increased by 20.5% from 1993 to 2019 in Northern Ireland. Differences in types of cancer were observed between sexes and across age groups of 18-29, 30-39 and 40-49 years. One and five-year net survival was mostly better in 18-29-year-olds for all cancers combined compared to older age groups for both sexes, but there were variations in specific cancer types. Poorer survival was observed for patients with brain/central nervous system, connective and soft tissue or lung cancers. Patients with early-onset cancers face unique supportive care needs and require holistic care. The impact of cancer treatment on fertility and fertility preservation treatments is an important consideration. Social media can be used for patient support, information, fundraising, advocacy work and recruitment to research studies. We also outline suggested future research priorities for early-onset cancers, spanning prevention, diagnosis, treatment and supportive care needs.

Inequalities in the decline and recovery of pathological cancer diagnoses during the first six months of the COVID-19 pandemic: a population-based study.

BACKGROUND: The restructuring of healthcare systems to cope with the demands of the COVID-19 pandemic has led to a reduction in clinical services such as cancer screening and diagnostics. METHODS: Data from the four Northern Ireland pathology laboratories were used to assess trends in pathological cancer diagnoses from 1st March to 12th September 2020 overall and by cancer site, sex and age. These trends were compared to the same timeframe from 2017 to 2019. RESULTS: Between 1st March and 12th September 2020, there was a 23% reduction in cancer diagnoses compared to the same time period in the preceding 3 years. Although some recovery occurred in August and September 2020, this revealed inequalities across certain patient groups. Pathological diagnoses of lung, prostate and gynaecological malignancies remained well below pre-pandemic levels. Males and younger/middle-aged adults, particularly the 50-59-year-old patient group, also lagged behind other population demographic groups in terms of returning to expected numbers of pathological cancer diagnoses. CONCLUSIONS: There is a critical need to protect cancer diagnostic services in the ongoing pandemic to facilitate timely investigation of potential cancer cases. Targeted public health campaigns may be needed to reduce emerging inequalities in cancer diagnoses as the COVID-19 pandemic continues.

Can different definitions of date of cancer incidence explain observed international variation in cancer survival? An ICBP SURVMARK-2 study.

BACKGROUND: Differences in registration practices across population-based cancer registries may contribute to international variation in survival estimates. In particular, there are variations in recorded date of incidence (DOI) as cancer registries have access to different sources of information and use different rules to determine an official DOI. This study investigates the impact of different DOI rules on cancer survival estimates. MATERIALS AND METHODS: Detailed data on dates of pathological confirmation and hospital admittance were collected from three registries participating in the ICBP SURVMARK-2 project (England, Northern Ireland and Norway). Multiple dates of incidence were determined for each cancer patient diagnosed during 2010-2014 by applying three sets of rules that prioritize either: a) histological date, b) hospital admittance date or c) the earliest date recorded. For each set of rules and registry, 1- and 5-year net survival were estimated for eight cancer sites (oesophagus, stomach, colon, rectum, liver, pancreas, lung and ovary). RESULTS: The mean difference between different DOIs within a country and cancer site ranged from 0.1-23 days. The variation in 1- and 5-year net survival using different DOIs were generally small for all registries and cancer sites. Only for liver and pancreatic cancer in Norway and ovarian cancer in England, were larger 1-year survival differences, of 2-3 % found. CONCLUSION: In the ongoing discussion of the comparability of survival estimates across registry populations, the use of different DOI definitions can be considered to have a very limited impact.

Insights into the factors associated with achieving the preference of home death in terminal cancer: A national population-based study.

ABSTRACTObjectives:Most terminally ill cancer patients prefer to die at home, yet only a minority are able to achieve this. Our aim was to investigate the factors associated with cancer patients achieving their preference to die at home. METHODS: This study took the form of a mortality followback, population-based, observational survey of the relatives of deceased cancer patients in Northern Ireland. Individuals who registered the death of a friend or relative (aged ≥ 18 years) between 1 December 2011 and 31 May 2012, where the primary cause of death was cancer (ICD10: C00-D48), who were invited to take part. Preferred and actual place of death, and patient, service, and clinical data were collected using the QUALYCARE postal questionnaire. Multivariable logistic regression was employed to investigate the factors associated with achieving a home death when preferred. RESULTS: Some 467 of 1,493 invited informants completed the survey. The 362 (77.5%) who expressed a preference for dying at home and spent time at home in their final 3 months were included in our analysis. Of these, 53.4% achieved their preference of a home death. Factors positively associated with achieving a home death were: living in an affluent area, receipt of good and satisfactory district nurse care, discussing place of death with health professionals, and the caregiver's preference for a home death. Being older than 80 years of age, being a Presbyterian, and being unconscious most of the time during their final week were negatively associated with achieving a home death. SIGNIFICANCE OF RESULTS: Communication, care satisfaction, and caregiver preferences were all associated with home death. Our findings will help inform the design of future interventions aimed at increasing the proportion of patients achieving their preferred place of death at home, for example, by targeting interventions toward older patients and those from the most deprived communities.

Investigation of the international comparability of population-based routine hospital data set derived comorbidity scores for patients with lung cancer.

INTRODUCTION: The International Cancer Benchmarking Partnership (ICBP) identified significant international differences in lung cancer survival. Differing levels of comorbid disease across ICBP countries has been suggested as a potential explanation of this variation but, to date, no studies have quantified its impact. This study investigated whether comparable, robust comorbidity scores can be derived from the different routine population-based cancer data sets available in the ICBP jurisdictions and, if so, use them to quantify international variation in comorbidity and determine its influence on outcome. METHODS: Linked population-based lung cancer registry and hospital discharge data sets were acquired from nine ICBP jurisdictions in Australia, Canada, Norway and the UK providing a study population of 233 981 individuals. For each person in this cohort Charlson, Elixhauser and inpatient bed day Comorbidity Scores were derived relating to the 4-36 months prior to their lung cancer diagnosis. The scores were then compared to assess their validity and feasibility of use in international survival comparisons. RESULTS: It was feasible to generate the three comorbidity scores for each jurisdiction, which were found to have good content, face and concurrent validity. Predictive validity was limited and there was evidence that the reliability was questionable. CONCLUSION: The results presented here indicate that interjurisdictional comparability of recorded comorbidity was limited due to probable differences in coding and hospital admission practices in each area. Before the contribution of comorbidity on international differences in cancer survival can be investigated an internationally harmonised comorbidity index is required.

The completeness and timeliness of cancer registration and the implications for measuring cancer burden.

BACKGROUND: Population based cancer registration provides a critical role in disease surveillance in terms of incidence, survival, cancer cluster investigations and prevalence trends, and therefore high levels of completeness and timeliness are required. This study estimates completeness and variation between early and late registrations in the N. Ireland Cancer Registry (NICR) and assesses the implications for reporting cancer incidence and for registry-based research. METHODS: Two main approaches assessed completeness. For the period 2010-2012, incidence reported in the first year of data publication was compared to incidence reported in subsequent years until 2015. Demographic characteristics and survival of incident cases ascertained before the first publication year were compared to those ascertained in subsequent years. The flow method approach was used to estimate completeness annually after the incident year. RESULTS: Overall incidence for all cancers increased between the first year of data publication and subsequent years up to 2015, irrespective of year of diagnosis. Late registrations had poorer survival. The flow method approach estimated the completeness of case ascertainment of NICR data to be 96% complete at five years for all cancers combined. CONCLUSION: The estimated completeness levels for the NICR are comparable to other high quality cancer registries internationally. While data timeliness has little impact on incidence estimates, delays in registration may have implications for specific research studies into incidence and survival. This means that improvements in the timeliness of reporting should be a target for all registries but not at the expense of completeness.

Bereavement outcomes: A quantitative survey identifying risk factors in informal carers bereaved through cancer.

BACKGROUND: Enabling patients to die in their preferred place is important but achieving preferred place of death may increase the informal carer's risk into bereavement. AIM: To determine risk factors of family carers bereaved through cancer in Northern Ireland. DESIGN: These results form part of a larger QUALYCARE-NI study which used postal questionnaires to capture quantitative data on carer's bereavement scores using the Texas Revised Inventory of Grief. SETTING/PARTICIPANTS: Participants were individuals who registered the death of a person between 1 December 2011 and 31 May 2012; where cancer (defined by ICD10 codes C00-D48) was the primary cause; where the deceased was over 18 years of age and death occurred at home, hospice, nursing home or hospital in Northern Ireland. Participants were approached in confidence by the Demography and Methodology Branch of the Northern Ireland Statistics and Research Agency. Those wishing to decline participation were invited to return the reply slip. Non-responders received a second questionnaire 6 weeks after initial invitation. Results indicated that risk factors positively influencing bereavement outcomes included patients having no preference for place of death and carers remaining in employment pre- or post-bereavement. In contrast, patients dying in hospital, carers stopping work, being of lower socio-economic status and close kinship to the deceased negatively affected bereavement scores. Family carers should be adequately supported to continue in employment; priority should be given to assessing the financial needs of families from lower socio-economic areas; and bereavement support should focus on close relatives of the deceased.

Incidence of colorectal cancer in a population-based cohort of patients with Barrett's oesophagus.

OBJECTIVE: Previous studies have shown a positive association between colorectal cancer and Barrett's oesophagus, but this association is disputed. No population-based studies have examined the incidence of this cancer in patients with Barrett's oesophagus. MATERIAL AND METHODS: The present study comprised a population-based cohort of patients with Barrett's oesophagus (constructed using pathology reports of all oesophageal biopsies in Northern Ireland 1993-99; cohort subclassified according to whether specialized intestinal metaplasia (SIM) was present, absent, or not commented on in biopsies). Cases of colorectal cancer were identified by linking with the Northern Ireland Cancer Registry. The comparison group used was the general population in Northern Ireland. RESULTS: A total of 2969 patients with Barrett's oesophagus were followed for a total of 14,014 person-years (mean 4.7 years). SIM was present in 1670 patients (56.2%), absent in 545 (18.4%) and not commented on in 754 (25.4%). Colorectal cancer was diagnosed in 39 patients; 22 patients had cancer diagnosed at least 6 months after diagnosis of Barrett's oesophagus. There was no increased risk of colorectal cancer: the standardized incidence ratio (SIR) for cancer diagnosed at least 6 months after entry into the cohort was 0.82 (95% CI, 0.48-1.17); this risk did not alter with SIM status or gender. To assess a possible effect of diagnostic bias, we calculated SIRs for cancers occurring after at least 3 months, after at least 1 month and at any time after diagnosis of Barrett's oesophagus. These were 0.94 (0.57-1.30), 1.09 (0.69-1.48) and 1.46 (1.00-1.92), respectively. CONCLUSIONS: The incidence of colorectal cancer was not elevated in patients with Barrett's oesophagus. Diagnostic bias may explain why previous studies have found an association.

Have patients with esophagitis got an increased risk of adenocarcinoma? Results from a population-based study.

AIM: To examine an increased risk of esophageal adenocarcinoma is restricted to patients who develop Barrett's esophagus or whether esophagitis per se is a risk factor for adenocarcinoma. METHODS: A population-based cohort of patients with histological evidence of esophagitis without Barrett's esophagus was constructed using electronic pathology reports relating to all esophageal biopsies in Northern Ireland between 1993 and 1996. Person-years of follow-up and incident cases of esophageal cancer were calculated by linking the cohort to death files and the Northern Ireland Cancer Registry records. Standardized incidence ratios (SIR) were calculated for esophageal cancers (adenocarcinoma, squamous cell carcinoma (SCC), and histologically unspecified cancers). RESULTS: A total of 2 013 patients in the cohort provided 13 559 patient-years of follow-up (mean follow-up 6.7 years). None of the patients developed adenocarcinoma. Three patients developed SCC, and six developed histologically unspecified cancers. The SIR for all esophageal cancers and for SCC were 2.73 (95%CI 1.25-5.19) and 2.93 (95%CI 0.61-8.59), respectively. In a sensitivity analysis in which all unspecified esophageal cancers were treated as adenocarcinomas, the SIR for adenocarcinoma was 2.64 (0.97-5.75). CONCLUSION: The risk of adenocarcinoma is not elevated in patients with histological evidence of esophagitis without Barrett's esophagus; however, these patients may have a moderately increased risk of SCC. Further studies are required to confirm these findings, which suggest that Barrett's esophagus, not esophagitis, is the key precursor lesion in the development of adenocarcinoma.