Estimating 10-year risk of lung and breast cancer by occupation in Switzerland.

Introduction: Lung and breast cancer are important in the working-age population both in terms of incidence and costs. The study aims were to estimate the 10-year risk of lung and breast cancer by occupation and smoking status and to create easy to use age-, and sex-specific 10-year risk charts. Methods: New lung and breast cancer cases between 2010 and 2014 from all 5 cancer registries of Western Switzerland, matched with the Swiss National Cohort were used. The 10-year risks of lung and breast cancer by occupational category were estimated. For lung cancer, estimates were additionally stratified by smoking status using data on smoking prevalence from the 2007 Swiss Health Survey. Results: The risks of lung and breast cancer increased with age and were the highest for current smokers. Men in elementary professions had a higher 10-year risk of developing lung cancer compared to men in intermediate and managerial professions. Women in intermediate professions had a higher 10-year risk of developing lung cancer compared to elementary and managerial professions. However, women in managerial professions had the highest risk of developing breast cancer. Discussion: The 10-year risk of lung and breast cancer differs substantially between occupational categories. Smoking creates greater changes in 10-year risk than occupation for both sexes. The 10-year risk is interesting for both patients and professionals to inform choices related to cancer risk, such as screening and health behaviors. The risk charts can also be used as public health indicators and to inform policies to protect workers.

Breast cancer and occupation: Non-parametric and parametric net survival analyses among Swiss women (1990-2014).

Introduction: Occupation can contribute to differences in risk and stage at diagnosis of breast cancer. This study aimed at determining whether occupation, along with skill level and the socio-professional category, affect the breast cancer survival (BCS) up to 10 years after diagnosis. Materials and methods: We used cancer registry records to identify women diagnosed with primary invasive breast cancer in western Switzerland over the period 1990-2014 and matched them with the Swiss National Cohort. The effect of work-related variables on BCS was assessed using non-parametric and parametric net survival methods. Results: Study sample included 8,678 women. In the non-parametric analysis, we observed a statistically significant effect of all work-related variables on BCS. Women in elementary occupations, with low skill level, and in paid employment not classified elsewhere, had the lowest BCS, while professionals, those with the highest skill level and belonging to top management and independent profession category had the highest BCS. The parametric analysis confirmed this pattern. Considering elementary occupations as reference, all occupations but Craft and related trades had a hazard ratio (HR) below 1. Among professionals, technicians and associate professionals, and clerks, the protective effect of occupation was statistically significant and remained unchanged after adjustment for age, calendar period, registry, nationality, and histological type. After adjusting for tumor stage, the HRs increased only slightly, though turned non-significant. The same effect was observed in top management and independent professions and supervisors, low level management and skilled laborers, compared to unskilled employees. Conclusion: These results suggest that work-related factors may affect BCS. Yet, this study was conducted using a limited set of covariates and a relatively small study sample. Therefore, further larger studies are needed for more detailed analyses of at risk occupations and working conditions and assessing the potential interaction between work-related variables and tumor stage.

Work-Related Factors and Lung Cancer Survival: A Population-Based Study in Switzerland (1990-2014).

While previous Swiss studies have demonstrated differences in lung cancer mortality between occupational groups, no estimates are available on the association of occupation-related factors with lung cancer survival. This study aimed at determining whether occupation or work-related factors after diagnosis affect lung cancer survival. We used cancer registry records to identify lung cancer patients diagnosed between 1990 and 2014 in western Switzerland (n = 5773) matched with the Swiss National Cohort. The effect of occupation, the skill level required for the occupation, and the socio-professional category on 5-year lung cancer survival was assessed using non-parametric and parametric methods, controlling for histological type and tumour stage. We found that the net survival varied across skill levels and that the lowest skill level was associated with worse survival in both men and women. In the parametric models with minimal adjustment, we identified several occupational groups at higher risk of mortality compared to the reference category, particularly among men. After adjustment for histological type of lung cancer and tumour stage at diagnosis, most hazard ratios remained higher than 1, though non-statistically significant. Compared to top managers and self-employed workers, workers in paid employment without specific information on occupation were identified as the most at-risk socio-professional category in nearly all models. As this study was conducted using a relatively small sample and limited set of covariates, further studies are required, taking into account smoking habits and administrated cancer treatments. Information on return to work and working conditions before and after lung cancer diagnosis will also be highly valuable for analysing their effect on net lung cancer survival in large nationwide or international studies. Such studies are essential for informing health and social protection systems, which should guarantee appropriate work conditions for cancer survivors, beneficial for their quality of life and survival.

High burden of invasive and non-invasive cancer among women aged 20-49 years: the situation in Geneva, Switzerland.

BACKGROUND: The pattern of cancer among young adults aged 20-49 years is different than that observed in other age groups, most notably women present higher rates than those observed among men. Estimations of the burden of cancer disease among women of this age group, however, rarely include both invasive and non-invasive disease. METHODS: We calculated incidence rates of invasive and non-invasive cancers for women and men aged 20-49 years and by cancer site for the period 2014-2018 in the canton of Geneva, Switzerland using data from the population-based cancer registry. RESULTS: Between 2014 and 2018, the incidence rates of invasive and non-invasive cancers among women were 177.6/100,000 and 166.4/100,000, respectively. The rates among men of the same age were 110.6/100,000 and 31.8/100,000, respectively. Just three cancers, breast, thyroid and melanoma accounted for 70% of all invasive cancers among women. In situ cervical cancer represented over 70% of non-invasive disease. CONCLUSION: Cancer among women aged 20-49 years is quite frequent. This is primarily a result of increasing risk, increased diagnosis or both and highlights the need for better primary prevention strategies, personalized risk assessment and tailored screening, as well as increased awareness of women and health professionals about health risks in young adults.

Cohort profile: population-based cohorts of patients with colorectal cancer and of their relatives in Geneva, Switzerland.

PURPOSE: Colorectal cancer (CRC) is the third leading cause of cancer death worldwide. Variability between patients in prognosis and treatment response is partially explained by traditional clinicopathological factors. We established a large population-based cohort of patients with CRC and their first-degree and second-degree relatives registered in the Canton of Geneva, to evaluate the role of family history and tumour biomarkers on patient outcomes. PARTICIPANTS: The cohort includes all patients with CRC diagnosed between 1985 and 2013. Detailed information on patient and tumour characteristics, treatment and outcomes were extracted from the Geneva Cancer Registry database, completed by medical records review and linkage with administrative and oncogenetics databases. Next-generation tissue microarrays were constructed from tissue samples of the primary tumour. A prospective follow-up of the cohort is realised annually to collect data on outcomes. First-degree and second-degree relatives of patients are identified through linkage with the Cantonal Population Office database and information about cancer among relatives is retrieved from the Geneva Cancer Registry database. The cohort of relatives is updated annually. FINDINGS TO DATE: The cohort includes 5499 patients (4244 patients with colon cancer and 1255 patients with rectal cancer). The great majority of patients were diagnosed because of occurrence of symptoms and almost half of the cases were diagnosed with an advanced disease. At the end of 2019, 337 local recurrences, 1143 distant recurrences and 4035 deaths were reported. At the same date, the cohort of first-degree relatives included 344 fathers, 538 mothers, 3485 children and 375 siblings. Among them, we identified 28 fathers, 31 mothers, 18 siblings and 53 children who had a diagnosis of CRC. FUTURE PLANS: The cohort will be used for long-term studies of CRC epidemiology with focus on clinicopathological factors and molecular markers. These data will be correlated with the most up-to-date follow-up data.

Occupational Factors and Socioeconomic Differences in Breast Cancer Risk and Stage at Diagnosis in Swiss Working Women.

Socioeconomic differences in breast cancer (BC) incidence are driven by differences in lifestyle, healthcare use and occupational exposure. Women of high socioeconomic status (SES) have a higher risk of BC, which is diagnosed at an earlier stage, than in low SES women. As the respective effects of occupation and SES remain unclear, we examined the relationships between occupation-related variables and BC incidence and stage when considering SES. Female residents of western Switzerland aged 18−65 years in the 1990 or 2000 census, with known occupation, were linked with records of five cancer registries to identify all primary invasive BC diagnosed between 1990 and 2014 in this region. Standardized incidence ratios (SIRs) were computed by occupation using general female population incidence rates, with correction for multiple comparisons. Associations between occupation factors and BC incidence and stage at diagnosis were analysed by negative binomial and multinomial logistic regression models, respectively. The cohort included 381,873 women-years and 8818 malignant BC, with a mean follow-up of 14.7 years. Compared with reference, three occupational groups predominantly associated with a high socioprofessional status had SIRs > 1: legal professionals (SIR = 1.68, 95%CI: 1.27−2.23), social science workers (SIR = 1.29; 95%CI: 1.12−1.49) and some office workers (SIR = 1.14; 95%CI: 1.09−1.20). Conversely, building caretakers and cleaners had a reduced incidence of BC (SIR = 0.69, 95%CI: 0.59−0.81). Gradients in BC risk with skill and socioprofessional levels persisted when accounting for SES. A higher incidence was generally associated with a higher probability of an early-stage BC. Occupation and SES may both contribute to differences in risk and stage at diagnosis of BC.

Research on occupational diseases in the absence of occupational data: a mixed-method study among cancer registries of Western Switzerland.

The contribution of occupation-related diseases to the global burden of disease is greatly underestimated, mainly due to a shortage of occupational exposure data. This problem is particularly salient in Switzerland, where no estimates of occupation-related disease burden exist, even for the well-recognised occupational cancers, such as malignant pleural mesothelioma and lung cancer. To overcome this situation, we launched a research project "Examining Cancers and Labour Indicators to assess the Burden" (ExCaLIBur). Within this project, we aimed to assess the need for and quality (i.e., completeness, accuracy and precision) of occupation registration in all cancer registries of Western Switzerland. We also aimed to find a relevant and feasible strategy to collect this information in the future. We applied a mixed research method. We observed that, independently of the level of precision (5-3-2-1-digit aggregation level), the accuracy was lesser in the registries that were able to actively search and verify occupational information. Overall, the distinction of occupations based on the 3-digit code presents an acceptable compromise in terms of precision. Having such occupations registered in all, or most, Swiss cancer registries routinely would obviously be valuable for epidemiological surveillance of occupational cancers in Switzerland. However, it seems less obvious how these data could fulfill the research objectives, since a better precision than 3-digit occupational coding is challenging to achieve. Currently, the collection of occupational data by the Swiss cancer registries remains feasible in the frame of specific research projects on occupational cancers. However, available data sources, as well as lack of financial and human resources, will continue to affect quality of the collected occupation data. Therefore, the usage of the standardised questionnaire retracing the individual occupational history to enable further assessment of individual exposure to potential occupational hazards is recommended. However, this approach will disable the Swiss registries to insuring their epidemiological surveillance mission with respect to occupational cancers in Switzerland, for which national statistics remain limited.

NAFLD and MAFLD as emerging causes of HCC: A populational study.

BACKGROUND & AIMS: There are conflicting data regarding the epidemiology of hepatocellular carcinoma (HCC) arising in the context of non-alcoholic and metabolic-associated fatty liver disease (NAFLD and MAFLD). We aimed to examine the changing contribution of NAFLD and MAFLD, stratified by sex, in a well-defined geographical area and highly characterised HCC population between 1990 and 2014. METHODS: We identified all patients with HCC resident in the canton of Geneva, Switzerland, diagnosed between 1990 and 2014 from the prospective Geneva Cancer Registry and assessed aetiology-specific age-standardised incidence. NAFLD-HCC was diagnosed when other causes of liver disease were excluded in cases with type 2 diabetes, metabolic syndrome, or obesity. Criteria for MAFLD included one or more of the following criteria: overweight/obesity, presence of type 2 diabetes mellitus, or evidence of metabolic dysregulation. RESULTS: A total of 76/920 (8.3%) of patients were diagnosed with NAFLD-HCC in the canton of Geneva between 1990 and 2014. Between the time periods 1990-1994 and 2010-2014, there was a significant increase in HCC incidence in women (standardised incidence ratio [SIR] 1.83, 95% CI 1.08-3.13, p = 0.026) but not in men (SIR 1.10, 95% CI 0.85-1.43, p = 0.468). In the same timeframe, the proportion of NAFLD-HCC increased more in women (0-29%, p = 0.037) than in men (2-12%, p = 0.010) while the proportion of MAFLD increased from 21% to 68% in both sexes and from 7% to 67% in women (p <0.001). From 2000-2004 to 2010-2014, the SIR of NAFLD-HCC increased to 1.92 (95% CI 0.77-5.08) for men and 12.7 (95% CI 1.63-545) in women, whereas it decreased or remained stable for other major aetiologies of HCC. CONCLUSIONS: In a populational cohort spanning 25 years, the burden of NAFLD and MAFLD associated HCCs increased significantly, driving an increase in HCC incidence, particularly in women. LAY SUMMARY: Hepatocellular carcinoma (HCC) is the most common type of liver cancer, increasingly arising in patients with liver disease caused by metabolic syndrome, termed non-alcoholic fatty liver disease (NAFLD) or metabolic-associated fatty liver disease (MAFLD). We assessed all patients with HCC between 1990 and 2014 in the canton of Geneva (western Switzerland) and found an increase in all HCC cases in this timeframe, particularly in women. In addition, we found that HCC caused by NAFLD or MAFLD significantly increased over the years, particularly in women, possibly driving the increase in overall HCC cases.

Concordance of tumour characteristics and survival clustering among pairs of first-degree relatives with breast cancer.

BACKGROUND: Family history is a known risk factor for breast cancer, but its prognostic value and the prognostic value of tumour characteristics in relation to family history has not been clearly established. In addition, studies of intra-familial tumour characteristics and prognosis in population-based settings are very rare. Two previous studies have suggested that breast cancer prognosis clusters within families. However, both studies lack information on HER2 expression status, which is a strong prognostic factor and could contribute to the observed results. METHODS: We conducted a population-based study on 145 mother-daughter and sister-sister affected pairs using data extracted from the Geneva Cancer Registry. Histopathological characteristics were determined in archived tumour blocks by immunochemistry techniques. Breast cancer survival among family members was studied according to patient and tumour characteristics. RESULTS: No significant intra-familial agreement of pathological characteristic features was observed. We found that relatives of breast cancer patients experienced a much higher risk of breast cancer death compared to the general population. However, we did not find significant concordance in good and poor breast cancer-specific survival between pairs. The small number of family pairs and deaths from breast cancer may partly explain our results. CONCLUSIONS: Large-scale studies with accurate data on strong prognosticators are still needed to confirm the possibility of familial inheritance of breast cancer prognosis.  .

Using ecological socioeconomic position (SEP) measures to deal with sample bias introduced by incomplete individual-level measures: inequalities in breast cancer stage at diagnosis as an example.

BACKGROUND: When studying the influence of socioeconomic position (SEP) on health from data where individual-level SEP measures may be missing, ecological measures of SEP may prove helpful. In this paper, we illustrate the best use of ecological-level measures of SEP to deal with incomplete individual level data. To do this we have taken the example of a study examining the relationship between SEP and breast cancer (BC) stage at diagnosis. METHODS: Using population based-registry data, all women over 18 years newly diagnosed with a primary BC in 2007 were included. We compared the association between advanced stage at diagnosis and individual SEP containing missing data with an ecological level SEP measure without missing data. We used three modelling strategies, 1/ based on patients with complete data for individual-SEP (n = 1218), or 2/ on all patients (n = 1644) using an ecological-level SEP as proxy for individual SEP and 3/ individual-SEP after imputation of missing data using an ecological-level SEP. RESULTS: The results obtained from these models demonstrate that selection bias was introduced in the sample where only patients with complete individual SEP were included. This bias is redressed by using ecological-level SEP to impute missing data for individual SEP on all patients. Such a strategy helps to avoid an ecological bias due to the use of aggregated data to infer to individual level. CONCLUSION: When individual data are incomplete, we demonstrate the usefulness of an ecological index to assess and redress potential selection bias by using it to impute missing individual SEP.

Are disparities of waiting times for breast cancer care related to socio-economic factors? A regional population-based study (France).

The increasing number of breast cancer cases may induce longer waiting times (WT), which can be a source of anxiety for patients and may play a role in survival. The aim of this study was to examine the factors, in particular socio-economic factors, related to treatment delays. Using French Cancer Registry databases and self-administered questionnaires, we included 1,152 women with invasive non-metastatic breast cancer diagnosed in 2007. Poisson regression analysis was used to identify WTs' influencing factors. For 973 women who had a malignant tissue sampling, the median of overall WT between the first imaging procedure and the first treatment was 44 days (9 days for pathological diagnostic WT and 31 days for treatment WT). The medical factors mostly explained inequalities in WTs. Socio-economic and behavioral factors had a limited impact on WTs except for social support which appeared to be a key point. Better identifying the factors associated with increase in WTs will make it possible to develop further interventional or prospective studies to confirm their causal role in delay and at last reduce disparities in breast cancer management.

Health-related quality of life is a prognostic factor for survival in older patients after colorectal cancer diagnosis: A population-based study.

BACKGROUND: Studies carried out in the context of clinical trials have shown a relationship between survival and health-related quality of life in colorectal cancer patients. AIMS: We assessed the prognostic value of health-related quality of life at diagnosis and of its longitudinal evolution on survival in older colorectal cancer patients. METHODS: All patients aged ≥65 years, diagnosed with new colorectal cancer between 2003 and 2005 and registered in the Digestive Cancer Registry of Burgundy were eligible. Patients were asked to complete the European Organisation for Research and Treatment of Cancer Quality of Life Questionnaire Core 30 at inclusion, three, six and twelve months after. Multivariate regression models were used to evaluate the prognostic value of health-related quality of life scores at diagnosis and their deterioration on relative survival. RESULTS: In multivariate analysis, a role functioning dimension lower than median was predictive of lower survival (hazard ratio=3.1, p=0.015). After three and six months of follow-up, patients with greater appetite loss were more likely to die, with hazard ratios of 4.7 (p=0.013) and 3.7 (p=0.002), respectively. CONCLUSIONS: Health-related quality of life assessments at diagnosis are independently associated with older colorectal cancer patients' survival. Its preservation should be a major management goal for older cancer patients.

Kaposi's sarcoma: a population-based cancer registry descriptive study of 57 consecutive cases diagnosed between 1977 and 2009.

BACKGROUND: There are few epidemiological data available on rare skin cancer, including Kaposi's sarcoma (KS), which is a multifocal illness affecting the skin, mucosa, and viscera. Four different types of KS have been described: classic, AIDS-associated, iatrogenic, and African. The purpose of this study was to describe the epidemiology and evolution of the different types of KS in the Doubs region of France. PATIENTS AND METHODS: A retrospective population-based study was conducted, including 57 patients with KS from the Doubs region between 1977 and 2009. These patients were identified by the tumor registry of the Doubs region. RESULTS: A larger proportion of AIDS-associated KS (61%) was observed compared to classic KS (30%) and iatrogenic KS (9%). No cases of African KS were observed. Most new cases were observed between 1987 and 1996 due to the AIDS explosion. The patients were predominantly male, with a male/female ratio at 10 : 4 (34 for AIDS-associated KS). The mean age of patients was 50.2 (higher in classic and iatrogenic KS and lower in AIDS-associated KS). Mucosal and visceral forms were more common in AIDS-associated KS, explaining the higher rate of mortality due to KS (21%). CONCLUSION: KS, which used to be a rare illness, as it existed only in the classic form, has become more common since the appearance of HIV and, to a lesser extent, of immunosuppressive treatment.

[Evaluation of medical information systems as a mean of identification of incident cases of colorectal cancer]. / Evaluation du PMSI comme moyen d'identification des cas incidents de cancer colorectal.

BACKGROUND: Cancer registries are a reliable source of data to estimate national cancer incidence rates, but they are not always available in all regions. This study assessed the value of medical information systems (PMSI) to identify incident cases of colorectal cancer METHODS: Two algorithms were elaborated to identify these incident cases in the PMSI database. The first algorithm was based on diagnosis and medical procedure codes and the second algorithm was based exclusively on diagnoses and the absence of diagnoses over the last five years. The results obtained for two departments were compared with those derived from two cancer registries, constituting the reference data. We then elaborated two multivariate logistic regression models to correct the national number of incident cases estimated by the algorithm adopted after evaluation of the results. RESULTS: The first algorithm provided results that were very close to those derived from the regional registries (646 vs 645 cases) with a good sensitivity and positive predictive value of 75%. The second algorithm overestimated the incidence by about 50% with a positive predictive value of 60% and was therefore not adopted for the national estimation. By applying the first algorithm to the national PMSI MCO database (medicine, surgery, obstetrics), and after modelling, the estimated incidence differed by only 2.34% compared to that observed by all 14 registries. The national estimation of cancer incidence was 39,122 [37,020; 41,224] cases for 2005, which is consistent with the figure published by the Francim national registry network (37,413). CONCLUSION: This study demonstrates the value of PMSI data for estimation of national incidence rates for certain cancers in the absence of cancer registries. However, raw data must be corrected and can be achieved by the method proposed here.

Trends in invasive breast cancer incidence among French women not exposed to organized mammography screening: an age-period-cohort analysis.

BACKGROUND: The long tenure of the Doubs cancer registry (France) and the late implementation of a mass screening program provide a unique opportunity to assess the relative contributions of age, period and cohort effects to the increase in female invasive breast cancer incidence, while avoiding the influence of an organized screening program. METHODS: Population and incidence data were provided for the Doubs region during the 1978-2003 period. Breast cancer counts and person-years were tabulated into 1-year classes by age and time period. Age-period-cohort models with parametric smooth functions were fitted to the data, assuming a Poisson distribution for the number of observed cases. RESULTS: A total of 5688 incident cases of invasive breast cancer in women were diagnosed in women aged 30-84 years in the Doubs region between 1978 and 2003. The annual percentage increase in incidence is 2.09%. Age effects rise dramatically until age 50, and at a slower pace afterwards. Large cohort curvature effects (p<10(-6)), show departure from linear trends, with a significant peak for women born around 1940. Period curvature effects are lower in magnitude (p=0.01). CONCLUSION: Both cohort and period effects are involved in the marked increase in breast cancer incidence over a 25-year period in the Doubs region. Although the future trend for breast cancer incidence is difficult to predict, the introduction of an organized screening program, and the sharp decline in hormone replacement therapy use will likely contribute to period effects in future analyses.

Age-period-cohort modelling of non-Hodgkin's lymphoma incidence in a French region: a period effect compatible with an environmental exposure.

BACKGROUND: The incidence of non-Hodgkin's lymphoma (NHL) has risen steadily during the last few decades in all geographic regions covered by cancer registration for reasons that remain unknown. The aims of this study were to assess the relative contributions of age, period and cohort effects to NHL incidence patterns and therefore to provide clues to explain the increasing incidence. METHODS: Population and NHL incidence data were provided for the Doubs region (France) during the 1980-2005 period. NHL counts and person-years were tabulated into one-year classes by age (from 20 to 89) and calendar time period. Age-period-cohort models with parametric smooth functions (natural splines) were fitted to the data by assuming a Poisson distribution for the observed number of NHL cases. RESULTS: The age-standardised incidence rate increased from 4.7 in 1980 to 11.9 per 100,000 person-years at risk in 1992 (corresponding to a 2.5-fold increase) and stabilised afterwards (11.1 per 100,000 in 2005). Age effects showed a steadily increasing slope up to the age of 80 and levelled off for older ages. Large period curvature effects, both adjusted for cohort effects and non-adjusted (p < 10-4 and p < 10-5, respectively), showed departure from linear periodic trends; period effects jumped markedly in 1983 and stabilised in 1992 after a 2.4-fold increase (compared to the 1980 period). In both the age-period-cohort model and the age-cohort model, cohort curvature effects were not statistically significant (p = 0.46 and p = 0.08, respectively). CONCLUSIONS: The increased NHL incidence in the Doubs region is mostly dependent on factors associated with age and calendar periods instead of cohorts. We found evidence for a levelling off in both incidence rates and period effects beginning in 1992. It is unlikely that the changes in classification (which occurred after 1995) and the improvements of diagnostic accuracy could largely account for the 1983-1992 period-effect increase, giving way to an increased exposure to widely distributed risk factors including persistent organic pollutants and pesticides. Continued NHL incidence and careful analysis of period effects are of utmost importance to elucidate the enigmatic epidemiology of NHL.

Rare skin cancer: a population-based cancer registry descriptive study of 151 consecutive cases diagnosed between 1980 and 2004.

BACKGROUND: There are few epidemiological data available on rare skin cancer, including Merkel cell carcinoma, Paget's disease, adnexal carcinoma, and sarcoma. We conducted this study to investigate the epidemiology of rare skin cancer diagnosed in the department of Doubs from 1980 to 2004. METHODS: Data were collected from a population-based cancer registry from 1980 to 2004. Diagnosis was based on the 3(rd) edition of the International Classification of Diseases for Oncology. The incidence rates were standardized on world population. RESULTS: One hundred and fifty one patients were investigated (88 women and 63 men). Median age for the diagnosed disease was 63 years. The standardized incidence rate was 0.82/100 000 person-year (95% CI = 0.68-0.96) and increased from 0.25 in 1980-1984 to 1.50 in 2000-2004. Fifty nine cases (39%) were sarcomas, 35 (23%) adnexal carcinomas, 27 (18%) Merkel cell carcinoma and 27 (18%) Paget's disease. The standardized incidence rates were 0.37/100 000 (0.27-0.47) for sarcomas, 0.16 (0.10-0.22) for adnexal tumors, 0.13 (0.08-0.18) for Merkel cell carcinoma, and 0.15 (0.09-0.21) for Paget's disease. CONCLUSIONS: Our results based on a population-based cancer registry showed an increase of the standardized incidence rate for all types of rare skin tumors. These results may be useful when considering the growing interest in rare diseases in identifying risk factors and planning scientific research programmes.

Primary cutaneous lymphomas: a population-based descriptive study of 71 consecutive cases diagnosed between 1980 and 2003.

Although primary cutaneous lymphomas (PCL) are the second most common group of extra-nodal non-Hodgkin lymphomas, few epidemiological data are available in the literature, and most of them are provided by large databases from population-based cancer registries in the US or patients attending a single institution. We conducted this study to investigate the epidemiological and clinical features of PCL diagnosed in the department of Doubs from 1980 to 2003. Data were collected from the Doubs cancer registry from 1980 to 2003. Seventy-one patients with PCL were investigated. 82% were cutaneous T-cell lymphoma (CTCL) and 18% were cutaneous B-cell lymphoma (CBCL). Among CTCL, mycosis fungoides (MF) represented 58% and Sezary syndrome 10%. The standardised incidence rate of PCL was 0.42 for 100 000 person-years and significantly increased from 0.21 in 1980-1984 to 0.70 in 2000-2003 (p <0.05). The incidence rate of CTCL was 0.34 for 100 000 person-year and significantly increased from 0.2 to 0.57 (p <0.05). For MF and CBCL, the incidence rates were 0.20 and 0.08, respectively and did not vary significantly from 1980-1984 to 2000-2003. Five-year survival was 64.5% for PCL patients similar to MF patients. Our results provide updated data on the incidence of PCL in France.