Multicenter comparative multimodality surveillance of women at genetic-familial risk for breast cancer (HIBCRIT study): interim results.

PURPOSE: To prospectively compare clinical breast examination (CBE), mammography, ultrasonography (US), and contrast material-enhanced magnetic resonance (MR) imaging for screening women at genetic-familial high risk for breast cancer and report interim results, with pathologic findings as standard. MATERIALS AND METHODS: Institutional review board of each center approved the research; informed written consent was obtained. CBE, mammography, US, and MR imaging were performed for yearly screening of BRCA1 or BRCA2 mutation carriers, first-degree relatives of BRCA1 or BRCA2 mutation carriers, or women enrolled because of a strong family history of breast or ovarian cancer (three or more events in first- or second-degree relatives in either maternal or paternal line; these included breast cancer in women younger than 60 years, ovarian cancer at any age, and male breast cancer at any age). RESULTS: Two hundred seventy-eight women (mean age, 46 years +/- 12 [standard deviation]) were enrolled. Breast cancer was found in 11 of 278 women at first round and seven of 99 at second round (14 invasive, four intraductal; eight were <or=10 mm in diameter). Detection rate per year was 4.8% (18 of 377) overall; 4.3% (11 of 258) in BRCA1 or BRCA2 mutation carriers and first-degree relatives of BRCA1 or BRCA2 mutation carriers versus 5.9% (seven of 119) in women enrolled because of strong family history; and 5.3% (nine of 169) in women with previous personal breast and/or ovarian cancer versus 4.3% (nine of 208) in those without. In six (33%) of 18 patients, cancer was detected only with MR imaging. Sensitivity was as follows: CBE, 50% (95% confidence interval [CI]: 29%, 71%); mammography, 59% (95% CI: 36%, 78%); US, 65% (95% CI: 41%, 83%); and MR imaging, 94% (95% CI: 82%, 99%). Positive predictive value was as follows: CBE, 82% (95% CI: 52%, 95%); mammography, 77% (95% CI: 50%, 92%); US, 65% (95% CI: 41%, 83%); and MR imaging, 63% (95% CI: 43%, 79%). CONCLUSION: Addition of MR imaging to the screening regimen for high-risk women may enable detection of otherwise unsuspected breast cancers. (c) RSNA, 2007

Treatment challenges in and outside a specialist network setting: Pancreatic neuroendocrine tumours

Pancreatic Neuroendocrine Neoplasms comprise a group of rare tumours with special biology, an often indolent behaviour and particular diagnostic and therapeutic requirements. The specialized biochemical tests and radiological investigations, the complexity of surgical options and the variety of medical treatments that require individual tailoring, mandate a multidisciplinary approach that can be optimally achieved through an organized network. The present study describes currents concepts in the management of these tumours as well as an insight into the challenges of delivering the pathway in and outside a Network

Treatment challenges in and outside a network setting: Head and neck cancers

Head and neck cancer (HNC) is a rare disease that can affect different sites and is characterized by variable incidence and 5-year survival rates across Europe. Multiple factors need to be considered when choosing the most appropriate treatment for HNC patients, such as age, comorbidities, social issues, and especially whether to prefer surgery or radiation-based protocols. Given the complexity of this scenario, the creation of a highly specialized multidisciplinary team is recommended to guarantee the best oncological outcome and prevent or adequately treat any adverse effect. Data from literature suggest that the multidisciplinary team-based approach is beneficial for HNC patients and lead to improved survival rates. This result is likely due to improved diagnostic and staging accuracy, a more efficacious therapeutic approach and enhanced communication across disciplines. Despite the benefit of MTD, it must be noted that this approach requires considerable time, effort and financial resources and is usually more frequent in highly organized and high-volume centers. Literature data on clinical research suggest that patients treated in high-accrual centers report better treatment outcomes compared to patients treated in low-volume centers, where a lower radiotherapy-compliance and worst overall survival have been reported. There is general agreement that treatment of rare cancers such as HNC should be concentrated in high volume, specialized and multidisciplinary centers. In order to achieve this goal, the creation of international collaboration network is fundamental. The European Reference Networks for example aim to create an international virtual advisory board, whose objectives are the exchange of expertise, training, clinical collaboration and the reduction of disparities and enhancement of rationalize migration across Europe. The purpose of our work is to review all aspects and challenges in and outside this network setting planned for the management of HNC patients

Testicular germ-cell tumours and penile squamous cell carcinoma: Appropriate management makes the difference

Germ-cell tumours (GCT) of the testis and penile squamous cell carcinoma (PeSCC) are a rare and a very rare uro-genital cancers, respectively. Both tumours are well defined entities in terms of management, where specific recommendations - in the form of continuously up-to-dated guide lines-are provided. Impact of these tumour is relevant. Testicular GCT affects young, healthy men at the beginning of their adult life. PeSCC affects older men, but a proportion of these patients are young and the personal consequences of the disease may be devastating. Deviation from recommended management may be a reason of a significant prognostic worsening, as proper treatment favourably impacts on these tumours, dramatically on GCT and significantly on PeSCC. RARECAREnet data may permit to analyse how survivals may vary according to geographical areas, histology and age, leading to assume that non-homogeneous health-care resources may impact the cure and definitive outcomes. In support of this hypothesis, some epidemiologic datasets and clinical findings would indicate that survival may improve when appropriate treatments are delivered, linked to a different accessibility to the best health institutions, as a consequence of geographical, cultural and economic barriers. Finally, strong clues based on epidemiological and clinical data support the hypothesis that treatment delivered at reference centres or under the aegis of a qualified multi-institutional network is associated with a better prognosis of patients with these malignancies. The ERN EURACAN represents the best current European effort to answer this clinical need

Lower incidence rates but thicker melanomas in Eastern Europe before 1992: A comparison with Western Europe

The objective of this study was to investigate the epidemiology of melanoma across Europe with regard to Breslow thickness and body-site distribution. Incidence data from Cancer Incidence in 5 Continents and the EUROCARE-melanoma database were used: 28 117 melanoma cases from 20 cancer registries in 12 European countries, diagnosed between 1978 and 1992. Regression analysis and general linear modelling were used to analyse the data. Melanomas in Eastern Europe were on average 1.4 mm thicker (P<0.05) than in Western Europe and appeared more often on the trunk. From 1978 to 1992, their Breslow thickness had decreased in Western but not Eastern Europe. There was a latitude gradient in incidence, with highest rates in southern regions in Eastern Europe and an inverse gradient in Western Europe, with highest rates in the North. Mortality:incidence ratios were less favourable in southern parts across Europe, especially in Eastern Europe. If Eastern European populations copy the sunbathing behaviour of the West it is likely that in the near future a higher melanoma incidence can be expected there

Survival for eight major cancers and all cancers combined for European adults diagnosed in 1995-99: results of the EUROCARE-4study

Background: EUROCARE is the largest population-based cooperative study on survival of patients with cancer. The EUROCARE project aims to regularly monitor, analyse, and explain survival trends and between-country diff erences in survival. This report (EUROCARE-4) presents survival data for eight selected cancer sites and for all cancers combined, diagnosed in adult (aged 6515 years) Europeans in 1995\u201399 and followed up until the end of 2003. Methods: We analysed data from 83 cancer registries in 23 European countries on 2 699 086 adult cancer cases that were diagnosed in 1995\u201399 and followed up to December, 2003. We calculated country-specifi c and mean-weighted age-adjusted 5-year relative survival for eight major cancers. Additionally, case-mix-adjusted 5-year survival for all cancers combined was calculated by countries ranked by total national expenditure on health (TNEH). Changes to survival were analysed relative to cases diagnosed in 1990\u201394. Findings: Mean age-adjusted 5-year relative survival for colorectal (53\ub78% [95% CI 53\ub73\u201354\ub71]), lung (12\ub73% [12\ub71\u201312\ub75]), breast (78\ub79% [78\ub76\u201379\ub72]), prostate (75\ub77% [75\ub72\u201376\ub72]), and ovarian (36\ub73% [35\ub77\u201337\ub70]) cancer was highest in Nordic countries (except Denmark) and central Europe, intermediate in southern Europe, lower in the UK and Ireland, and worst in eastern Europe. Survival for melanoma (81\ub76% [81\ub70\u201382\ub73]), cancer of the testis (94\ub72% [93\ub74\u201395\ub70]), and Hodgkin\u2019s disease (80\ub70% [79\ub70\u201381\ub70]) varied little with geography. All-cancer survival correlated with TNEH for most countries. Denmark and UK had lower all-cancer survival than countries with similar TNEH; Finland had high all-cancer survival, but moderate TNEH. Survival increased and intercountry survival diff erences narrowed between the data for 1990\u201394 and 1995\u201399 for, notably, Hodgkin\u2019s disease (range 66\ub71\u201382\ub79 [IQR 72\ub72\u201378\ub76] vs 74\ub70\u201383\ub79 [78\ub76\u201381\ub79]), colorectal (29\ub74\u201356\ub77 [45\ub78\u201354\ub71] vs 38\ub78\u201359\ub77 [50\ub77\u201357\ub75]), and breast (61\ub77\u201382\ub77 [72\ub73\u201378\ub73] vs 69\ub73\u201387\ub76 [76\ub76\u201382\ub77]) sites. Interpretation: Increases in survival and decreases in geographic diff erences over time, which are mainly due to improvements in health-care services in countries with poor survival, might indicate better cancer care. Wealthy countries with high TNEH generally had good cancer outcomes, but those with conspicuously worse outcomes than those with similar TNEH might not be allocating health resources effi ciently

Electronic availability of EUROCARE-3 data: a tool for further analysis.

The EUROCARE-3 CD-ROM has been developed to provide more detailed data with respect to those published in the monograph. The CD-ROM provides estimates of age-specific and age-standardised survival figures, cumulative and interval-specific survival, observed and relative survival for 47 cancer sites or combinations of sites, based on >4 million adult cancer patients diagnosed from 1983 to 1994 and reported from 56 European cancer registries. In addition, the CD-ROM provides observed survival proportions for 25 childhood cancer entities based on 23,000 young patients diagnosed from 1990 to 1994. Survival indicators, corresponding standard errors and confidence intervals can be selected according to cancer site, registry or country, sex, age class and disease duration. Basic graphical display and export facilities have also been provided. As an example of how to use this CD-ROM, this paper will report a descriptive analysis of relative survival patterns for all cancers combined, by age, sex and country. The EUROCARE-3 CD-ROM can be ordered free of charge or directly downloaded at http://www.eurocare.it

Survival of children with Wilms' tumour in Europe.

A total 2535 cases of Wilms' tumours registered in children aged 0--14 years by 34 population-based cancer registries in 16 countries of Europe in 1978--1992 and followed-up until the end of 1994 were included in this EUROCARE study. Overall 5-year observed survival of all children diagnosed in 1985--1989 was 83%, 95% confidence interval (CI) 80--85. Relatively large differences were observed between the European countries, with significantly lower survival of patients registered in the formerly socialist countries, Estonia, Poland and Slovakia. Overall European survival was slightly lower in comparison with results reported from the USA and Australia, which demonstrate a potential for improvement. Over the study period, overall survival adjusted for age, sex and country has increased significantly. This favourable trend is attributed primarily to improvements in treatment, particularly to the introduction of new chemotherapeutic agents

The EUROCARE-3 database: methodology of data collection, standardisation, quality control and statistical analysis.

The EUROCARE database contains data on 6.5 million cancer patients diagnosed from 1978 to 1994 in populations covered by 67 cancer registries in 22 European countries. The quality-checked entries specify age, sex, diagnosis date, cancer site, morphology, microscopic confirmation and vital status, as well as containing broad indicators of stage. For EUROCARE-3, which refers to diagnoses from 1990 to 1994, 3389 cases with major data problems and 142,525 second or subsequent cancers were removed, leaving more than 2 million cases for analysis. From these data, observed and relative survival for each cancer site and country were calculated at 1, 3 and 5 years from diagnosis. Overall European survival for each cancer site and for all cancers combined were calculated combining country-specific survival figures. Overall, 1.1% of cases were lost to follow-up, 4.2% were known from death certificates only and 1.2% were known at autopsy only. The percentage of microscopically confirmed cases varied with cancer site and country, and was always higher in northern European countries. Comparison of quality indicators for the EUROCARE-3 database with earlier EUROCARE databases indicates that data quality and standardisation have improved