Descriptive epidemiology of Kaposi sarcoma in Europe. Report from the RARECARE project

Kaposi sarcoma (KS) is a virus-related malignancy which most frequently arises in skin, though visceral sites can also be involved. Infection with Kaposi sarcoma herpes virus (KSHV or HHV-8) is required for development of KS. Nowadays, most cases worldwide occur in persons who are immunosuppressed, usually because of HIV infection or as a result of therapy to combat rejection of a transplanted organ, but classic Kaposi sarcoma is predominantly a disease of the elderly without apparent immunosuppression. We analyzed 2667 KS incident cases diagnosed during 1995–2002 and registered by 75 population-based European cancer registries contributing to the RARECARE project. Total crude and age-standardized incidence rate was 0.3 per 100,000 per year with an estimated 1642 new cases per year in the EU27 countries. Age-standardized incidence rate was 0.8 per 100,000 in Southern Europe but below 0.3 per 100,000 in all other regions. The elevated rate in southern Europe was attributable to a combination of classic Kaposi sarcoma in some Mediterranean countries and the relatively high incidence of AIDS in several countries. Five-year relative survival for 2000–2002 by the period method was 75%. More than 10,000 persons were estimated to be alive in Europe at the beginning of 2008 with a past diagnosis of KS. The aetiological link with suppressed immunity means that many people alive following diagnosis of KS suffer comorbidity from a pre-existing condition. While KS is a rare cancer, it has a relatively good prognosis and so the number of people affected by it is quite large. Thus it provides a notable example of the importance of networking in diagnosis, therapy and research for rare cancers.     

Methodological aspects of estimating rare cancer prevalence in Europe: The experience of the RARECARE project

This paper describes the usage and the performance evaluation of the completeness index method in the ‘Surveillance of Rare Cancers in Europe project’ (RARECARE) for estimating rare cancer prevalence in Europe. The 15-year prevalence at 1st January 2003 for 255 cancers is obtained from a pool of 22 RARECARE cancer registries (CRs). Incidence and survival models are applied to the RARECARE database to estimate the parameters from which the completeness indices are calculated. Complete prevalence is obtained adjusting the observed 15-year prevalence by the completeness index, to account for those cancer survivors diagnosed before the CR activity started. Main factors influencing the performance of the completeness index method for rare cancers are the same as for common cancers: age distribution of incidence and lethality of the cancer. For cancers occurring in the elderly, with low survival rates and consequently a restricted number of long-term survivors we obtained completeness indices higher than 0.9. Values lower than 0.7 correspond to those cancers with good prognosis and/or incidence more concentrated at the younger ages, indicating that 15 years of follow up are insufficient to detect all prevalent cases. Validation analysis shows that for a restricted subgroup of rare cancers with very low incidence and low survival, the completeness indices were not able to adequately correct the observed prevalence even considering a registration period of 20 years. On average, sensitivity analyses show a slight overestimation of complete prevalence for rare and common cancers whose increasing incidence is known in literature. RARECARE is the largest project on rare cancers conducted to date. Improving health care programs for cancer survivors is a public health priority and prevalence data which provides important information in this field should be regularly asked to Member States and included in the EU health statistics.     

Increased incidence of rare cancers and varied age distributions by cancer group: A population-based cancer registry study in Hiroshima Prefecture,Japan

BackgroundEpidemiological characteristics of many types of rare cancers are limited especially in Asia. Therefore, this study aimed to describe the burden and changing time trends of rare cancers in Hiroshima, Japan.MethodsThe internationally agreed RARECAREnet list of rare cancers was used to identify patients diagnosed with cancers from 2005 to 2015 who were registered in the Hiroshima Prefecture Cancer Registry. Quality indicators specific to rare cancers were assessed by cancer grouping. Crude incidence rates (IRs) and age-standardized rates (ASRs) were calculated for 216 single cancers (rare and common) included in the list. A joinpoint regression was used to analyze age distribution and time trends in the ASRs for 12 internationally agreed rare cancer families. Quality indicators, ASRs, and IRs in Japan were identified to examine IR differences and the effects on data accuracy.ResultsThe 231,328 cases were used to calculate the IRs of each cancer. Epithelial tumors in rare families increased with age, but nonepithelial tumors occurred at any age. The proportion of rare cancer families to total cancers was stable. The time trend for families of head and neck cancers (annual percent change and 95 % confidence interval: 2.4 %; 1.2–3.7 %), neuroendocrine tumors (6.6 %; 5.1–8.1 %), and hematological cancers (4.3 %; 3.2–5.5 %) markedly increased.ConclusionThe ASRs of several rare cancers increased because of increased knowledge of these diseases, improved diagnostic techniques, and aggressive diagnoses.     

Cancer incidence in southern Iran, 1998-2002: results of population-based cancer registry

Purpose: The main aim of this study was to obtain population-based cancer incidence data for the entire population of Fars province in Iran, and to compare these rates with those obtained from a previous study in the same population ten years previously. Methods: Data were collected on all patients in major cities of Fars province who were diagnosed with cancer between 1998 and 2002. The data were computerized using SPSS (Chicago, IL) software, version 13.0, and MS EXCEL (Microsoft, Redmond, WA) software with Persian fonts. The results are presented as incidence rates of cases by site, sex, age, crude rates, and age-standardized rates per 100,000 person-years (ASRs), using the direct method of standardization to the world population. Results: During the 5-year study period, 8359 new cancer cases were registered. Diagnosis of cancer was based on histopathological criteria in 86.7%, clinical or radiological criteria in 9.4% and death certificate only in 3.9% of cases. According to the calculated ASRs, the 5 most frequent cancers in women were breast (13 per 100,000), stomach (4.4 per 100,000), lung and bronchus (2.9 per 100,000), uterus (2.7 per 100,000), and colon and rectum (2.6 per 100,000); and in men, the 5 most frequent types were stomach (9.2 per 100,000), bladder (6.8 per 100,000), lung and bronchus (6.3 per 100,000), lymphocytic leukemia (4.1 per 100,000), and skin melanoma (3.8 per 100,000). The ASR for all cancers in men was 64.5 per 100,000, and that for women was 55.5 per 100,000. Conclusion: Considering the limitations of this study, our results should be taken as the minimum incidence rates of cancers in Fars province, southern Iran. Significant differences were observed between the two study periods. However, we most likely have underestimated the frequencies of some tumors.     

Cancer burden in four countries of the Middle East Cancer Consortium (Cyprus; Jordan; Israel; Izmir (Turkey)) with comparison to the United States surveillance; epidemiology and end results program

It is important that population-based cancer registries provide accurate and reliable data for public health purposes. These data are essential data for planning of cancer control and prevention. In this study, we examined cancer incidence rates (year 2005–2010) in four MECC registries (Cyprus, Jordan, Israel, Izmir (Turkey)) and compared with the rates in the US. The overall age-standardized incidence rates for males were highest in the US followed by Israeli Jews, Izmir (Turkey), Cyprus, Israeli Arabs, and lowest in Jordan. In women the rates of cancer of all sites were also highest in US women followed by Israeli Jews, Cyprus, Israeli Arabs, Izmir (Turkey), and lowest in Jordan. It is of interest that although site-specific cancer rates differ between the countries studied, prostate, lung and colorectal cancers are within the five most common cancers males in all countries studied. In females, breast colorectal and endometrium cancers are three of the five most common cancers in females in all countries studied. The results presented in this paper can have implications for opportunities in cancer control and prevention in these countries. Future studies on individual cancer sites with highest rates in these Countries are currently underway.     

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

BackgroundPopulation 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.MethodsTwo 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.ResultsOverall 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.ConclusionThe 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.     

Cancer in adolescents and young adults: National incidence and characteristics in Japan

ObjectiveAdolescents and young adults (AYA) with cancer are confronted with unique challenges in areas of paramount concern within their age group, such as fertility, education, career, and delayed and long-term effects of treatment. However, the extent and depth of the problem has never been examined in the Japanese population. The aim of this study was to describe the status of cancer patients in the AYA population, using data from the hospital-based cancer registry (HBCR).Study designPatients included in the HBCR from January 2011 to December 2014 were included in this study to evaluate the incidence and cancer distribution trends among AYA. The total number and the proportion of AYA (15–39 years of age) stratified by sex, age, and cancer type were obtained. The incidence of age-specific cancer among AYA was also calculated.ResultsWe identified 30,394 male (35.1%) and 56,100 female (64.9%) cancer patients in the population, which collectively constituted about 3% of all invasive cancer cases. The incidence of cancer in AYA was estimated as 86.2 per 100,000 per year, and increased with age. The most affected population was women between 35 and 39 years of age (35%). Breast cancer was the most common type of cancer, followed by cervical, uterine, and thyroid cancers.ConclusionA substantial number of AYA are diagnosed with cancer every year. The distribution of cancer types in AYA was dependent on age and sex. These diversities in cancer types can inform researchers and policy makers to fine-tune their studies and policies.     

Profile of cancer in the Eastern Mediterranean region: The need for action

BackgroundMany countries in the Eastern Mediterranean region (EMR) are undergoing marked demographic and socioeconomic transitions that are increasing the cancer burden in region. We sought to examine the national cancer incidence and mortality profiles as a support to regional cancer control planning in the EMR.MethodsGLOBOCAN 2012 data were used to estimate cancer incidence and mortality by country, cancer type, sex and age in 22 EMR countries. We calculated age-standardized incidence and mortality rates (per 100,000) using direct method of standardization.ResultsThe cancer incidence and mortality rates vary considerably between countries in the EMR. Incidence rates were highest in Lebanon (204 and 193 per 100,000 in males and females, respectively). Mortality rates were highest in Lebanon (119) and Egypt (121) among males and in Somalia (117) among females. The profile of common cancers differs substantially by sex. For females, breast cancer is the most common cancer in all 22 countries, followed by cervical cancer, which ranks high only in the lower-income countries in the region. For males, lung, prostate, and colorectal cancer in combination represent almost 30% of the cancer burden in countries that have attained very high levels of human development.ConclusionsThe most common cancers are largely amenable to preventive strategies by primary and/or secondary prevention, hence a need for effective interventions tackling lifestyle risk factors and infections. The high mortality observed from breast and cervical cancer highlights the need to break the stigmas and improve awareness surrounding these cancers.     

Japanese cancer screening programs during the COVID-19 pandemic: Changes in participation between 2017-2020

BackgroundThe impact of the coronavirus disease 2019 (COVID-19) pandemic on cancer screening participation is a global concern. A national database of screening performance is available in Japan for population-based cancer screening, estimated to cover approximately half of all cancer screenings.MethodsUtilizing the fiscal year (FY) 2017–2020 national database, the number of participants in screenings for gastric cancer (upper gastrointestinal [UGI] series or endoscopy), colorectal cancer (fecal occult blood test), lung cancer (chest X-ray), breast cancer (mammography), and cervical cancer (Pap smear) were identified. The percent change in the number of participants was calculated.ResultsCompared with the pre-pandemic period (FY 2017–2019), in percentage terms FY 2020 recorded the largest decline in gastric cancer UGI series (2.82 million to 1.91 million, percent change was −32.2 %), followed by screening for breast cancer (3.10 million to 2.57 million, percent change was −17.2 %), lung cancer (7.92 million to 6.59 million, percent change was −16.7 %), colorectal cancer (8.42 million to 7.30 million, percent change was −13.4 %), cervical cancer (4.26 million to 3.77 million, percent change was −11.6 %), and gastric cancer via endoscopy (1.02 million to 0.93 million, percent change was −9.0 %).ConclusionThe number of participants in population-based screenings in Japan decreased by approximately 10–30 % during the pandemic. The impact of these declines on cancer detection or mortality should be carefully monitored.     

Probabilities of dying from cancer and other causes in French cancer patients based on an unbiased estimator of net survival: A study of five common cancers

BackgroundNet survival is the survival that would be observed if cancer were the only possible cause of death. Although it is an important epidemiological tool allowing temporal or geographical comparisons, it cannot inform on the “crude” probability of death of cancer patients; i.e., when taking into account other possible causes of deaths.MethodsIn this work, we provide estimates of the crude probabilities of death from cancer and from other causes as well as the probability of being alive up to ten years after cancer diagnosis according to the age and year of diagnosis. Based on a flexible excess hazard model providing unbiased estimates of net survival, our methodology avoids the pitfalls associated with the use of the cause of death. We used data from FRANCIM, the French network of cancer registries, and studied five common cancer sites: head and neck, breast, prostate, lung, and colorectal cancers.ResultsFor breast, prostate, and colorectal cancers, the impact of the other causes on the total probability of death increased with the age at diagnosis whereas it remained negligible for lung and head and neck cancers whatever the age. For breast, prostate, and colorectal cancer, the more recently was the cancer diagnosed, the less was the probability of death from cancer.ConclusionThe crude probability of death is an intuitive concept that may prove particularly useful in choosing an appropriate treatment, or refining the indication of a screening strategy by allowing the clinician to estimate the proportion of cancer patients who will die specifically from cancer.     

Active follow-up versus passive linkage with cancer registries for case ascertainment in a cohort

BackgroundAscertaining incident cancers is a critical component of cancer-focused epidemiologic cohorts and of cancer prevention trials. Potential methods: for cancer case ascertainment include active follow-up and passive linkage with state cancer registries. Here we compare the two approaches in a large cancer screening trial.MethodsThe Prostate, Lung, Colorectal and Ovarian (PLCO) cancer screening trial enrolled 154,955 subjects at ten U.S. centers and followed them for all-cancer incidence. Cancers were ascertained by an active follow-up process involving annual questionnaires, retrieval of records and medical record abstracting to ascertain and confirm cancers. For a subset of centers, linkage with state cancer registries was also performed. We assessed the agreement of the two methods in ascertaining incident cancers from 1993 to 2009 in 80,083 subjects from six PLCO centers where cancers were ascertained both by active follow-up and through linkages with 14 state registries.ResultsThe ratio (times 100) of confirmed cases ascertained by registry linkage compared to active follow-up was 96.4 (95% CI: 95.1–98.2). Of cancers ascertained by either method, 86.6% and 83.5% were identified by active follow-up and by registry linkage, respectively. Of cancers missed by active follow-up, 30% were after subjects were lost to follow-up and 16% were reported but could not be confirmed. Of cancers missed by the registries, 27% were not sent to the state registry of the subject’s current address at the time of linkage.ConclusionLinkage with state registries identified a similar number of cancers as active follow-up and can be a cost-effective method to ascertain incident cancers in a large cohort.     

The currently declining incidence of cancer of unknown primary

Cancer of unknown primary (CUP) is a heterogeneous group of metastatic cancers for which a primary tumor cannot be identified after a standardized work-up. The biology of CUP has not been fully elucidated and epidemiologic data may be helpful in this regard. The variations in the incidence-rate over time and between countries reflect changes in the risk factors for CUP, incidence trends of the primary tumors that potentially contribute to the burden of CUP and changes in the diagnostic technologies and practice. CUP accounted for 3–5% of cancers in the historical series but its incidence seems to decline in the recent publications. This paper reviews the published cancer-registry studies in order to identify and understand the variations in the incidence-rates of CUP.     

Difference in net survival using regional and national life tables in Japan

BackgroundNational life table is commonly used for estimating cancer net survival. However, the national life table does not reflect condition of people in local area accurately, because there are disparities in cancer mortality rates among the local area in many cases. We investigated magnitude of difference in cancer net survival using the local area in Japan and Japanese life tables.MethodsWe analyzed data from 32,942 cancer patients diagnosed between 2010 and 2012 in Aomori prefecture, Japan. Expected survival rates in Aomori (ESA) and Japan (ESJ) were estimated based on the life table of each area. Five-year net survival rates using ESA and the ESJ were estimated using the Pohar-Perme method.ResultsThe difference between net survival rates using the ESA (NSA) and the ESJ (NSJ) were larger than in men (0.3－3.0%) than in women (0.1－0.8%). The largest difference in the net survival rate was observed in prostate cancer patients, because the difference in the expected survival in oldest old men was remarkable.ConclusionTwo factors affected the difference in the net survival rates resulting from the sensitivity analysis. The difference was larger (1) among older patients or (2) with a longer observation period (person-years).     

Population-based incidence of lymphoid neoplasms: Twenty years of epidemiological data in the Girona province,Spain

BackgroundThe aim of this study was to describe incidence patterns of lymphoid neoplasms in the Girona province (Spain) (1996–2015), and to predict the number of cases in Spain during 2020.MethodsData were extracted from the Girona cancer registry. Incident cases were classified using the ICD-O-3, third revision, and grouped according to the WHO 2008 classification scheme. Age-adjusted incidence rates to the European standard population (ASRE) were estimated and incidence trends were modeled using Joinpoint.Results4367 lymphoid neoplasms were diagnosed in the Girona province. The ASRE for overall lymphoma was 37.1 (95% CI: 36.0; 38.2), with a marked male predominance in almost all subtypes. During 1996–2015, incidence trends remained stable for broader lymphoma categories. According to our predictions, 17,950 new cases of LNs will be diagnosed in Spain in 2020.ConclusionsThis ‘real-world’ data will provide valuable information to better inform etiological hypotheses and plan future health-care services.     

Patterns of cancer in geographic and endogamous subdivisions of the Hutterite Brethren of Canada

The Hutterite Brethren comprise a religious isolate and live on communal agricultural farms (colonies) in North America. In 1976 there were approximately 15,000 Canadian Brethren living in 179 colonies of the three endogamous subdivisions, the Dariusleut, Lehrerleut, and Schmiedeleut. Dariusleut and Lehrerleut colonies are located in both Alberta and Saskatchewan, and the Schmiedeleut are in Manitoba. Brethren were identified on population-based cancer registries of the three Prairie Provinces and among death registrations in the vital statistics of Alberta and Saskatchewan. The method of ascertainment was by a search for the 15 contemporary surnames and verification by address. 89 male and 91 female Brethren were identified who had cancer during the period, 1956--1975. The numbers of observed cancers were less than expected from provincial incidence rates for males and females in each province. The largest deficits were for female Brethren in Manitoba and Saskatchewan. There is a marked deficiency of cancer of the uterine cervix among female Brethren. In males there is a significant deficit of lung cancer. The Hutterite way of life contributes to a low risk for cancers of smoking-associated sites. However, there is evidence that male Brethren in Alberta may be at relatively increased risk for stomach cancer and leukemias. The site distribution patterns of cancers among the three endogamous leut are similar.     

Effects of the length of central cancer registry operations on identification of subsequent cancers and on survival estimates

BackgroundPopulation-based cancer survival analyses have traditionally been based on the first primary cancer. Recent studies have brought this practice into question, arguing that varying registry reference dates affect the ability to identify earlier cancers, resulting in selection bias. We used a theoretical approach to evaluate the extent to which the length of registry operations affects the classification of first versus subsequent cancers and consequently survival estimates.MethodsSequence number central was used to classify tumors from the New York State Cancer Registry, diagnosed 2001–2010, as either first primaries (value = 0 or 1) or subsequent primaries (≥2). A set of three sequence numbers, each based on an assumed reference year (1976, 1986 or 1996), was assigned to each tumor. Percent of subsequent cancers was evaluated by reference year, cancer site and age. 5-year relative survival estimates were compared under four different selection scenarios.ResultsThe percent of cancer cases classified as subsequent primaries was 15.3%, 14.3% and 11.2% for reference years 1976, 1986 and 1996, respectively; and varied by cancer site and age. When only the first primary was included, shorter registry operation time was associated with slightly lower 5-year survival estimates. When all primary cancers were included, survival estimates decreased, with the largest decreases seen for the earliest reference year.ConclusionsRegistry operation length affected the identification of subsequent cancers, but the overall effect of this misclassification on survival estimates was small. Survival estimates based on all primary cancers were slightly lower, but might be more comparable across registries.     

Future of cancer incidence in Shanghai,China: Predicting the burden upon the ageing population

AimThe age-specific cancer patterns have changed significantly over the last few decades in urban Shanghai. Predicting the cancer incidence in an ageing population can help to anticipate future resource needs, evaluate primary prevention strategies, and inform further research studies.Materials and MethodsAnnual cancer cases and population data from 1988 to 2013 were collected from Shanghai Cancer Registry. A Bayesian age-period-cohort model was applied to project the future cancer incidence with demographical changes from 2014 to 2025.ResultsFrom 1988 through 2013, the urban population aged < 65 years decreased by 19.5%, while the population aged ≥ 65 years increased by 58.4%. In the same period, cancer cases increased by 66.0% (from 8315 to 13,806) and 88.6% (from 7448 to 14,048) in these two populations, respectively. From 2014–2025, the population size is expected to decrease by an additional 29.6% in people aged < 65 years, while it will increase by an additional 68.3% in people aged ≥ 65 years. Correspondingly, the model predicts an 87.5% and 143.4% increase in cancer cases for these two populations, respectively. The most pronounced increase was found in thyroid cancer in both sexes, followed by prostate, kidney, and colon cancer in men. In women, lung, kidney, and cervical cancer in women was expected to increase.ConclusionsThe number of cancer cases in urban Shanghai, especially in older people, is expected to significantly increase in the next decade. Particular strategies targeting the elderly are required to combat the cancers.     

Epidemiological trends of neuroendocrine tumours over three decades in Queensland,Australia

IntroductionWhile neuroendocrine tumours (NETs) account for only a small proportion of cancer diagnoses, incidence has been rising over time. We examined incidence, mortality and survival over three decades in a large population-based registry study.MethodsThis retrospective study included all cases (n = 4580) of NETs diagnosed from 1986 to 2015 in Queensland, Australia. We examined directly age-standardised incidence and mortality rates. The impact on overall survival according to demographic factors and primary site was modelled using multivariable Cox proportional hazards regression (HR). Cause-specific and relative survival were estimated using the Kaplan-Meier survival function.ResultsAnnual incidence increased from 2.0 in 1986 to 6.3 per 100,000 in 2015, while mortality remained stable. The most common primary site was appendix followed by lung, small intestine and rectum. Rectal, stomach, appendiceal and pancreatic NETs had the greatest rate increase, while lung NETs decreased over the same period. Five-year cause-specific survival improved from 69.4% during 1986–1995 to 92.6% from 2006 to 2015. Survival was highest for appendiceal and rectal NETs and lowest for pancreas and unknown primary sites. The risk of dying within five years of diagnosis was about 40% higher for males (HR = 1.41, 95%CI 1.20–1.65) and significantly higher for patients aged over 40 years compared to younger patients (p < 0.001).ConclusionThis study, including 30 years of data, found significantly increasing rates of NETs and confirms results from elsewhere. Increasing survival over time in this study, likely reflects increased awareness, improvements in diagnostic imaging, greater use of endoscopy and colonoscopy, and the development of new therapies.     

Roles of cancer stem cells in gastrointestinal cancers

Cancer stem cells (CSCs) are the main cause of tumor growth, invasion, metastasis and recurrence. Recently, CSCs have been extensively studied to identify CSC-specific surface markers as well as signaling pathways that play key roles in CSCs self-renewal. The involvement of CSCs in the pathogenesis of gastrointestinal (GI) cancers also highlights these cells as a priority target for therapy. The diagnosis, prognosis and treatment of GI cancer have always been a focus of attention. Therefore, the potential application of CSCs in GI cancers is receiving increasing attention. This review summarizes the role of CSCs in GI cancers, focusing on esophageal cancer, gastric cancer, liver cancer, colorectal cancer, and pancreatic cancer. In addition, we propose CSCs as potential targets and therapeutic strategies for the effective treatment of GI cancers, which may provide better guidance for clinical treatment of GI cancers.