Tumours In Sweden Research Articles

Cost-effectiveness of proton beam therapy vs. conventional radiotherapy for patients with brain tumors in Sweden: results from a non-randomized prospective multicenter study

BackgroundThis study assessed the cost-effectiveness of proton beam therapy (PBT) compared to conventional radiotherapy (CRT) for treating patients with brain tumors in Sweden.MethodsData from a longitudinal non-randomized study performed between 2015 and 2020 was used, and included adult patients with brain tumors, followed during treatment and through a one-year follow-up. Clinical and demographic data were sourced from the longitudinal study and linked to Swedish national registers to get information on healthcare resource use. A cost-utility framework was used to evaluate the cost-effectiveness of PBT vs. CRT. Patients in PBT group (n = 310) were matched with patients in CRT group (n = 40) on relevant observables using propensity score matching with replacement. Costs were estimated from a healthcare perspective and included costs related to inpatient and specialized outpatient care, and prescribed medications. The health outcome was quality-adjusted life-years (QALYs), derived from the EORTC-QLQ-C30. Generalized linear models (GLM) and two-part models were used to estimate differences in costs and QALYs.ResultsPBT yielded higher total costs, 14,639 US$, than CRT, 13,308 US$, with a difference of 1,372 US$ (95% CI, -4,914–7,659) over a 58 weeks’ time horizon. Further, PBT resulted in non-significantly lower QALYs, 0.746 compared to CRT, 0.774, with a difference of -0.049 (95% CI, -0.195–0.097). The probability of PBT being cost-effective was < 30% at any willingness to pay.ConclusionsThese results suggest that PBT cannot be considered a cost-effective treatment for brain tumours, compared to CRT.Trial registrationNot applicable.

Change in tissue resistance after irreversible electroporation in liver tumors as an indicator of treatment success - A multi-center analysis with long term follow-up

IntroductionA nationwide multicenter study was performed to examine whether there is a correlation between decrease in tissue resistance and time to local tumor recurrence after irreversible electroporation (IRE) in patients with hepatocellular carcinoma (HCC) and colorectal cancer liver metastases (CRCLM). MethodsAll patients treated with IRE for liver tumors in Sweden from 2011 until 2018 were included. Patient characteristics and recurrence patterns were obtained from medical records and radiological imaging. All procedural data from the IRE hardware at the three hospitals performing IRE were retrieved. The resistance during each pulse and the change during each treatment were calculated. The electrode pair with the smallest decrease in tissue resistance was used and compared with the time to LTP. Results149 patients with 206 tumors were treated. Exclusion due to missing and inaccurate data resulted in 124 patients with 170 tumors for the analyses. In a multivariable Cox regression model, a smaller decrease in tissue resistance and larger tumor size were associated with shorter time to local tumor recurrence for CRCLM, but not for HCC. ConclusionThere was an association between a decrease in tissue resistance and time to local tumor recurrence for CRCLM. The decrease in resistance, in combination with a rise in current, may be the parameters the interventionist should use during IRE to decide if the treatment is successful.

The Burden of Multiple Basal Cell Carcinomas: A Population-wide Study.

Basal cell carcinoma (BCC) is a common skin cancer type and affected individuals are known to be at risk of developing multiple consecutive tumours. Research into BCC multiplicity has, thus far, been challenging, due to a lack of national registration. This registry-based cohort study aimed to analyse the occurrence of multiple BCCs in Sweden, and risk factors for subsequent primary BCCs. Data regarding all histopathologically verified, primary BCC tumours in Sweden from 2004 to 2017 was extracted from the Swedish BCC Registry. Risk of developing a subsequent BCC in relation to person-related factors was estimated with Cox regression analysis. Cumulative risk of BCC development after 1 or 3 earlier BCCs was estimated. In total, 39.9% of individuals with a registered BCC had at least 2 registered tumours. The risk of developing a subsequent BCC increased significantly in males, older age, and with residence in southern Sweden. The cumulative 5-year risk of developing an additional BCC after first diagnosis was approximately 30% in males and 27% in females and increased after multiple previous BCCs. This study showed the cumulative risk of a subsequent BCC to increase with a history of multiple BCCs, indicating the need for clinical surveillance in these individuals.

The Swedish childhood tumor biobank: systematic collection and molecular characterization of all pediatric CNS and other solid tumors in Sweden

The Swedish Childhood Tumor Biobank (BTB) is a nonprofit national infrastructure for collecting tissue samples and genomic data from pediatric patients diagnosed with central nervous system (CNS) and other solid tumors. The BTB is built on a multidisciplinary network established to provide the scientific community with standardized biospecimens and genomic data, thereby improving knowledge of the biology, treatment and outcome of childhood tumors. As of 2022, over 1100 fresh-frozen tumor samples are available for researchers. We present the workflow of the BTB from sample collection and processing to the generation of genomic data and services offered. To determine the research and clinical utility of the data, we performed bioinformatics analyses on next-generation sequencing (NGS) data obtained from a subset of 82 brain tumors and patient blood-derived DNA combined with methylation profiling to enhance the diagnostic accuracy and identified germline and somatic alterations with potential biological or clinical significance. The BTB procedures for collection, processing, sequencing, and bioinformatics deliver high-quality data. We observed that the findings could impact patient management by confirming or clarifying the diagnosis in 79 of the 82 tumors and detecting known or likely driver mutations in 68 of 79 patients. In addition to revealing known mutations in a broad spectrum of genes implicated in pediatric cancer, we discovered numerous alterations that may represent novel driver events and specific tumor entities. In summary, these examples reveal the power of NGS to identify a wide number of actionable gene alterations. Making the power of NGS available in healthcare is a challenging task requiring the integration of the work of clinical specialists and cancer biologists; this approach requires a dedicated infrastructure, as exemplified here by the BTB.

An analysis of gender differences in treatment and outcome of periampullary tumours in Sweden – A national cohort study

BackgroundLittle is known of possible gender differences in treatment of periampullary tumours and outcome after pancreatoduodenectomy (PD), and the aim of this study was therefore to investigate any variances from national multicentre perspective. MethodsData from the Swedish National Registry for Pancreatic and Periampullary Cancer for all patients diagnosed with a periampullary tumour from 2012 throughout 2017 was collected. The material was analysed in two groups, men and women, for palliative treatment and curative intended resection. ResultsA total of 5677 patients were included, 2906 (51%) men and 2771 (49%) women. Women were older than men, 72 (65–78) years vs. 70 (64–76), p < 0.001. A lesser proportion of women were planned for resection (1131 (41%) vs. 1288 (44%), p = 0.008), but after adjusting for age and tumour location no difference was seen. Postoperative morbidity was equal, but women had significantly better long-term survival than men. The survival was equal for palliative men and women. ConclusionNo gender bias could be established when analysing treatment for periampullary tumours in Sweden, even though less women were offered surgery. Data suggest that even though women were older they tolerate surgery well and hence offering PD at a higher age for women could be suggested.

Reproductive and obstetrical outcomes with the overall survival of fertile-age women treated with fertility-sparing surgery for borderline ovarian tumors in Sweden: a prospective nationwide population-based study

To assess the efficacy of fertility-sparing surgery (FSS) in terms of reproductive outcomes by following FSS for borderline ovarian tumors (BOTs) and comparing the safety of FSS versus radical surgery (RS). Nationwide cohort study based on prospectively recorded data. Sweden. All women of reproductive age (18-40 years) treated in Sweden for stage I BOT with the use of FSS or RS from 2008 to 2015, identified in the Swedish Quality Registry for Gynecologic Cancer (SQRGC). FSS or RS. Reproductive outcomes: natural conception, use of assisted reproductive technology (ART), live birth and obstetrical outcomes. Safety outcome: overall survival (OS) rates, comparing women undergoing FSS versus RS. The FSS cohort was linked to the Swedish Medical Birth Register to identify all women who had given birth after FSS and to obtain detailed obstetrical data. For information on ART treatment, the National Quality Registry for Assisted Reproduction was consulted. OS rate comparisons were conducted by means of Kaplan-Meier estimates. Of the 277 women with BOTs, 213 (77%) underwent FSS, 183 (86%) unilateral salpingo-oophorectomy, and 30 (14%) cystectomy. Following FSS, 50 women gave birth to 62 healthy children, 8% of which were preterm. Only 20 (9%) of the women underwent ART treatment. OS was similar in women treated with FSS and RS. Natural fertility was maintained after FSS; only 9% required ART treatment. FSS was also deemed to be equivalent to RS regarding survival outcome.

A nationwide, population-based study of school grades, delayed graduation, and qualification for school years 10-12, in children with brain tumors in Sweden.

As many as 95.7% of children diagnosed with a brain tumor will experience persistent late effects as adults. These include difficulties with general executive functions, lower IQ, and mental fatigue, which may negatively affect school performance. Through the Swedish Childhood Cancer Registry, we identified 475 children born between 1988 and 1996, diagnosed with a brain tumor before their 15th birthday. School grades in "Swedish," "mathematics," and "English," if their graduation was delayed, and qualification for school years 10-12 were compared with 2197 matched controls. Furthermore, we checked for interaction effects between sex and age at diagnosis, and possible effects of tumor grade (high or low) as well as parents' education. Children treated for a brain tumor performed worse in the subjects compared to controls and also had delayed graduation to a greater extent. Fewer children treated for a brain tumor than controls qualified for school years 10-12. Children treated at a young age, especially females, and children whose parents have low education seem to be at particular risk. Unexpectedly, there were no differences in outcomes between survivors with high- and low-grade tumors. It is important that schools provide regular pedagogical assessment and individualized support to meet the different needs of children treated for a brain tumor. Children treated for low-grade tumors do not perform better than children treated for high-grade tumors, despite the lighter treatment, and hence require the same attention and support.

Treatment Patterns and Survival among Patients with Metastatic Gastroenteropancreatic Neuroendocrine Tumours in Sweden - a Population-based Register-linkage and Medical Chart Review Study.

Background: Gastroenteropancreatic neuroendocrine tumours (GEP-NETs) are neoplasms derived from the endocrine system in the gastrointestinal tract and pancreas. Treatment options include surgery; pharmacological treatments like somatostatin analogues (SSA), interferon alpha, molecular targeted therapy and chemotherapy; and peptide receptor radionuclide therapy.The objective of this study was to describe treatment patterns and survival among patients with metastatic GEP-NET grade 1 or 2 in Sweden.Methods: Data was obtained via linkage of nationwide registers. Patients diagnosed with metastatic GEP-NET grade 1 or 2 in Sweden between 2005 and 2013 were included (n=811; National population). In addition, medical chart review was performed for the subpopulation diagnosed at Sahlgrenska University Hospital, Gothenburg (n=127; Regional population). Treatment patterns, including treatment sequences, and overall survival were assessed.Results: Most patients had small intestinal NET (76%). In the regional population, 72% had grade 1 tumours; 50% had functioning tumours. The two most common first-line treatments were surgery (57%) and SSA (25%). After first-line surgery, 46% received SSA, while 40% had no further treatment. After first-line SSA, 52% received surgery, while 27% had no further treatment. Overall median survival time from date of diagnosis was 7.0 years (95% CI 6.2-not reached). Among patients with distant metastases, pancreatic NET (vs. small intestinal NET) was associated with poorer survival (HR 1.9; 95% CI 1.1-3.3), as were liver metastases (HR 3.2; 95% CI 1.5-7.0).Conclusions: First-line surgery was typically followed by SSA or no further treatment. Among patients with distant metastases, pancreatic NET or liver metastases were associated with a poorer survival.

Cost-effectiveness of Pembrolizumab for Patients with Advanced, Unresectable, or Metastatic Urothelial Cancer Ineligible for Cisplatin-based Therapy

BackgroundThere is an unmet need for effective therapies for patients with advanced or metastatic urothelial cancer who cannot tolerate cisplatin-based chemotherapy. Cisplatin-ineligible patients experience a high frequency of adverse events from the most commonly used standard of care treatment, carboplatin plus gemcitabine, or alternative treatment with gemcitabine monotherapy. Pembrolizumab is a potent, highly selective humanised monoclonal antibody that releases checkpoint inhibition of the immune response system, and provides a new alternative for these patients. ObjectiveTo assess the cost-effectiveness of pembrolizumab for first-line treatment of urothelial carcinoma ineligible for cisplatin-based therapy in patients with strongly PD-L1–positive tumours in Sweden. Design, setting, and participantsParametric survival curves were fitted to overall survival, progression-free survival, and time on treatment data from KEYNOTE-052 to extrapolate clinical outcomes. A simulated treatment comparison and a network meta-analysis were conducted to estimate the comparative efficacy of pembrolizumab versus carboplatin plus gemcitabine and gemcitabine monotherapy. EQ-5D data from KEYNOTE-052 were used to estimate utility, while resource use and cost inputs were estimated using Swedish regional pricing lists and clinician opinion. Outcome measurements and statistical analysisThe model reported costs, life years, and quality-adjusted life years (QALYs), and results were tested using deterministic and probabilistic sensitivity analysis. Results and limitationsWe estimated that pembrolizumab would improve survival by 2.11 and 2.16 years and increase QALYs by 1.71 and 1.75 compared to carboplatin plus gemcitabine and gemcitabine monotherapy, respectively. Pembrolizumab was associated with a cost increase of €90520 versus carboplatin plus gemcitabine and €95055 versus gemcitabine, with corresponding incremental cost-effectiveness ratios of €53055/QALY and €54415/QALY. ConclusionsAt a willingness-to-pay threshold of €100000/QALY, pembrolizumab is a cost-effective treatment versus carboplatin plus gemcitabine and versus gemcitabine. Patient summaryThis is the first analysis to show that pembrolizumab is a cost-effective option for first-line treatment of cisplatin-ineligible patients with locally advanced or metastatic urothelial carcinoma in Sweden.

PCN91 - Cost-of-Illness of Metastatic Gastroenteropancreatic Neuroendocrine Tumours In Sweden – A Register-Linkage Population-Based Study

PCN91 - Cost-of-Illness of Metastatic Gastroenteropancreatic Neuroendocrine Tumours In Sweden – A Register-Linkage Population-Based Study

Abstract P5-17-02: Treatment and prognosis of DCIS during twenty years. A population-based register study from a Swedish cohort

Abstract The increasing incidence of ductal carcinoma in situ (DCIS) of the breast has been attributed to the wide adoption of mammography screening programmes. The aim of the present study was to analyse trends in incidence, treatment and outcome of DCIS over a 20-year time period in a Swedish health care region, with a source population of two million, based on systemically collected data in a regional Breast Cancer Quality Registry started in 1992. All patients registered with a diagnosis of primary DCIS in the Breast Cancer Quality Registry in the Uppsala-Örebro healthcare region between 1992 and 2012 were included. The study period was divided into four time periods. The registry contains information on tumour characteristics, treatment and follow-up data and is linked to the Swedish cancer registry, to which reporting of all newly diagnosed malignant tumours in Sweden is mandated. To verify the validity of the Breast Cancer Quality Registry, 300 women recorded with a diagnosis of DCIS were randomly selected and their medical records were collected to compare clinical data, treatment data and subsequent breast cancer events compared to registry data. The completeness and reliability of the registration of most key variables were overall good, 91-99%. A total of 2,952 patients with DCIS were registered, of which eight were men. The proportion of DCIS to all diagnosed breast cancers was 9.5%, with no clear increase over time. The majority of the DCIS cases were detected by screening (68%). Tumour size increased over time; in 1992-1997 36.4% were larger than 15mm compared to 64.8% in 2008-2012. The frequency of mastectomy increased from 23.0% to 39.0% and the proportion of patients receiving adjuvant radiotherapy after breast-conserving surgery increased from 30.1% to 67.6%. Axillary lymph node clearance declined over time while the proportion of patients who underwent sentinel node biopsy increased from 1.4% in 1998-2002 to 33.9% in 2003-2007 and 54.9% in 2008-2012. 1992-19971998-20022003-20072008-2012P valueCases693628835796 DCIS size &amp;gt; 15mm252(36.4%)257(40.9%)339(53.8%)511(64.2%)&amp;lt;0.0001Mastectomy161(23.2%)150(23.9%)323(38.7%)313(39.3%)&amp;lt;0.0001BCS519(74.9%)468(74.5%)506(60.6%)476(59.8%)&amp;lt;0.0001BCS+RT156/519(30.0%)178/468(38.0%)347/506(68.6%)322/476(67.6%)&amp;lt;0.0001BCS= Breast conserving surgery, RT= radiotherapy There was no statistical difference in the cumulative incidence of recurrent breast events over time or between different treatment modalities. The relative 5- and 10- year survival rates were 99.0% and 97.0% respectively with no clear trend over time. In conclusion, while the proportion of DCIS did not increase over time between 1992 and 2012, there was a trend towards more intensified management to achieve local control. The increased tumor size over time could be secondary to a higher diagnostic activity, which clearly is manifested by the increased use of sentinel node biopsy. Citation Format: Wadsten C, Heyman H, Holmqvist M, Ahlgren J, Lambe M, Sund M, Wärnberg F. Treatment and prognosis of DCIS during twenty years. A population-based register study from a Swedish cohort. [abstract]. In: Proceedings of the Thirty-Eighth Annual CTRC-AACR San Antonio Breast Cancer Symposium: 2015 Dec 8-12; San Antonio, TX. Philadelphia (PA): AACR; Cancer Res 2016;76(4 Suppl):Abstract nr P5-17-02.

Long-term survival in women with borderline ovarian tumors: a population-based survey of borderline ovarian tumors in Sweden 1960-2007.

We conducted an evaluation of incidence and survival of women with borderline ovarian tumors in Sweden. All women diagnosed with borderline ovarian tumor in the Swedish Cancer Register 1960-2007 (n = 6252) combined with follow up in the Swedish Death Registry to 1 July 2009 were included. Estimation of age-standardized relative survival rate according to time periods for diagnosis. The incidence of borderline ovarian tumors increased during the study period, with a steep increase during the 1980s. The age standardized 5-year relative survival including all borderline tumors diagnosed 2000-07 was 97% (95% CI 92-99%). In women aged ≤64 years, the 10-year relative survival related to age at diagnosis of borderline tumors ranged from 95 to 98% and was 89% in women aged 65-74 years. In a multivariable analysis including age and decade of diagnosis relative survival for every decade increased. The 10-year relative survival in women with mucinous and serous borderline tumors did not differ significantly (p = 0.121). Results of the present study are reassuring about long-term survival in women with borderline ovarian tumors. The age-standardized relative survival rate increased across time periods for diagnosis. There was no difference in long-term survival between mucinous and serous borderline ovarian tumors.

Brain tumors in Sweden: Data from a population-based registry 1999–2012

Background. The Swedish brain tumor registry has, since it was launched in 1999, provided significant amounts of data on histopathological diagnoses and on important aspects of surgical and medical management of these patients. The purpose is mainly quality control, but also as a resource for research.Methods. Three Swedish healthcare regions, constituting 40% of the Swedish population, have had an almost complete registration. The following parameters are registered: diagnosis according to SNOMED/WHO classification, symptoms, performance status, pre- and postoperative radiology, tumor size and localization, extent of surgery and occurrence of postoperative complications, postoperative treatment, such as radiotherapy and/or chemotherapy, other treatments, complications and toxicity, occurrence of reoperation/s, participation in clinical trials, multidisciplinary conferences and availability of a contact nurse.Results. Surgical radicality has been essentially constant, whereas the use of early (within 72 hours) postoperative CT and MRI has increased, especially for high-grade glioma, which is a reflection of quality of surgery. Survival of patients with high-grade glioma has increased, especially in the age group 60–69. Patients aged 18–39 years had a five-year survival of 40%. Waiting times for the pathological report has been slightly prolonged. Geographical differences do exist for some of the variables.Conclusion. Population-based registration is valuable for assessment of clinical management, which could have impact on patient care. As a result of short survival and/or the propensity to affect cognitive functions this patient group has considerable difficulties to make their voices heard in society. We therefore believe that a report like the present one can contribute to the spread of knowledge and increase the awareness for this patient group among caregivers and policy makers.

European quality assurance programme in rectal cancer – are we ready to launch?

There have been initiatives to create a European audit project. This paper addresses the issue of differences in data collected by different registries. Patients with rectal cancer treated in 2008 and recorded in quality registries from Belgium, Germany/Poland, Spain and Sweden were analyzed. The comparison included number of patients, gender, age, American Society of Anesthesiology (ASA) classification, preoperative diagnostic and staging procedures, neoadjuvant therapy, surgical treatment and quality of surgery, postoperative complications and adjuvant treatment. The Belgian database consisted of 622 patients, the German/Polish database consisted of 3,393 patients, the Spanish database consisted of 1,641 patients and the Swedish database consisted of 1,826 patients. The percentage of patients in each ASA stage was highly variable. MRI use was highest in Spain and Sweden and very low in Germany/Poland. The percentage of cT4 stage tumours in Sweden was much higher than in all other countries. Sweden recorded the highest percentage of primary metastatic disease (20.3%) and Belgium recorded the lowest (10.2%). Neoadjuvant therapy in different protocols was administered to 41.2% patients in Germany/Poland, to 50.8% in Spain, to 55.2% in Belgium and to 62% in Sweden. Laparoscopic surgery (conversion rate) was performed for cure in 5% (28%) of patients in Sweden, in 20.8% (20.6%) in Spain, in 28.6% (15.2%) in Belgium and in 14.5% (8.9%) in Germany/Poland. The 30-day mortality for anterior resection, abdominoperineal excision and Hartmann's procedure in Sweden, Belgium and Spain was 2.0%, 2.3% and 3.1%, respectively. The German/Polish database reported an in-hospital mortality of 3.2%. A European quality assurance project in rectal cancer is possible only after data collection is standardized.

Incidence patterns of small bowel cancer in a population-based study in Sweden: Increase in duodenal adenocarcinoma

BackgroundIncreasing incidences of malignant small bowel tumours have been reported, but data from European populations are limited. This study aimed to clarify the incidence patterns of malignant small bowel tumours in Sweden. MethodsPatients with a first and primary malignant small bowel tumour were identified from the Swedish Cancer Register during the study period 1960–2009. Sex-specific and age-standardised incidence rates of these tumours were calculated by their anatomical location and histological type in different time periods. Figures were plotted to show the proportions and incidence rates over time, and joinpoint loglinear regression models were estimated to assess any time trends. ResultsA total of 6604 patients with malignant small bowel tumours were identified. The age-standardised incidence of all malignant small bowel tumours increased from 14.2 to 19.7 per 1,000,000 person-years during the study period. The incidence of duodenal cancer increased more than 3-fold (from 1.6 to 5.4 per 1,000,000 person-years), which was mainly expained by a dramatical rising trend of adenocarcinoma of the duodenum (from 0.7 to 4.2 per 1,000,000 person-years). Malignant tumours of small bowel with unspecified anatomical locations showed a slight increase (from 7.0 to 7.9 per 1,000,000). The incidence of small bowel tumours in other locations or of other histological types was more stable. ConclusionsThe incidence of small bowel malignancies has increased during the period 1960–2009. Among the specific types of small bowel cancer, a particularly rapid increase was found for duodenal adenocarcinoma.

Incidence and survival analyses in children with solid tumours diagnosed in Sweden between 1983 and 2007

Solid tumours constitute 40% of childhood malignancies. The Swedish Childhood Cancer Registry is population based and includes all children with cancer reported from the six paediatric oncology centres in Sweden. The aim was to investigate incidence and survival. We used the new WHO ICCC-3 for reclassification of the patients. Incidence and survival analyses were performed in the study population. Two thousand four hundred and eighty-seven children (<15 years) were diagnosed with solid tumours in Sweden between 1983 and 2007. The distribution of diagnoses was similar to that reported in other studies. The annual incidence was 65.3 per million children. The survival rates at 10 years of follow-up have improved significantly when comparing the two time periods, 1983-1995 and 1995-2007 (76 vs. 82%; p < 0.01). The mean annual incidence of solid tumours in children was 65.3/million and has been stable during the study period. Survival rates for solid tumours at 5, 10 and 20 years follow-up were 80, 79 and 76%, respectively.

Pediatric Organ Transplantation and Risk of Premalignant and Malignant Tumors in Sweden

Increased cancer risks are well documented in adult organ transplant recipients. However, the spectrum of malignancies and risk in the pediatric organ transplant population are less well described. We identified all solid organ transplanted patients aged <18 in Sweden between 1970-2007 (n = 536) in the National Patient Register and linked to the Cancer Register. Nationwide rates were used to calculate standardized incidence rate ratios and 95% CI estimating the association between transplant and cancer during maximum 36 years of follow-up. Nearly 7% of pediatric solid organ transplant recipients developed a premalignant or malignant tumor during follow-up. Transplantation was associated with an increased risk of any cancer (n = 24, SIR = 12.5, 95% CI: 8.0-18.6): non-Hodgkin lymphoma (NHL) (n = 13, SIR = 127, 95% CI: 68-217), renal cell (n = 3, SIR = 105, 95% CI: 22-307), vulva/vagina (n = 3, SIR = 665, 95% CI: 137-1934) and nonmelanoma skin cancers (n = 2, SIR = 64.7, 95% CI: 7.8-233.8). NHL typically appeared during childhood, while other tumors were diagnosed during adulthood. Apart from short-term attention toward the potential occurrence of NHL, our results suggest cancer surveillance into adulthood with special attention to skin, kidneys and the female genitalia.

Borderline ovarian tumors in Sweden 1960–2005: Trends in incidence and age at diagnosis compared to ovarian cancer

The aim of the study was to investigate long-term trends in the incidence of borderline tumors and ovarian cancer in Sweden during 1960-2005, based on data from the population-based Swedish Cancer Register. We identified 6,288 patients with borderline ovarian tumors and a total of 34,977 cases of ovarian cancer during the study period. The age-standardized incidence of borderline ovarian tumors increased from 1.0 to 5.3 per 100,000 women-years from 1960-1964 to 2000-2005 and the incidence of ovarian cancer increased from 16.4 to 19.7 per 100,000 women-years from 1960-1964 to 1980-1989 and then declined to 16.6 per 100,000 women-years to the period 2000-2005. Borderline ovarian tumors comprised 15% of all primary ovarian neoplasms and the proportion increased from 8.3 to 23.6% during the study period. The median age at diagnosis for patients with borderline ovarian tumors and ovarian cancer was 55.2 and 61.6 years, respectively. In women younger than 40 years, 34% of all primary ovarian malignancies consisted of borderline ovarian tumors. For the 5 birth cohorts evaluated, the peaks of incidence occurred in stepwise younger age for each younger birth cohort for both borderline tumors and ovarian cancer. The increasing incidence of borderline ovarian tumors could be explained by an increase in diagnostic activity and by a lack of protective effect of oral contraceptive use. The decline in invasive tumors could be explained by a combination of factors, where the contribution of each is uncertain: shifting exposure to risk factors, a protective effect of oral contraceptive use, and increased detection of and removal of precancerous lesions.

Prenatal ultrasound and the risk of childhood brain tumour and its subtypes

We carried out a nationwide case–control study of childhood brain tumours in Sweden (n=512) by histological subtype in relation to prenatal ultrasound, extracting data from antenatal records and the Medical Birth Register. We found no increased risk for brain tumour after ultrasound exposure, either for all tumours or for any subgroup.

A hypothesis-generating search for new genetic breast cancer syndromes – a national study in 803 Swedish families

Among Swedish families with an inherited predisposition for breast cancer, less than one third segregate mutations in genes known to be associated with an increased risk of breast cancer in combination with other types of tumours. In a search for new putative familial breast cancer syndromes we studied Swedish families undergoing genetic counselling during 1992-2000.Four thousand families from counselling clinics in Sweden were eligible for study. Families with breast cancer only were excluded, as were families with mutations in genes already known to be associated with malignant diseases. We identified 803 families with two or more cases of breast cancer and at least one other type of cancer. The observed proportion of different types of non-breast cancer was compared with the percentage distribution of non-breast cancer tumours in Sweden in 1958 and 1999.We found tumours in the colon, ovary, endometrium, pancreas and liver, as well as leukaemia in a significantly larger proportion of the study population than in the general population in both years. These tumours were also seen among families where several members had one additional tumour, suggesting that malignancies at these sites, in combination with breast tumours, could constitute genetic syndromes. Endometrial carcinoma has not previously been described in the context of breast cancer syndromes and the excess of malignancies at this site could not be explained by secondary tumours. Thus, we suggest that endometrial carcinoma and breast cancer constitute a new breast cancer syndrome. Further investigation is warranted to categorize phenotypes of both breast and endometrial tumours in this subgroup.