BACKGROUND: Germline genetic susceptibilities of rare cancers of the esophagus, stomach, small intestine, testis, (nonmedullary) thyroid gland and bone with high familial risks are not well known. Here, we use familial risk data from the Swedish Family-Cancer Database which contains records of cancers in Swedish families obtained over a century. We compare familial risks for offspring diagnosed with any of these cancers when their parent had or had not that cancer. We review the global literature of the reported constitutional variants that may explain part of the familial risk. MAIN BODY: Familial risks for esophageal and stomach cancers are about 2.0 and apart from early-onset stomach cancer few high-risk variants are known. Genetic studies may be hampered by dominant environmental risk factors for these cancers. Small intestinal carcinoids have a very high familial risk (28 between siblings) but no high-risk genes have been identified to explain this. Low-risk polygenic variants have been identified. Small intestinal adenocarcinoma is a manifestation in Lynch syndrome. Testicular and thyroid cancers are characterized by high familial risk (about 5) which may be explained largely by a polygenic background, although thyroid cancer is a component in a number of rare cancer syndromes. Several predisposing genes have been identified for bone cancer (familial risk 7). CONCLUSIONS: The discussed cancers are rare and they present with a relatively high familial risk, in spite of lacking identified high-penetrant constitutional variants. It is possible that the polygenic component, already recognized for testis cancer, is stronger than previously expected. Thus polygenic models with rare high/moderate- and low-risk variants could fit the familial risk and shape the germline genetic landscape of these cancers. Polygenic background may have clinical implications.
- Publikační typ
- časopisecké články MeSH
- přehledy MeSH
INTRODUCTION: Thyroid cancer (TC) is diagnosed in several histological types which differ in their clinical characteristics and survival. We aim to describe how they influence TC survival in Sweden. METHODS: Cancer data were obtained from the Swedish cancer registry between years 1999 and 2018, and these were used to analyze relative survival. RESULTS: Relative survival for all TC improved when analyzed in 10-year periods, and female survival improved more than male survival. Female survival advantage appeared to be present also for specific histological types, although case numbers were low for rare types. Female 5-year relative survival for TC was 100% for follicular, 95.1% for oncocytic, 93.4% for papillary, 89.7% for medullary, and 6.1% for anaplastic cancer. Among the clinical TNM classes, only T4 and M1 stages were associated with decreased survival compared to T1-3 and M0. Anaplastic cancer presented most often at high T and M1 stages, in contrast to other TC. Curiously, the diagnostic age for anaplastic M1 patients was lower than that for M0 patients. Both anaplastic and medullary cancers did not show age-dependent increases in the probability of metastases, in contrast to the main histological types. This could indicate the presence of several types of anaplastic and medullary cancers. CONCLUSION: The poor survival for anaplastic TC is an extreme contrast to the excellent survival of differentiated TC. As less than 20% of anaplastic cancer patients survived one year, urgent diagnosis and initiation of treatment are important. Facilitated treatment pathways have been instituted in Denmark resulting in improved survival. Anaplastic cancer should be a target of a major research focus.
- Publikační typ
- časopisecké články MeSH
OBJECTIVES: We describe age-specific survival in thyroid cancer (TC) from Denmark, Finland, Norway, and Sweden over a 50-year period. DESIGN: Population-based survival study. METHODS: Relative 5-year survival data were obtained from the NORDCAN database for the years 1972-2021. RESULTS: In the first period 1972-1976, 5-year survival in TC in Finland, Norway, and Sweden was 90% or higher, but a strong negative step-wise age gradient was observed, which was worse for men than women. Over time, survival increased, and in the final period, 2017-2021, survival for all women and Danish men up to age 69 years was about 90% or higher and, for men from the other countries, only marginally lower. Even for older women survival reached 80%, for older men somewhat less. CONCLUSIONS: Age disadvantage in TC survival was for the most part corrected over the 50-year period, and the remaining task is to boost survival for the oldest patients.
- MeSH
- incidence MeSH
- lidé MeSH
- míra přežití MeSH
- nádory štítné žlázy * terapie MeSH
- registrace MeSH
- rizikové faktory MeSH
- senioři MeSH
- věkové rozložení MeSH
- Check Tag
- lidé MeSH
- mužské pohlaví MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- Geografické názvy
- Dánsko MeSH
- Finsko MeSH
- Norsko MeSH
- Skandinávie a severské státy MeSH
- Švédsko MeSH
OBJECTIVES: We analyze survival in thyroid cancer from Denmark (DK), Finland (FI), Norway (NO), and Sweden (SE) over a 50-year period (1971-2020), and additionally consider concomitant changes in incidence and mortality. DESIGN: Population-based survival study. METHODS: Relative 1-, 5/1 (conditional)-, and 5-year survival data were obtained from the NORDCAN database for years 1971-2020. Incidence and mortality rates were also assessed. RESULTS: A novel consistent observation was that 1-year survival was worse than 5/1-year survival but the difference between these decreased with time. Relative 1-year survival in thyroid cancer (mean for the 4 countries) reached 92.7% for men and 95.6% for women; 5-year survival reached 88.0% for men and 93.7% for women. Survival increased most for DK which started at a low level and reached the best survival at the end. Male and female incidence rates for thyroid cancer increased 3- and 4-fold, respectively. In the same time, mortality halved for men and for women, it decreased by 2/3. CONCLUSIONS: We documented worse relative survival in the first year than in the 4 subsequent years, most likely because of rare anaplastic cancer. Overall survival in thyroid cancer patients increased in the Nordic countries in the course of 50 years; 5-year survival was close to 90% for men and close to 95% for women. Even though overdiagnosis may explain some of 5-year survival increase, it is unlikely to influence the substantial increase in 1-year survival. The unmet need is to increase 1-year survival by diagnosing and treating aggressive tumors before metastatic spread.
- MeSH
- databáze faktografické MeSH
- lidé MeSH
- nádory štítné žlázy * diagnóza epidemiologie MeSH
- Check Tag
- lidé MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- Geografické názvy
- Finsko MeSH
- Norsko MeSH
Global survival studies have shown favorable development in most cancers but few studies have considered laryngeal cancer, particularly over extended periods or in populations for which medical care is essentially free of charge. We analyzed laryngeal and lung cancer incidence and survival in Finland (FI) and Sweden (SE) over a 50-year period (1970-2019) using data and statistical tools from the Nordcan database. Laryngeal cancer reached an incidence maximum in FI men in 1965, which in SE men occurred over 10 years later and peaking at 42% of the FI maximum. The FI incidence halved in 20 years while halving of the SE rate took almost twice as long. At maximum the male rate exceeded the female rate 20 times in FI and 10 times in SE. Incidence rates for lung cancer were approximately 10 times higher than those for laryngeal cancer, and they peaked 5 to 10 years after laryngeal cancer in both countries. The female lung cancer rates increased through the follow-up time but laryngeal cancer rates were relatively stable. Relative 1-year survival data for laryngeal cancer remained at around 85% through 50 years, and 5-year survival lagged constantly around 65%. For lung cancer 1-year survival improved and reached about 50% by 2019. Even 5-year survival improved reaching 20 to 30%, except for FI men. Incidence rates for laryngeal and lung cancers have drastically decreased in FI and SE men parallel to reduced smoking prevalence. In females, rates have clearly increased in lung but not in FI laryngeal cancer. This finding warrants further investigations into possible contributing factors, other than smoking. Survival in laryngeal cancer has not improved compared to the positive development in lung cancer. Historical smoking prevalence was unrelated of survival trends. As long-term survival in these cancers remains discouraging, the most efficient way to fight them is to target the main cause and promote non-smoking.
- MeSH
- incidence MeSH
- lidé MeSH
- nádory hrtanu * epidemiologie MeSH
- nádory plic * epidemiologie MeSH
- Check Tag
- lidé MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- práce podpořená grantem MeSH
- Geografické názvy
- Finsko MeSH
- Švédsko MeSH
