Materiały źródłowe

• #1 Exploring the current status of neuroendocrine tumours: a population-based analysis of epidemiology, management and use of resources | BMC Cancer | Full Text

  https://bmccancer.biomedcentral.com/articles/10.1186/s12885-019-6412-8

  Neuroendocrine tumours (NETs) are rare malignancies characterised by its capacity to synthesise and secrete monoamines, due to its neuroendocrine origin. Its varied locations and symptoms have traditionally been responsible for extended delays in their diagnosis. […] Data collected over the past 20years in several European countries and the USA estimated an incidence of 15 per 100,000 inhabitants, with an increasing tendency over the past years. […] A delay of 52months on average has been reported in patients with NETs between the first symptoms and diagnosis, and it is common for patients to be examined by several different doctors before receiving the correct diagnosis. […] A 2 fold increase was measured in the number of NETs diagnoses between 2010 and 2015. All data suggest that tumours primarily affect the pancreas, lung and the gastrointestinal system, and that tumours are mainly resected, which supposes an important portion of economic costs.

• #2 Gastrointestinal neuroendocrine tumors in 2020

  https://www.wjgnet.com/1948-5204/full/v12/i8/791.htm

  Neuroendocrine tumors (NETs) arise from the diffuse system of neuroendocrine cells i.e. cells with features of both nerve cells (which can receive message from the nervous system) and endocrine cells (which have the ability to synthesize and secrete monoamines, peptides and hormones). The incidence and prevalence of NETs have been increasing over the last few decades. NETs constitute only 0.5% of all malignant conditions and 2% of all malignant tumors of the GI. In the United States, the incidence and prevalence of NETs have been increasing over the last few decades possibly due to early-stage detection, increased awareness, and widespread use of endoscopy and imaging studies for various gastrointestinal diseases. There was a 6.4-fold increase in annual age-adjusted incidence of NETs from 1973 (1.09 per 100000 persons) to 2012 (6.98 per 100000 persons). The prevalence also increased from 0.006% in 1993 to 0.048% in 2012. NETs are more prevalent in females than in males with a ratio of 2.5:1. The 2017 WHO Classification of GI-NETs is outlined in the Table 1. Gastrointestinal neuroendocrine tumors are diagnosed and staged by endoscopy with biopsy, endoscopic ultrasound, serology of biomarkers, imaging studies and functional somatostatin scans. Patients should be kept under surveillance program following treatment of GI-NETs.

• #3 Epidemiology, Incidence, and Prevalence of Neuroendocrine Neoplasms: Are There Global Differences?

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8118193/

  The purpose of our review is to explore global epidemiologic trends of gastroenteropancreatic (GEP) neuroendocrine tumors (NETs). Specifically, we sought to examine whether there are differences in incidence, prevalence, distribution (by primary tumor site, tumor grade, tumor stage at presentation), and overall survival of GEP NETs between different regions of the world. […] GEP NET incidence rates are rising steadily in North America, Asia, and Europe, though this rise appears to be most profound in North America. The distribution of GEP NETs differs regionally as in North America small intestinal and rectal NETs are most prevalent, in Asia rectal and pancreatic NETs are most prevalent, and in Europe small intestinal and pancreatic NETs are most prevalent. Overall survival for patients with GEP NETs appears to be improving with time.

• #4 SciELO Brazil – Epidemiological profile of neuroendocrine tumors in adults in Brazil Epidemiological profile of neuroendocrine tumors in adults in Brazil

  https://www.scielo.br/j/aem/a/zMTWhBXtjQ3ZWQj6dg6NYGF/

  Neuroendocrine tumors (NETs) are a set of diseases that originate from neuroendocrine cells, which comprises a diffuse endocrine system present in various organs of the body. These tumors are more frequent in the gastrointestinal tract (70%) and the bronchopulmonary system (20%-30%). A NET incidence rate of 1-5 per 100,000 inhabitants has been estimated for several European countries and the USA employing 20 years of data. However, no comprehensive studies on this rare neoplasm are available in Brazil. In this context, the aim of this study was to characterize the epidemiological NET profile in the country. […] A total of 15,859 cases were identified, most occurring in males (53.4%) and in individuals under 65 years old (63.3%). […] A NET incidence rate of 1-5 per 100,000 inhabitants has been estimated for USA and several European countries employing 20 years of data, confirming that NETs are a rare neoplasm.

• #5 Epidemiologic trends of and factors associated with overall survival in patients with neuroendocrine tumors over the last two decades in the USA in: Endocrine Connections Volume 12 Issue 12 (2023)

  https://ec.bioscientifica.com/view/journals/ec/12/12/EC-23-0331.xml

  Updated epidemiological data of neuroendocrine tumors are currently lacking. Thus, we performed epidemiological and survival analyses on a large cohort of patients with neuroendocrine tumors and developed a new nomogram to predict survival. […] This population-based study examined 112,256 patients with neuroendocrine tumors between 2000 and 2018 using data from the Surveillance, Epidemiology, and End Results program. […] The age-adjusted incidence per 100,000 persons of neuroendocrine tumors increased from 4.90 in 2000 to 8.19 in 2018 (annual percentage change, 3.40; 95% confidence interval, 3.133.67), with the most significant increases in grade 1, localized stage, and appendix neuroendocrine tumors. The age-adjusted mortality rate increased 3.1-fold from 2000 to 2018 (annual percentage change, 4.14; 95% confidence interval, 3.145.15). The 1-, 5-, and 10-year relative survival rates for all neuroendocrine tumors were 80.5%, 68.4%, and 63.5%, respectively. […] The incidence, prevalence, and mortality rate of neuroendocrine tumors continued to increase over the last two decades. Additionally, the nomogram could accurately quantify the risk of death in patients with neuroendocrine tumors and had good clinical practicability.

• #6 Epidemiology, Incidence, and Prevalence of Neuroendocrine Neoplasms: Are There Global Differences?

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8118193/

  The incidence and prevalence of NETs continues to rise globally, with the greatest rates of increase in nations such as the USA, Canada, and Norway. In the USA alone, the incidence of NETs has increased more than 6-fold over the last 4 decades, with a predominant rise in localized tumors rather than metastatic tumors; current disease prevalence of NETs in the USA approximates 170,000 patients. […] The largest retrospective population-based study querying the SEER database identified 64,971 NET patients between 1973 and 2012. Among primary tumor sites, GEP NETs demonstrated the highest incidence rate, with 3.56 cases per 100,000. […] The crude annual incidence of all GEP NENs was 5.83 cases per 100,000. The crude annual incidence of GEP NENs rose from 2.37 cases per 100,000 in 2003 to 8.35 cases per 100,000 in 2009; there was an increasing linear trend in crude incidence rates during the study period.

• #7 An updated analysis of the epidemiologic trends of neuroendocrine tumors in Taiwan | Scientific Reports

  https://www.nature.com/articles/s41598-021-86839-2

  The incidence of neuroendocrine tumors (NETs) has been increasing in recent decades. […] The incidence of NETs was 0.244 per 100,000 in 1996 and increased to 3.162 per 100,000 in 2015. […] Overall, the incidence of NETs in Taiwan has continued to increase. […] Rising incidence trends of NETs have been reported in many places around the world. […] The rising incidence trend of NETs is partly due to the increased awareness of NETs by the physicians and the execution of screening programs for prevalent cancers, particularly colorectal cancer. […] The incidence of all NETs increased from 0.244 per 100,000 in 1996 to 3.162 per 100,000 in 2015 [annual percentage change (APC)=15.44, P<0.0001]. [...] The incidence of pancreatic NET increased from 0.017 per 100,000 in 1996 to 0.446 per 100,000 in 2015 with an APC of 28.04 (P<0.0001). [...] The overall survival of pancreatic NETs improved after introduction of targeted therapies whereas the survival improvement of other gastrointestinal tract and pulmonary NETs awaits evaluation in the near future.

• #8 Epidemiological characteristics of neuroendocrine neoplasms in Beijing: a population-based retrospective study | BMC Public Health | Full Text

  https://bmcpublichealth.biomedcentral.com/articles/10.1186/s12889-024-18845-8

  The incidence of neuroendocrine neoplasms (NENs) is rising rapidly worldwide. […] Our study aimed to explore the epidemiological characteristics of NENs in Beijing. […] From 1998 to 2018, the incidence of NENs in Beijing initially showed a significant increasing trend, from 1.07/100,000 to 3.53/100,000; this began to plateau after 2013. […] The age-specific incidence rate increased with age and peaked in the age group 70-74 years. […] The incidence in men was significantly higher than that in women (4.41/100,000 vs. 1.69/100,000). […] Most NENs were diagnosed at a late stage. […] We found that NENs originating from the lung had worse overall survival than extrapulmonary NENs, and male patients had worse survival than female patients. […] Our findings provide a reference regarding the epidemiological statistics of NENs in Beijing to contribute to the prevention, diagnosis, and treatment of these specific tumors.

• #9 Epidemiology of Neuroendocrine Neoplasms and Results of Their Treatment with [177Lu]Lu-DOTA-TATE or [177Lu]Lu-DOTA-TATE and [90Y]Y-DOTA-TATE—A Six-Year Experience in High-Reference Polish Neuroendocrine Neoplasm Center

  https://www.mdpi.com/2072-6694/15/22/5466

  Neuroendocrine neoplasms (NENs) are tumors originating from neuroendocrine cells, with increasing global incidence and prevalence. The worldwide incidence and prevalence of the NENs are estimated to be 6/100,000 and 35/100,000, respectively. Those numbers are increasing every decade, requiring higher and higher diagnosis and treatment costs. The epidemiological data show that the annual incidence of NEN is estimated at 5.86 per 100,000 persons/year; this value continues to increase. The prevalence is estimated at 35/100,000 but may be considerably higher due to the occurrence of silent, non-functioning tumors. The primary aim of the present study was to analyze the epidemiology and outcomes of RLT for NEN patients in one of the biggest ENETS-certified centers in Poland. In long-term observation, RLT leads to disease stabilization in over half of the patients with progressive disease. Organized coordination of NEN treatment in high-reference centers ensures the continuity of patient care.

• #10 Rising Incidence of Neuroendocrine Tumors in Singapore: An Epidemiological Study

  https://www.wjoes.com/abstractArticleContentBrowse/WJOES/20681/JPJ/fullText

  The primary outcome studied was the trend in age-standardized incidence rates and based on our data, an almost fourfold increase in the age-standardized incidence rate was observed over the last two decades or so. […] In conclusion, the age-standardized incidence of NETs in Singapore has demonstrated an almost fourfold increase over the last 22 years.

• #11 Medwin Publishers | Epidemiology of Neuroendocrine Tumors (Nets) and Socio- Clinical Profile of Patients According to Data from the National Cancer Registry and the Federal Patient Registry of the MOLNEO Medical Society for Treatment of Neuroendocrine Tum

  https://medwinpublishers.com/article-description.php?artId=9278

  The Incidence of NETs has increased 6,4 – fold over the last 40 years. This growth is noted by statistics from most countries of the world. […] From 2017 to 2019 the number of NET patients included in the National Cancer Registry of the Russian Federation has increased from 21 668 NET patients (prevalence – 14.75 per 100,000 population) to 25,782. In 2019, the prevalence of NETs was 17.54 per 100,000 population. […] If over a 10- year period (2010 -2019) the number of patients with malignant tumors followed-up in oncology dispensaries has increased by 40.6%, the number of patients with confirmed diagnosis of neuroendocrine tumors increased by 83.9%. The number of newly identified cases of NETs increased much more intensively than number of all malignant tumor during the same period also: in 2019, the number of newly identified cases was 1.8-fold larger than it had been in 2010 – respectively 7,623 and 4,198 cases. The increase in the incidence of NETs has been 3.5-fold larger than the increase in the total number of cases of malignant tumors.

• #12 Neuroendocrine Tumors: Epidemiology | SpringerLink

  https://link.springer.com/chapter/10.1007/978-3-031-56968-5_3

  Neuroendocrine tumors are a class of malignancies that are increasing in prevalence and incidence around the world. […] National health databases suggest that the total numbers of cases of neuroendocrine malignancies are increasing but vary geographically and biologically. […] The cases of small intestinal neuroendocrine tumors are greatest in the United States and northern Europe, whereas in East Asia, rectal neuroendocrine tumors are the most common. […] The cases of pancreatic neuroendocrine tumors have also been rising. […] With evolving diagnostics and treatments, epidemiological data will change as staging and survival improve.

• #13 SciELO Brazil – Epidemiological profile of neuroendocrine tumors in adults in Brazil Epidemiological profile of neuroendocrine tumors in adults in Brazil

  https://www.scielo.br/j/aem/a/zMTWhBXtjQ3ZWQj6dg6NYGF/?lang=en

  Neuroendocrine tumors (NETs) are a set of diseases that originate from neuroendocrine cells, which comprises a diffuse endocrine system present in various organs of the body. These tumors are more frequent in the gastrointestinal tract (70%) and the bronchopulmonary system (20%-30%). A NET incidence rate of 1-5 per 100,000 inhabitants has been estimated for several European countries and the USA employing 20 years of data. However, no comprehensive studies on this rare neoplasm are available in Brazil. In this context, the aim of this study was to characterize the epidemiological NET profile in the country. […] A total of 15,859 cases were identified, most occurring in males (53.4%) and in individuals under 65 years old (63.3%). […] A NET incidence rate of 1-5 per 100,000 inhabitants has been estimated for USA and several European countries employing 20 years of data, confirming that NETs are a rare neoplasm.

• #14 Key Statistics for Pancreatic Neuroendocrine Tumor | American Cancer Society

  https://www.cancer.org/cancer/types/pancreatic-neuroendocrine-tumor/about/key-statistics.html

  Pancreatic neuroendocrine tumors (pNETs) are rare. Less than 2% of all cancers found in the pancreas each year are pNETs. […] The number of pNETs diagnosed each year; however, has been rising over time. This is thought to be partly because they are being found more often incidentally (by accident), when imaging tests such as CT or MRI scans of the abdomen are done for other reasons. […] Most people with pNETs are older, with the average age at diagnosis being 60.

• #15 Neuroendocrine Neoplasms of the Appendix: A Review of the Literature | Anticancer Research

  https://ar.iiarjournals.org/content/38/2/601

  Appendiceal neuroendocrine neoplasms (ANENs) comprise rare tumors of the appendix, mainly affecting young populations and characterized by a rather favorable prognosis. The aim of this review was to summarize the current knowledge on these neoplasms, focusing on the management and follow-up of such patients, which still remain under debate. ANENs account for 0.16-2.3% of appendectomies and are usually diagnosed incidentally. […] Gastrointestinal neuroendocrine tumors (GI-NETs), otherwise categorized as GI-neuroendocrine neoplasia (GI-NENs) by the European Neuroendocrine Tumor Society (ENETS), are increasingly diagnosed today with an estimated annual incidence rate of 2-5/100,000. Previous epidemiological data showed that appendiceal NENs (ANENs) were the most frequent neuroendocrine neoplasms of the GI tract. Nevertheless, their percentage of total GI-NENs has decreased from 17-28% to 2-5%, due to the concomitant overall rise in other types of GI-NEN.

• #16 Epidemiological characteristics of neuroendocrine neoplasms in Beijing: a population-based retrospective study | BMC Public Health | Full Text

  https://bmcpublichealth.biomedcentral.com/articles/10.1186/s12889-024-18845-8

  The standardized incidence rate of NETs was 0.24/100,000 in 1996 and rose to 3.16/100,000 in 2015 in Taiwan, China; this rate for gastroenteropancreatic NENs (GEP-NENs) in Japan was 3.53/100,000 in 2016. […] The incidence of NENs was also significantly correlated with increasing age. […] The age-specific incidence rate increased with age and peaked in the age group 70-74 years. […] The proportion of patients diagnosed as having an advanced stage of SCLC and LCNEC was 89.04%; the proportion diagnosed as having an advanced stage of pulmonary carcinoid tumor was 65.15%; and the proportion of patients diagnosed as having an advanced stage of extrapulmonary NEC and NET was 73.85% and 53.97%, respectively. […] The more advanced the disease stage, the worse the survival. […] The stage of NENs at diagnosis was found to be closely related to survival.

• #17 Gastrointestinal Neuroendocrine Tumors: Symptoms and Treatment

  https://my.clevelandclinic.org/health/diseases/24594-gastrointestinal-neuroendocrine-tumors

  GI NET affects about 4 in 100,000 people. Each year, about 8,000 adults in the U.S. are diagnosed with gastrointestinal endocrine tumors. The condition typically affects people aged 55 to 65, but it can affect adults of any age. GI NET is more common in white people than in Black people. Women are slightly more likely to develop GI NET than men. […] There are several ways to treat GI NET. Healthcare providers will recommend treatment based on your cancers grade, treatment side effects, your overall health and your personal preferences. Treatments include active surveillance, surgery and medical treatments. […] Unfortunately, theres no known way to prevent GI NET. Medical researchers have discovered some links between certain hereditary syndromes and/or medical conditions that may increase your risk of developing neuroendocrine tumors. Talk to your healthcare provider about your medical history and your familys medical history. They may recommend specific tests to monitor for early signs of GI NET so they can catch and treat tumors early on.

• #18 Neuroendocrine Neoplasms of the Appendix: A Review of the Literature | Anticancer Research

  https://ar.iiarjournals.org/content/38/2/601

  There seems to be a slight female predominance for ANENs, whereas small bowel NENs are more common in men. In contrast to other appendiceal tumors and other NENs, which tend to occur in older patients, ANENs show highest incidence rates at 15-19 years of age in women and 20-29 years in men. […] Follow-up for patients with small tumors (1 cm) treated with appendectomy and excised in clear margins (R0) is not suggested by the ENETS guidelines. Furthermore, follow-up is also not mandatory for ANENs larger than 1 cm for which right hemicolectomy was implemented, no additional risk factors were present and no lymphovascular invasion or residual disease were identified in the histological examination. On the contrary, although not completely evidence-proven, according to the latest guidelines, long-term follow-up is needed when lymph node involvement is present, locoregional disease is identified postoperatively, as well as in cases in which the tumor is of high stage. Regular monitoring is necessary for patients with tumors sized between 1 and 2 cm with features indicating a higher risk for lymph node dissemination of the disease, such as mesoappendiceal invasion 3 mm, localization in the base of the appendix, vascular infiltration or intermediate differentiation (G2). […] Despite their indolent course, ANENs may relapse. Therefore, lifetime observation is necessary for those with tumors 2 cm, or 1 cm with additional risk factors.

• #19 Global burden of neuroendocrine tumors and changing incidence in Kentucky | Oncotarget

  https://www.oncotarget.com/article/24983/text/

  The cause of this increase is not entirely clear but experts have attributed the pattern to an increased awareness and improved diagnostics. The hike in incidence of this otherwise rare tumor has alerted public health stakeholders, which has resulted in establishment of specialized NET registries worldwide. […] Based on our analysis, the incidence of NETs reported in Kentucky according to the KCR database increased from 3.1 (1995) to 10.3 (2015) per 100,000 cases. In contrast, according to SEER data, the incidence increased from 3.9 to 6.61 between 1995 and 2012.

• #20 Rectal neuroendocrine tumors: Current advances in management, treatment, and surveillance

  https://www.wjgnet.com/1007-9327/full/v28/i11/1123.htm

  An increasing incidence of r-NENs has been reported over the past few years, as illustrated in The Surveillance, Epidemiology, and End Results (SEER) database, and the same trend was confirmed in the German registry, as well as in the Asian registers, even if the highest incidence rate (IR) was reported in the United States, where IR was approximately 1.1 per 100000 population and increased tenfold between 1970 and 2000s. In Europe, the IR was lower when compared to the SEER database, with the highest IR reported in Norway (0.25 per 100000 population) and the lowest in Austria. This is probably due to an underreporting of the disease because of a lack of national registries. […] The increasing incidence is related to the increased participation in screening colonoscopy programs. Even if there are limited data on the r-NENs diagnosed through colorectal cancer (CRC) screening programs, in a Polish screening CRC program cohort of 50148 participants, a prevalence of r-NENs of 0.05%-0.07% was reported. Similarly in another English study, the diagnosis rates of NENs identified in the English bowel cancer screening program were 29 rectal and 18 colonic per 100000 colonoscopies, accounting for a prevalence of 0.047%.

• #21 Epidemiology, treatment and outcomes of gastroenteropancreatic neuroendocrine neoplasms | Scientific Reports

  https://www.nature.com/articles/s41598-024-81518-4

  To investigate incidence, treatment patterns and outcomes of gastroenteropancreatic neuroendocrine neoplasms (GEP-NEN) in the United States. Overall survival was evaluated using averaged Cox regression. GEP-NEN incidence increases, likely due to improved detection and diagnosis. Treatment patterns have evolved to follow the latest international guidelines and site-specific improvement in survival is noted. Although GEP-NEN are considered rare, several large population-based studies have reported an increasing incidence over the past four decades, possibly attributable to increased use of cross-sectional imaging and endoscopic procedures and more widely adopted classification schemes. In our cohort, treatment effectiveness varied by stage and grade: across all strata, patients treated with surgical resection demonstrated longest overall survival. This is in line with international guidelines that recommend surgical resection as the mainstay of treatment for low grade local or locoregional GEP-NEN. The addition of systemic therapy was most effective in high stage G3 NEN. Factors associated with improved survival on multivariate analysis included having higher income ($63,333) having private insurance, Medicare, or other government insurance, and receiving treatment at an academic center.

• #22 Gastrointestinal Neuroendocrine Tumors: Symptoms and Treatment

  https://my.clevelandclinic.org/health/diseases/24594-gastrointestinal-neuroendocrine-tumors

  Your prognosis, or expected outcome, may depend on when a tumor was diagnosed and treated. Approximately 97% of people with GI NET who received treatment before the tumor spread were alive five years after diagnosis. That five-year survival rate drops to 95% if the tumor spreads to nearby tissues or lymph nodes. Approximately 67% of people whose GI NET spread outside of their digestive tracts were alive five years after diagnosis.

• #23 GI Neuroendocrine Tumors Increasing in Incidencelogo-32logo-40logo-60NEJM Journal WatchnejmJW_1L_RGB-b

  https://www.jwatch.org/na54176/2021/10/07/gi-neuroendocrine-tumors-increasing-incidence

  Survival rates differ according to tumor grade, primary site, age, and tumor size. Gastroenteropancreatic (GEP) neuroendocrine tumors (NETs) are a rare, heterogenous group of diseases with a natural history ranging from indolent to highly aggressive. There is evidence of increased incidence of these tumors from recent retrospective series. Investigators now report a cohort study evaluating epidemiologic and survival data in nearly 44,000 patients with GEP NETs from 1975 to 2015 from the U.S. Surveillance, Epidemiology, and End Results (SEER) Program. […] Incidence was similar in men and women, with age-adjusted incidence rates increasing over time. Rates increased similarly across races and some ethnicities but were more stable in American Indian and Alaska natives. Rates of increase were highest for localized and for grade 1 NETs, and rectal and small bowel tumors had the highest prevalence. Mean age at diagnosis for localized disease increased by 9 years over the study period. […] This large series supports the observation of an increased incidence of GEP NETs. An increase in diagnosis may result from increasing use of cross-sectional imaging, and overall survival may continue to improve with the advent of more effective systemic and regional therapies.

• #24 Trends of Incidence and Survival of Gastrointestinal Neuroendocrine Tumors in the United States: A Seer Analysis

  https://www.jcancer.org/v03p0292.htm

  The overall incidence rate of gastrointestinal NETs has increased since 1975 and is evident for all gastrointestinal organ sites (P 0.001), except NETs originating in the appendix (P = 0.466). […] We found that NETs of the rectum and appendix had the best prognosis, with 95.6% and 90.3% 5-year disease-specific survival, respectively (Table 3). […] Prognosis was also evaluated by decade of diagnosis. […] What is promising is that survival rates for colon NETs seem to have significantly improved over time. […] However, the improved survival for colon and small intestinal NETs was limited to non-Hispanic whites and not observed among blacks or Hispanics. […] To conclude, gastrointestinal NETs are a disease entity with increased incidence rates among all anatomic sites, except for appendiceal NETs. However, disparities are seen in survival rates according to anatomic location, race/ethnicity, and geographic region.

• #25 Epidemiological characteristics of neuroendocrine neoplasms in Beijing: a population-based retrospective study | BMC Public Health | Full Text

  https://bmcpublichealth.biomedcentral.com/articles/10.1186/s12889-024-18845-8

  The rising trend in rural incidence was obviously higher than that in urban incidence, and the gap narrowed gradually in the first decade; the suburban incidence exceeded that of urban incidence and maintained a certain gap in the second decade. […] The median follow-up was 10.24 years. […] The OS differed significantly according to the histology subtype as well as by sex. […] The 5-year OS rate of pulmonary NENs was 19.45% and that of extrapulmonary NENs was 54.02%. […] The prognosis of NENs from the rectum and appendix was the best, with a median survival time of 24.6 years and 30.0 years, respectively. […] This study will contribute to the diagnosis and management of these diseases.

• #26 The Epidemiology of Neuroendocrine Tumors in Taiwan: A Nation-Wide Cancer Registry-Based Study | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0062487

  The incident NET cases diagnosed between January 1, 1996 and December 31, 2008 were identified from the TCR, which was established in 1979 to monitor the incidence and the mortality rates of cancer in Taiwan. […] The crude annual incidence rates of NETs in Taiwan from 1996 to 2008 were calculated for all sites combined, by each site, and by sex, using the annual population reported by the Directorate-General of Budget, Accounting, and Statistics of Taiwan. […] The 5-year observed survival was 50.4% for all NETs (43.4% for men and 61.8% for women, P0.0001). […] Using the TCR data, we observed that the age-standardized incidence rate of NETs in Taiwan increased steadily from 1996 to 2001 and in a more accelerated speed since 2002. […] Despite such increase, the age-standardized incidence rate of NETs in Taiwan remained lower than those of Norway and the US.

• #27

  https://journals.lww.com/10.1097/XCS.0000000000000105

  Pancreatic neuroendocrine tumor (PNET) survival outcomes differ by race. Current recommendations for surveillance of PNETs less than 2 cm in size are based on low malignant potential and low rates of lymph node metastases (LNM). […] The current recommendation for surveillance of PNETs of less than 2 cm in size is likely based on a low rate of LNM seen in a predominantly White population. The incidence of LNM in Black patients with tumors less than 2 cm in size is clinically relevant and concerning. Current guidelines may not be universally applicable, and a more aggressive approach to resection in Black patients with small PNETs may be warranted. […] Black patients with pancreatic neuroendocrine tumors are more likely than White patients to have lymph node metastases with smaller tumors; current size-based guidelines are not likely applicable to this patient population.

• #28

  https://link.springer.com/article/10.1007/s41969-023-00217-z

  Neuroendocrine neoplasms (NEN) are rare tumors, mainly located in the lungs, pancreas or gastrointestinal tract. In some NENs the origin remains unidentified. They are referred to as cancer of unknown primary (CUP). Since only 9-14% of NENs are CUP, data about prognosis and therapy is scarce. Therefore, this paper aims to summarize the current knowledge on patients with CUP-NENs. […] The available literature indicated that the median age of onset was higher in CUP compared to NENs of known origin. CUP had a comparatively higher rate of poorly differentiated neoplasia. The recommended imaging modality was computed tomography (CT), complemented by positron emission tomography (PET)/CT, using 68Gallium-labeled somatostatin analogues (68Ga DOTATOC, DOTANOC or DOTATATE PET/CT). Surgical resection was suggested as first-line therapy. Other treatment options included chemotherapy, somatostatin analogues, molecular therapy and radiotherapy. Compared to NENs of known origin, CUP were associated with a worse prognosis.

• #29

  https://link.springer.com/article/10.1007/s41969-023-00217-z

  The current data suggest that CUP-NEN are frequently associated with older age and higher grade compared to patients with known-origin NENs. This reflected a worse prognosis for CUP-NENs. […] NENs are a relatively rare disease and represent only 0.5% of all malignancies. Thus the incidence of NENs is around 2/100,000. Epidemiological data suggest that the incidence has considerably increased in recent years. […] For CUP within NEN cohorts, rates from 4 to 16% of NEN are reported. […] A typical CUP-NEN patient is 56-72 years old without a clear gender preference. […] Patients with CUP-NEN have a worse prognosis compared to patients with a known primary NEN. This is not only the case in patients with NEN but with tumors in general. […] Considering CUP in NENs, median survival has been reported to be 15.5 months. Again, survival of patients with CUP was shown to be shorter (11 months) compared to other patients (19 months) with metastatic NEN of known origin. […] The prognosis of CUPs in NENs is worse. Nevertheless, in CUP-NENs there are 15-20% of patients that have less aggressive tumors which can be treated and stabilized.

• #30

  http://www.bccancer.bc.ca/books/neuroendocrine-tumors/follow-up-of-neuroendocine-tumors

  Follow-up of Neuroendocrine Tumours: […] Low-risk Resected Disease […] All of the following: primary less than 2 cm, no nodal involvement, and low Ki67 index (5%) […] Low risk of recurrence […] Routine surveillance imaging or lab work is not recommended. Clinical follow up at the discretion of the treating physician. […] High-risk Resected Disease […] Surveillance per the table below if any of the following high risk features: primary greater than 2 cm, nodal involvement, high Ki67 (5%) […] If high risk and grade 3 disease period of recommended follow-up is 5 years […] If high risk and grade 1-2 period of recommended follow-up is 10 years […] Post-debulking with no visible residual disease […] Unresected disease (metastatic or residual-positive post-debulking) […] SOURCE: Follow-up of Neuroendocrine Tumors ( ) Page printed: . Unofficial document if printed. Please refer to SOURCE for latest information.

• #31 Rectal neuroendocrine tumors: Current advances in management, treatment, and surveillance

  https://www.wjgnet.com/1007-9327/full/v28/i11/1123.htm

  Rectal neuroendocrine neoplasms (r-NENs) are considered among the most frequent digestive NENs, together with small bowel NENs. Their incidence has increased over the past few years, and this is probably due to the widespread use of endoscopic screening for colorectal cancer and the advanced endoscopic procedures available nowadays. According to the current European Neuroendocrine Tumor Society (ENETS) guidelines, well-differentiated r-NENs smaller than 10 mm should be endoscopically removed in view of their low risk of local and distant invasion. R-NENs larger than 20 mm are candidates for surgical resection because of their high risk of distant spreading and the involvement of the muscularis propria. There is an area of uncertainty regarding tumors between 10 and 20 mm, in which the metastatic risk is intermediate and the endoscopic treatment can be challenging. Once removed, the indications for surveillance are scarce and poorly codified by international guidelines, therefore in this paper, a possible algorithm is proposed.

• #32 Optimising Outcomes and Surveillance Strategies of Rectal Neuroendocrine Neoplasms

  https://www.mdpi.com/2072-6694/15/10/2766

  If baseline imaging demonstrates no evidence of nodal or metastatic disease, then, for small lesions <10 mm without lymphovascular invasion and with a curative R0 resection, patients can be discharged with no further follow up required. [...] For patients with rectal NET G1 <10 mm, however, with the presence of lymphovascular invasion, MRI and sigmoidoscopy follow up is required for 5 years. [...] For patients with an R1 resection of <10 mm NET without further resection, then 12 monthly MRI and sigmoidoscopy could be considered. [...] A significant percentage of large rectal NETs could be grade 3, in which case an FDG-PET should be performed in addition to SSTR-PET imaging.

• #33 Diagnosis and Management of Upper Gastrointestinal Neuroendocrine Tumors

  https://www.e-ce.org/journal/view.php?number=7053

  For type 1 g-NETs 1 cm, endoscopic resection is widely recommended in Europe. […] Surveillance after resection of d-NETs can depend on many factors, such as local treatment protocol, resources available and accessibility of certain imaging modalities especially outside tertiary centres. […] Generally, surveillance by upper gastrointestinal endoscopy is recommended every 2 years after resection of d-NET but this interval has yet to be validated.

• #34 Diagnosis and Management of Upper Gastrointestinal Neuroendocrine Tumors

  https://www.e-ce.org/journal/view.php?number=7053

  Upper gastrointestinal neuroendocrine tumors (NETs) are rare tumors which are increasingly diagnosed by endoscopists. […] It is recognised that there appears to be a true increased incidence but incidentally found lesions represent a significant portion of this rise in tumors discovered. […] One large US based population study has recently reported a staggering 15-fold increase in the incidence of gastric NETs (g-NETs) since the 1970s. […] The annual age-adjusted incidence of g-NETs is estimated to be around 0.2 per 100,000 in Europe and has been reported to be around 0.3 per 100,000 in the United States. […] G-NETs represent approximately 4.6% of all NETs, according to SEER. […] The current surveillance interval recommended by the European Neuroendocrine Tumor Society is between 1 to 2 years.

• #35 Active Surveillance for pNET | American Cancer Society | American Cancer Society

  https://www.cancer.org/cancer/types/pancreatic-neuroendocrine-tumor/treating/active-surveillance.html

  Active surveillance, also known as watchful waiting or watch-and-wait, may be recommended for certain people. This approach is most often recommended for those who have low-grade pancreatic neuroendocrine tumors, which tend to grow slowly and may not cause symptoms or complications for a long time (sometimes months or even years). […] Instead of starting treatment right away, the tumor is carefully monitored through regular check-ups. These typically include: Physical examinations, Blood tests, Imaging tests, such as CT scans (or sometimes MRI). […] If active surveillance is considered an option, talk with your doctor about how often you will need to have these tests and scans. Treatment usually begins if there are signs that the tumor is getting bigger and causing symptoms or if its spreading.

• #36 Practice Pearl #1 Active Surveillance vs Treatment in Patients with NETs

  https://www.pharmacytimes.com/view/practice-pearl-1-active-surveillance-vs-treatment-in-patients-with-nets

  Active surveillance could be recommended when a patient is diagnosed with low-grade NETs or they have asymptomatic or nonfunctional metastatic neuroendocrine tumors. These tumors are growing more slowly, and they might not cause any problems for the patients for months to years. We would not start the active treatment until the tumor really starts showing signs of progression or until the patient becomes more symptomatic. […] For patients who have relatively low-volume disease, we might not need treatment. One of the things we will definitely treat is for those patients who have excess hormonal secretion, just because we are trying to control the release of hormones from the neuroendocrine tumor rather than control the overall tumor bulk. As you mentioned, however, for many of these patients who have nonfunctional neuroendocrine tumors, relatively indolent active surveillance is certainly justified, and you really emphasized that key point: We really do not have data regarding early initiation of somatostatin analogs vs later initiation of somatostatin analogs in these patients in terms of potentially deriving an overall survival benefit, which at the end of the day is the gold standard in oncology.

• #37 Epidemiology of gastro-entero-pancreatic neuroendocrine tumors in Krakow and Krakow district area | ECE2014 | 16th European Congress of Endocrinology | Endocrine Abstracts

  https://www.endocrine-abstracts.org/ea/0035/ea0035p554

  Epidemiology of gastro-entero-pancreatic neuroendocrine tumors in Krakow and Krakow district area […] Cancer registers are important tools in improving the knowledge of epidemiology of rare malignancies, such as gastro-entero-pancreatic neuroendocrine tumors. […] The standardized incidence rates of GEPNETs in Krakow and Krakow district area ranged from 1.5 to 2.7 cases/100 000 persons per year, an average of 2.1/100 000 persons per year. The highest incidence was noted in the oldest patients (4.8/100 000 persons per year in subjects aged 60 years and older). […] GEPNET incidence in Krakow and Krakow district area is similar to the incidence observed in most European countries. Cancer register was proved to be important tool in evaluation of GEPNET epidemiology.

• #38 Global burden of neuroendocrine tumors and changing incidence in Kentucky | Oncotarget

  https://www.oncotarget.com/article/24983/text/

  Neuroendocrine tumors (NETs) have a low incidence but relatively high prevalence. Over the last three decades, the incidence of NETs has risen 6-fold in the United States. […] KCR recorded 6179 individuals with newly diagnosed NETs between 1995 and 2015. Between 1995-2012, the incidence of NETs in KCR increased from 3.1 to 7.1 per 100,000 cases, while it increased from 3.96 to 6.61 in the SEER database. The incidence rates in both KCR and SEER databases were linear. […] NETs incidence between 1995 and 2015 show a linear increase in both KCR and SEER databases. […] Robust epidemiological surveillance of this population helps us recognize the quantum of disease burden and unique patterns of disease presentation, which are key in the use of effective screening, targeted diagnostics, and treatments.

• #39 Epidemiology, Incidence, and Prevalence of Neuroendocrine Neoplasms: Are There Global Differences?

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8118193/

  The magnitude of rise in GEP NET incidence rates appears to be most profound in North America. The reason for this phenomenon is undefined though health care utilization and underlying patient biology are anticipated to be contributing factors. […] Uniformly conducted population-based epidemiologic studies are needed in each country to assess the true healthcare burden of GEP NETs. Standardized data collection methods utilizing an up-to-date pathologic grading system (e.g., 2019 WHO NET grading criteria) and common data collection tools and analysis metrics (e.g., crude incidence rate, age-adjusted incidence rate) are necessary to draw meaningful conclusions.

• #40 The burden of diagnosed neuroendocrine tumors (NETs) is expected to increase at an annual growth rate (AGR) of 2.37% from an estimated 108,000 cases in 2020 to 133,000 in 2030 in the eight major markets (8MM*). This report reveals that the increase is at

  https://www.globaldata.com/store/report/neuroendocrine-tumors-epidemiology-analysis/

  The burden of diagnosed neuroendocrine tumors (NETs) is expected to increase at an annual growth rate (AGR) of 2.37% from an estimated 108,000 cases in 2020 to 133,000 in 2030 in the eight major markets (8MM*). […] An increase in the diagnosed incidence of NETs has been reported worldwide. The reason for this rise is not completely understood, but is most likely due to increased physician awareness, improvements in diagnostic imaging tests used and increased use of these tools. […] In the 8MM, GlobalData epidemiologists forecast that the diagnosed incident cases of NETs will increase from 107,776 cases in 2020 to 133,312 cases in 2030, at an Annual Growth Rate (AGR) of 2.37%. Urban China will have the highest number of NET cases among the 8MM throughout the forecast period, while Spain will have the lowest.

• #41 Gastroenteropancreatic Neuroendocrine Tumors Market

  https://www.delveinsight.com/blog/gastroenteropancreatic-neuroendocrine-tumours-market

  Gastroenteropancreatic Neuroendocrine Tumors are exclusively rare diseases, which might not affect a large population. As per Delveinsight analysis, in the 7MM countries [the United States, EU5 countries (Germany, France, Italy, Spain, and the United Kingdom), and Japan], the incident population of Gastroenteropancreatic Neuroendocrine Tumors was estimated to be almost 23k cases in 2020. […] Gastroenteropancreatic Neuroendocrine Tumors are exclusively rare diseases, which might not affect a large population. As per Delveinsight analysis, in the 7MM, the incident population of Gastroenteropancreatic Neuroendocrine Tumors was estimated to be almost 23K cases in 2020.

• #42 Neuroendocrine Tumors Market to Showcase Rapid Growth

  https://www.globenewswire.com/news-release/2024/11/25/2986962/0/en/Neuroendocrine-Tumors-Market-to-Showcase-Rapid-Growth-During-the-Study-Period-2020-2034-at-a-CAGR-of-5-1-DelveInsight.html

  The neuroendocrine tumors epidemiology section provides insights into the historical and current neuroendocrine tumors patient pool and forecasted trends for the 6MM. It helps recognize the causes of current and forecasted patient trends by exploring numerous studies and views of key opinion leaders. […] The neuroendocrine tumors market report proffers epidemiological analysis for the study period 20202034 in the 6MM segmented into: Total Incident Cases of NET, Cases of NETs by Grade, Stage-specific Cases of NET, Cases of NETs by Site, Cases of NETs by Functional Status. […] The total number of incident cases of NETs in the US was nearly 29,500 cases in 2023 and is projected to increase by 2034.

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