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Background: The reported incidence rates of vestibular schwannomas (VS) vary substantially, but it is unclear as to what extent the variation reflects differences in risk or recording practices. Our aim was to describe the incidence rates of VS in Denmark, Finland, Norway and Sweden between 1987 and 2007.Methods: Comprehensive data were available from all registries only for the period from 1987 to 2007. An analysis of a longer time period (1965-2007) was conducted with the Norwegian and Swedish data.Results: The average age-standardised incidence rates during 1987-2007 varied from 6.1 per 1,000,000 person-years (95% confidence interval (CI), 5.4-6.7) among Finnish men to 11.6 (95% CI, 10.4-12.7) in Danish men, and from 6.4 per 1,000,000 person-years (95% CI, 5.7-7.0) among Swedish women to 11.6 (95% CI, 10.5-12.8) among Danish women. An overall annual increase of 3.0% (95% CI 2.1-3.9) was observed when all countries and both sexes were combined, with considerable differences between countries. However, the practices of both reporting and coding VS cases varied markedly between countries and over time, which poses a challenge for interpretation of the results.Conclusion: The overall incidence of VS increased in all the four Nordic countries combined between 1987 and 2007, with marked differences between countries. However, the incidence rates more or less stabilised in the late 1990s, showing relatively constant incidence rates and even some decline after 2000. [ABSTRACT FROM AUTHOR]

Background: Immunosuppression and Merkel-cell polyomavirus (MCPyV) infection may have a role in the pathogenesis of Merkel-cell carcinoma (MCC), a rare neuroendocrine carcinoma of the skin.Methods: We studied incidence of chronic lymphocytic leukaemia (CLL) and MCC from the files of the Finnish Cancer Registry and the largest hospital of Finland, Helsinki University Central Hospital, from 1979 to 2006. Presence of MCPyV DNA in MCCs was investigated by quantitative PCR.Results: We identified 4164 patients diagnosed with CLL and 172 diagnosed with MCC. Six patients diagnosed with both diseases were found; CLL was the first diagnosis in four cases and MCC in two. The standardised incidence ratio (SIR) for CLL after the diagnosis of MCC was highly elevated, 17.9 (95% confidence interval (CI), 2.2-64.6; P<0.001), and the SIR for MCC after the diagnosis of CLL was also elevated, 15.7 (3.2-46.0, P<0.01). Merkel-cell polyomavirus DNA was present in all five MCCs with tumour tissue available for analysis.Conclusions: We conclude that patients diagnosed with CLL have a substantially increased risk for MCC, and vice versa. Merkel-cell polyomavirus DNA is frequently present in MCCs that occur in CLL patients. Immunosuppression related with CLL and viral infection might explain the association between CLL and MCC. [ABSTRACT FROM AUTHOR]

Background and purpose There have only been a few nationwide studies on the epidemiology and outcome of osteosarcoma. We report the clinical features, treatment, and prognosis of osteosarcoma in Finland for the period 1971-1990. Methods The study material was derived from popu-lation-based data from the national Finnish Cancer Registry. 302 osteosarcomas were reported during the study period. Histological slides could be retrieved for 199 cases and from histological re-examination, 139 (83%) of these cases were confirmed as osteosarcoma and were included in the analysis. The mean length of follow-up was 8 (0.1-28) years. Results The overall 5-year survival for the whole study population was 58%, with an improvement in survival during 1981-1990 (65%) compared to the period 1971-1980 (47%) (p=0.01). More chemotherapy was administered in the later time period. For metastasis-free survival, diagnosis in the 1970s as opposed to the 1980s (p=0.01) and large tumor size worsened outcome in univariate analysis. Patients who developed metastatic relapse within 10 months of the diagnosis had worse sarcoma-specific survival than those who developed metastases later. Limb-salvage surgery increased from 12% to 23% for patients with a peripheral tumor, with no increase in local relapses. Interpretation We recommend aggressive approach to treat recurrent disease, with a view to further improving survival. In a small country such as Finland it is necessary to concentrate treatment to only a few centers, to ensure a high quality of treatment. [ABSTRACT FROM AUTHOR]

Epidemiological studies have consistently shown elevated rates of breast cancer among female blood relatives of patients with ataxia telangiectasia (AT), a rare autosomal recessive disease. A large proportion of the members of AT families are carriers of AT-causing gene mutations in ATM (Ataxia Telangiectasia Mutated), and it has been hypothesised that these otherwise healthy carriers are predisposed to breast cancer. This is an extended and enlarged follow-up study of cancer incidence in blood relatives of 75 patients with verified AT in 66 Nordic families. Blood relatives were identified through population registry linkages, and the occurrence of cancer was determined from cancer registry files in each country and compared with national incidence rates. The ATM mutation carrier probabilities of relatives were assigned from the combined information on location in family, consanguinity, if any, and supplementary carrier screening in some families. Among the 1445 blood relatives of AT patients, 225 cancers were observed, with 170.4 expected, yielding a standardised incidence ratio (SIR) of 1.3 (95% confidence interval (CI), 1.1–1.4). Invasive breast cancer occurred in 34 female relatives (SIR, 1.7; 95% CI, 1.2–2.4) and was diagnosed in 21 women before the age of 55 years (SIR, 2.9; 95% CI, 1.8–4.5), including seven mothers of probands (SIR, 8.1; 95% CI, 3.3–17). When the group of mothers was excluded, no clear relationship was observed between the allocated mutation carrier probability of each family member and the extent of breast cancer risk. We concluded that the increased risk for female breast cancer seen in 66 Nordic AT families appeared to be restricted to women under the age of 55 years and was due mainly to a very high risk in the group of mothers. The findings of breast cancer risk in mothers, but not other likely mutation carriers, in this and other studies raises questions about the hypothesis of a simple causal relationship with ATM heterozygosity.British Journal of Cancer (2005) 93, 260–265. doi:10.1038/sj.bjc.6602658 www.bjcancer.com Published online 7 June 2005 [ABSTRACT FROM AUTHOR]

The present study was aimed at assessing differences between the Nordic countries, if any, in late mortality among five-year survivors of childhood cancer. All cases diagnosed before the age of 20 years, between 1960 and 1989, were collected from all Nordic cancer registries. In total, 13,689 patients were identified as five-year survivors and during the extended follow-up 12.3% of them died. Mortality was analysed by decade of diagnosis, for all sites, and for leukaemia, Hodgkin's lymphoma, and central nervous system tumours separately. Analyses were done within a Cox proportional hazards regression framework with adjustments made for gender and age at diagnosis. Hazard ratios were calculated in relation to a weighted Nordic mean based on the proportion of five-year survivors in each country. Overall late mortality was significantly higher in Denmark and Finland than in Norway and Sweden. This could not be explained by inverse differences in five-year survival. The differences diminished over time and had disappeared in the last period. The pattern was similar for both genders. The disappearance of the differences was most probably the effect of a closer collaboration between Nordic paediatric oncologists with development and implementation of common protocols for treatment of childhood cancers in all countries. [ABSTRACT FROM AUTHOR]

Objectives: To compare the risk of cancer between BRCA1 or BRCA2 mutation-positive and -negative families.Methods: We assessed standardized incidence ratios (SIR) in 107 Finnish breast cancer families (12 BRCA1, 11 BRCA2, 84 non-BRCA1/2) with confirmed genealogy. The observed numbers of cancer cases were compared to the expected ones; both numbers were based on the population-based Finnish Cancer Registry.Results: Risk of ovarian cancer for first-degree relatives was high in BRCA1 (SIR 29, 95% confidence interval 9.4-68) and in BRCA2 families (SIR 18. 8.3-35), but not increased for non-BRCA1/2 families (SIR 1.0, 0.2-2.9). The SIR for subsequent ovarian cancer among breast cancer patients was 61 (20-142), 38 (11-98), and 0 (0-4.2), respectively. The risk of subsequent new breast cancer among breast cancer patients was equally high in BRCA1 families (SIR 11, 3.6-26) and in BRCA2 families (SIR 10, 3.3-24) and somewhat lower in mutation-negative families (SIR 3.7, 2.1-6.1). The risk of breast cancer among relatives was markedly increased in all three groups. The only elevated SIR, besides breast and ovarian, was that for prostate cancer in BRCA2 families (SIR 4.9, 1.8-11).Conclusions: The excess risk of breast cancer in non-BRCA1/2 families suggests the existence of another predisposition gene which seems not to be linked with increased risk of ovarian cancer. [ABSTRACT FROM AUTHOR]

Objective: Five to ten percent of prostate cancers may be caused by inherited genetic defects. In order to explore the nature of inherited cancer risks in the genetically homogeneous Finnish population, we investigated the incidence of prostate cancer and other cancers in first-degree relatives of prostate cancer patients by linking the population-based parish records on relatives with the Finnish Cancer Registry (FCR) data.Methods: The study population was composed of first-degree relatives of two groups of prostate cancer patients diagnosed in Finland during 1988-1993: (1) all early-onset (<60 years) patients (n = 557) from the entire country, (2) a sample (n = 989) of prostate cancer patients diagnosed at an age of > 60 years. A total of 11,427 first-degree relatives were identified through parish records, and their cancer incidence was determined based on a total of 299,970 person-years. Standardized incidence ratios (SIR) were calculated based on expected cancer rates in the general population.Results: The SIR of prostate cancer was increased in both Cohort 1 (2.5, 95% CI 1.9-3.2) and Cohort 2 (1.7, 95% CI 1.4 2.1). The risk of prostate cancer was high for relatives of patients diagnosed at an early age, and then leveled off for patients in the median age of prostate cancer diagnosis (70-79 years). However, the prostate cancer risk for relatives of patients diagnosed > or = 80 years was again statistically significantly elevated (SIR 1.8, 95% CI 1.3-2.6), suggesting a contribution of genetic factors to prostate cancer also at a late age of onset. Gastric cancer was the only other cancer type with a significantly elevated risk among the relatives. Increased risk of gastric cancer was seen only in male relatives of prostate cancer patients diagnosed at an early age, with the highest risk detected for the male relatives of prostate cancer patients diagnosed at an age of 55 years or less (SIR 5.0, 95% CI 2.8-8.2).Conclusions: Our population-based study indicates that hereditary factors may play an important role in the development of prostate cancer among the relatives of men diagnosed both at younger and older ages. This finding is relevant in the context of our observations that HPCX (hereditary prostate cancer susceptibility locus on Xq27-28) linkage in Finland is found exclusively among families with late age of onset. The association of gastric cancer with prostate cancer has not been reported previously, and may reflect the effects of a novel predisposition locus, which increases the risk to both of these common tumor types. [ABSTRACT FROM AUTHOR]

Background: Epidemiologic studies of the families of patients with ataxia-telangiectasia (A-T), a recessive genetic neurologic disorder caused by mutation of the ATM gene, suggest that heterozygous carriers of an ATM mutation are at increased risk of cancer. A population-based study of cancer incidence in A-T families with unbiased selection and tracing of relatives would confirm this hypothesis.Methods: We conducted a study in the Nordic countries of 1218 blood relatives of 56 A-T patients from 50 families. The relatives were identified from population registries, and the occurrence of cancer was determined from cancer registry files in each country and compared with national incidence rates. All statistical tests were two-sided.Results: Among the 56 patients with A-T, we observed six cases of cancer (four leukemias and two non-Hodgkin's lymphomas) compared with 0.16 expected, yielding a standardized incidence ratio (SIR) of 37 (95% confidence interval [CI] = 13 to 80). Among the 1218 relatives, 150 cancers were recorded, with 126 expected (SIR = 1.19; 95% CI = 1.01 to 1.40). Invasive breast cancer occurred in 21 female relatives of A-T patients (SIR = 1.54; 95% CI = 0.95 to 2.36), including five of the 50 mothers (all of whom are obligate ATM mutation carriers) (SIR = 7.1; 95% CI = 2.3 to 17). Relatives who were less likely to be carriers of a mutant ATM allele had no increase or only a modest, statistically nonsignificant increase in the risk of breast cancer. There was no evidence of increased risk for cancer at any other site.Conclusions: We confirmed the previously recognized high risk of lymphoma and leukemia in A-T patients. Our data are also consistent with an increased risk of breast cancer among blood relatives of A-T patients. The epidemiologic findings suggest, however, that, even if ATM mutations are responsible for some breast cancer cases, ATM is a relatively weak genetic risk factor for the disease. [ABSTRACT FROM AUTHOR]

A total of 1,197 borderline ovarian tumors were reported to the Finnish Cancer Registry in 1973-1992. The mean age of the patients was 52 years, while the mean age of the 7,060 patients with an invasive epithelial ovarian carcinoma reported in the same time period was 62 years. The incidence of borderline ovarian tumors did not increase with age after patients became 35 years old and older. The overall age-adjusted incidence of borderline ovarian tumors was 1.8 per 100,000 women-years. Familial cancer occurrence during 1967-1992 was studied among relatives of 144 index patients diagnosed in 1980-1982. No borderline ovarian tumors were detected in the relatives, and only one of the 446 female first-degree relatives had an epithelial ovarian cancer. The expected number (borderline and invasive combined) was 1.9. The mothers of the index patients had an increased risk for pancreatic cancer (standardized incidence ratio 4.9, 95% confidence interval 1.0-14.3) and for cancer of the uterine cervix (standardized incidence ratio 7.8, 95% confidence interval 1.6-22.8). No significant increase in cancer risk was observed for fathers, brothers, or sisters of the patients with borderline ovarian tumors. Am J Epidemiol 1996; 144:548-53. [ABSTRACT FROM AUTHOR]

We assessed the undercount of meningiomas in a population-based cancer registry. A comprehensive material was formed by compiling hospital sources with the Finnish Cancer Registry database. The completeness of each source ranged 62-69%. The corrected age-standardised meningioma incidence was 2.9/100,000 for men and 13.0/100,000 for women, a third higher than the cancer registry figures. [ABSTRACT FROM AUTHOR]

Assesses the combined effect of breast cancer as the first primary tumor and colorectal cancer as the second primary tumor on patient survival in an unbiased way. Model-based method to control for the excess mortality related to the first cancer; Cohorts from the files of the Finnish Cancer Registry; Ratios of relative risks of excess death associated with colorectal carcinoma.

Presents a response to a letter to the editor about the increase in breast cancer risk among the female relatives of ataxia-telangiectasia patients, published in the September 2005 issue of the "British Journal of Cancer."

The main objective of this study is to evaluate whether serum enterolactone is associated with the breast cancer risk in a nested case-control study. Enterolactone concentrations were measured by time resolved fluoroimmunoassay in serum collected at four independent cross sectional population surveys from 206 women with breast cancer diagnosed during follow up and from 215 controls frequency matched to cases by study cohort, 5 year age group and study area. Findings of this study do not support the hypothesis that high serum enterolactone concentration is associated with reduced risk of breast cancer.

The Automated Childhood Cancer Information System (ACCIS) collects and presents data on childhood cancer in Europe. This report describes trends (1978-1997) and geographical differences (1988-1997) in incidence and survival for 6202 children with neuroblastoma from 59 registries in 19 countries, grouped into five regions (British Isles, West, East, North, and South). The age-standardised incidence rate (ASR) of neuroblastoma in Europe in 1988-1997 was 10.9 cases per million children, being highest in infants (52.6). The ASR of neuroblastoma increased in Europe from 8.4 in 1978-1982 to 11.6 in 1993-1997, mostly due to an increase in infants (from 35.4 to 57.8). Overall 5-year survival was 59%, ranging from 47% (East) to 67% (West). It improved markedly from 37% in 1978-1982 to 66% in 1993-1997, especially in infants. A certain amount of overdiagnosis in children under 2 years of age may explain the increased incidence rates and partially the increase in survival. Survival of older children (aged 2-14 years), which is likely to be largely affected by therapy, has also improved from 21% to 45%. [ABSTRACT FROM AUTHOR]

This prospective nation-wide study was performed to evaluate the effect of hospital category and subspeciality training on surgical treatment of ovarian cancer. Data were obtained from a questionnaire filled in by the operating unit, and from the surgical and histopathology reports. The survey included 307 patients. Half of them were operated in the university hospitals where gynaecologic oncologists performed 72% of the operations. This was the case in only 4% and 19% in the central and district hospitals, respectively. In university hospitals, pelvic lymphadenectomy was performed in 88%, and para-aortic lymphadenectomy in 73%, of the patients with stage I disease. The corresponding figures ranged from 11% to 21% in central and district hospitals. For stage III patients operated by gynaecologic oncologists, the estimated odds ratio for no macroscopic tumour was 3.0 times higher (95% CI 1.2-7.5) than for those operated by general gynaecologists. These results favour centralisation of surgical treatment of ovarian cancer. [ABSTRACT FROM AUTHOR]

Purpose: The population-based incidence of severe osteonecrosis (ON) necessitating total joint arthroplasty (TJA) in patients with hematological cancer is unknown. This study assessed the incidence of ON requiring primary TJA in children and young adults treated for leukemia or lymphoma. Methods: Patients diagnosed with leukemia or lymphoma before 31 years of age were identified from the Finnish and Danish Cancer Registries. These data were combined with those from the National Hospital Discharge and the Finnish Arthroplasty Registers. Data on the orthopedic procedures performed and the appropriate diagnosis codes given before the age of 40 were also retrieved. Results: The estimated cumulative incidence of TJA was 4.5% at 20 years for patients treated for chronic myeloid leukemia, followed by 2.1% for patients treated for acute myeloid leukemia. It was considerably lower in patients with acute lymphoblastic leukemia (ALL; 0.4%). Multivariate analysis revealed that allogeneic stem cell transplantation (allo-SCT) increased the risk of TJA (hazard ratio [HR]=9.4; 95% CI: 5.3-16.9). The risk of TJA was higher in patients diagnosed with cancer at 10-19 and 20-30 years of age than in those diagnosed before the age of 10 (HR=24; 95% CI: 3.1-176 and HR=26; 95% CI: 3.6-192 respectively). Conclusion: The incidence of ON requiring TJA was highest among patients with myeloid leukemias and lowest in patients treated for ALL. Allo-SCT and age ≥10 years at diagnosis were the most important risk factors for ON requiring TJA in hematological malignancies.

Population-based information on the survival of patients with myeloid malignancies is rare mainly because some entities were not recognized as malignant until the publication of the third revision of the International Classification of Diseases for Oncology and World Health Organization classification in 2000. In this study we report the survival of patients with myeloid malignancies, classified by updated criteria, in Europe. We analyzed 58,800 cases incident between 1995 to 2002 in 48 population-based cancer registries from 20 European countries, classified into HAEMACARE myeloid malignancy groupings. The period approach was used to estimate 5-year relative survival in 2000-2002. The relative overall survival rate was 37%, but varied significantly between the major groups: being 17% for acute myeloid leukemia, 20% for myelodysplastic/myeloproliferative neoplasms, 31% for myelodysplastic syndromes and 63% for myeloproliferative neoplasms. Survival of patients with individual disease entities ranged from 90% for those with essential thrombocythemia to 4% for those with acute myeloid leukemia with multilineage dysplasia. Regional European variations in survival were conspicuous for myeloproliferative neoplasms, with survival rates being lowest in Eastern Europe. This is the first paper to present large-scale, European survival data for patients with myeloid malignancies using prognosis-based groupings of entities defined by the third revision of the International Classification of Diseases for Oncology/World Health Organization classifications. Poor survival in some parts of Europe, particularly for treatable diseases such as chronic myeloid leukemia, is of concern for hematologists and public health authorities.

Long-term survivors of childhood cancer suffer from a higher mortality than the general population. Here we evaluate late and very late mortality, and patterns of causes of death, in 5-year survivors after childhood and adolescent cancer in cases diagnosed during four decades in the five Nordic countries. The study is population-based and uses data of the nationwide cancer registries and the cause of death registers. There were in all 37,515 incident cases, diagnosed with cancer before the age of 20 years, between 1960 and 1999. The 5-year survivor cohort used in the mortality analyses consisted of 21,984 patients who were followed up for vital status until December 31, 2005 (Norway, Sweden) or 2006 (Denmark, Finland, Iceland). At the latest follow-up, 2,324 patients were dead. The overall standardized mortality ratio was 8.3 and the absolute excess risk was 6.2 per 1,000 person-years. The pattern of causes of death varied markedly between different groups of primary cancer diagnosis, and was highly dependent on time passed since diagnosis. With shorter follow-up the mortality was mainly due to primary cancer, while with longer follow-up, mortality due to second cancer and noncancer causes became more prominent. Mortality between 5 and 10 years after diagnosis continued to decrease in patients treated during the most recent period of time, 1990-1999, compared to previous periods, while mortality after 10 years changed very little with time period. We conclude that improvement of definite survival demands not only reducing early but also late and very late mortality., (Copyright © 2011 UICC.)

Background: Merkel cell carcinoma (MCC) is a rare malignancy of the skin, and its incidence is reported to be rising. The purpose of this study was to calculate its incidence and survival ratios, and to describe the clinical characteristics of Merkel cell carcinoma patients in Finland., Methods: We calculated the incidence of MCC based on data from the Finnish Cancer Registry. In addition, patient files from hospitals and primary health care centres were reviewed for detailed data on the treatment and disease recurrence of 181 patients diagnosed with MCC in Finland during 1983-2004, and relative survival ratios were calculated for them., Results: The incidence (per 100,000) of MCC in Finland in 1989-2008 was 0.11 for men and 0.12 for women, adjusted for age to the world standard population. The mean age at diagnosis was 76 years (range 27-100), and 69% of the patients were women. The most common site of the primary tumour was the head and neck (53%). No extra benefit was gained from a wide surgical margin (≥ 2 cm) compared to a margin of 0.1-0.19 cm, but an intralesional excision was more often associated with local recurrence. None of the patients with Stage I-II disease who had received postoperative radiotherapy to the tumour bed had a local recurrence. The 5-year relative survival ratio amongst men was 36% (95% confidence interval 20-54%), and amongst women 69% (56 to -82%)., Conclusions: MCC is a rare disease in Finland, with incidence rates similar to those in the other Nordic countries. Our results support the view that complete excision with clear margins and post operative radiotherapy decrease local recurrences., (Copyright © 2011 Elsevier Ltd. All rights reserved.)