Materiały źródłowe

• #1 Carcinoid-syndrome: recent advances, current status and controversies

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5747542/

  Over the last few years there have been a number of advances in all aspects of carcinoid syndrome as well as new therapies. […] Recently, the frequency of carcinoid-syndrome in the US SEER database(20002011) was assessed in patients 65 yrs. of age. Of 9512 eligible NET patients,19% had carcinoid-syndrome and the proportion increased 72 % from 2000 to 2011(11% to 19%). This increase in carcinoid frequency mirrors the general increase in frequency of NETs in the SEER database, with a 6.4-fold age-adjusted increase from 1973 to 2012(1.09 to 6.98 per 100000), with the highest increases in the lung(1.49/100000), GI-tract(3.56/100000) and unknown primary sites(0.84/100000). […] In older studies, the carcinoid-syndrome was uncommon in patients with duodenal, rectal, CNS, gastric(type 1), colonic or appendiceal carcinoids(1%), whereas it was more than 8-fold more frequent in carcinoids of other areas of the small intestine, pulmonary tract, pancreas, Type 3 gastric carcinoids and Meckels diverticulum.

• #1 Carcinoid syndrome – Wikipedia

  https://en.wikipedia.org/wiki/Carcinoid_syndrome

  The incidence of neuroendocrine tumors in the US lies somewhere from 2.7 to 4.3 per 100,000 people and appears to be increasing over time. The incidence of the carcinoid syndrome is about 0.27 per 100,000 people in the US, about 10% of all people with neuroendocrine tumors. There does not appear to be any variance by gender however patients of African American ethnicity appear to be affected by the carcinoid syndrome more often.

• #1 Carcinoid Syndrome, Symptoms, Diagnosis, Treatment

  https://crinetics.com/carcinoid-syndrome-symptoms-diagnosis-treatment/

  Carcinoid syndrome is a group of symptoms some patients may present when they have a specific type of cancer called a neuroendocrine tumor, or NET. NETs are a rare, slow-growing type of cancer – they only represent about 1% of all cancers – and can occur in any number of places in the body, most often in the digestive tract. […] Not all neuroendocrine tumors cause carcinoid syndrome, but it commonly develops if the NET spreads from the small bowel (or other locations) to the liver. In these cases, the hormones the NET secretes cannot be filtered out by the liver as they normally would, so they get into the body’s circulatory system and cause the symptoms of carcinoid syndrome. […] About 33,000 patients. […] 5-year survival of 30-70% (grade 1 and 2 NETs). […] Most studies: 10% of NETs result in carcinoid syndrome. One major study reported up to 19%.

• #1 Carcinoid Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK448096/

  Carcinoid syndrome is caused by neuroendocrine tumors that metastasize to the liver, with the majority of these tumors originating from the midgut, particularly the appendix and small intestine. Neuroendocrine tumors without liver metastases typically do not produce carcinoid syndrome, as the liver metabolizes the biogenic amines produced by the primary tumor. Less commonly, tumors arising from the foregut or thorax can also result in carcinoid syndrome. […] […] Neuroendocrine tumors are relatively rare, but their incidence and prevalence have increased, likely due to improved diagnostic techniques and greater clinical awareness. The annual incidence of neuroendocrine tumors is estimated to be approximately 6.98 cases per 100,000 individuals, with a prevalence of approximately 35 cases per 100,000 individuals in the United States. These tumors can affect individuals of all racial and ethnic backgrounds, although some studies suggest a slightly higher prevalence among Caucasians. Neuroendocrine tumors are generally more common in females than males, depending on the primary tumor site. For instance, a female predominance is observed in pancreatic and gastrointestinal neuroendocrine tumors. However, the gender distribution can vary across anatomical sites and tumor types. The peak age for diagnosis typically falls between the fifth and seventh decades of life.

• #1 Carcinoid Syndrome Market: Analysis of Epidemiology, Pipeline Products, and Key Companies Working in the Market | Companies – Aquestive Therapeutics, Crinetics Pharmaceuticals – The Globe and Mail

  https://www.theglobeandmail.com/investing/markets/markets-news/GetNews/20849184/carcinoid-syndrome-market-analysis-of-epidemiology-pipeline-products-and-key-companies-working-in-the-market-companies-aquestive-therapeutics-crinetics-pharmaceuticals/

  NETs represent the second most common prevalent gastrointestinal cancers with a prevalence of 171,321 and an annual incidence rate of 6.98 per 100,000 as per the Surveillance, Epidemiology, and End Results (SEER) program of the National Cancer Institute. […] The frequency of CS in the US Surveillance, Epidemiology, and End Results (SEER) database (20002011) was reported to be 19% in patients 65 years, with an increasing incidence over the years mirroring the general increase in frequency of Neuroendocrine neoplasms (NENs). […] As per NHS, About 4,000 people are diagnosed with a neuroendocrine tumor each year in the UK, but not everyone with a tumor will have carcinoid syndrome. […] DelveInsight’s 'Carcinoid Syndrome Epidemiology Forecast to 2032′ report delivers an in-depth understanding of the disease, historical and forecasted Carcinoid Syndrome epidemiology in the 7MM, i.e., the United States, EU5 (Germany, Spain, Italy, France, and the United Kingdom), and Japan.

• #1 Carcinoid syndrome epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Carcinoid_syndrome_epidemiology_and_demographics

  The incidence of carcinoid syndrome is estimated to be 2 cases per 100,000 individuals worldwide. Carcinoid syndrome is a disease that tends to affect the elderly population. The median age at diagnosis is 60.9 years. Females are more commonly affected with carcinoid syndrome than males. Carcinoid syndrome usually affects individuals of the Caucasian race. African American, Latin American, and Asian individuals are less likely to develop carcinoid syndrome. […] Carcinoid tumors represent about 0.5% of all newly diagnosed malignancies. Carcinoid tumors account for 75% of all gastrointestinal endocrine tumors. Ovarian carcinoid tumors account for 0.3% of all ovarian tumors and 0.5% of carcinoid tumors. […] The annual incidence in the United States is approximately 3.56 per 100,000 population.

• #1 Malignant Carcinoid Syndrome: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/282515-overview

  The incidence of clinical carcinoid tumors is estimated to be 1.5-1.9 cases per 100,000 population; the actual frequency is almost certainly higher, because many carcinoid tumors never produce the related syndrome. It is estimated that about 8000 gastrointestinal (GI) tract-related carcinoid tumors are diagnosed each year in the United States. […] Epidemiologic studies have reported incidences of carcinoid tumors ranging from 0.79 to 1.88 per 100,000 population; a study from the Netherlands found an incidence of 1.95 per 100,000 population. […] A Swedish autopsy study reported an incidence of 8.4 cases per 100,000 population. […] Carcinoids occur most frequently in patients aged 50-70 years. Age at diagnosis ranges from 10-93 years (mean age 55 y). A population-based study of 1786 patients 65 years of age with carcinoid syndrome, identified from the Surveillance, Epidemiology, and End Results Medicare database, found that carcinoid syndrome was more common in women than in men and more common in Whites than in Blacks, Hispanics, or people of other races.

• #1 Carcinoid syndrome epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Carcinoid_syndrome_epidemiology_and_demographics

  Carcinoid syndrome is a disease that tends to affect the elderly population. […] The median age at diagnosis is 60.9 years. […] Females are more commonly affected with carcinoid syndrome than males. […] Carcinoid syndrome usually affects individuals of the Caucasian race. African American, Latin American, and Asian individuals are less likely to develop carcinoid syndrome.

• #1 Neuroendocrine Tumor (Carcinoid Syndrome) | 5-Minute Clinical Consult

  https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/1688569/all/Neuroendocrine_Tumor__Carcinoid_Syndrome_?q=Serotonin+Syndrome

  Carcinoid syndrome is more common in midgut intestinal tumors (up to 50%); less common with bronchial carcinoids. […] Incidence rates for gastroenteropancreatic (GEP) NETs are roughly 2.5 to 5.0 cases per 100,000. […] GEP-NET is the second most common digestive cancer. […] About 0.5% of all malignant diseases are neuroendocrine (carcinoid) tumors originating in the GI or bronchopulmonary systems. […] Incidence and prevalence have increased substantially over several decades, perhaps due to advances in imaging. […] True prevalence is difficult to determine because they are often asymptomatic. […] Estimated at 35/100,000 persons.

• #1 Gastrointestinal Neuroendocrine Tumors (PDQ®) – NCI

  https://www.cancer.gov/types/gi-neuroendocrine-tumors/hp/gi-neuroendocrine-treatment-pdq

  The age-adjusted incidence of neuroendocrine (carcinoid) tumors worldwide is approximately 2 per 100,000 people. The average age at diagnosis is 61.4 years. Neuroendocrine tumors (also called NETs) represent about 0.5% of all newly diagnosed malignancies. […] Carcinoid syndrome, which occurs in fewer than 20% of patients with neuroendocrine tumors, is caused by the release of metabolically undergraded vasoactive amines into the systemic circulation. It is associated with flushing, abdominal pain and diarrhea, bronchoconstriction, and carcinoid heart disease. […] Approximately 35% of neuroendocrine tumors of the small intestine are associated with carcinoid syndrome. The relatively common neuroendocrine tumors of the appendix and rectum rarely produce this syndrome, and neuroendocrine tumors from other sites have intermediate risks. Investigations using echocardiographic criteria for carcinoid heart disease found prevalences ranging from 35% to 77% among patients with carcinoid syndrome. […] The presence and severity of carcinoid heart disease, particularly tricuspid valve dysfunction, is associated with shortened survival.

• #1 Exploring Carcinoid Syndrome in Neuroendocrine Tumors: Insights from a Multidisciplinary Narrative Review

  https://www.mdpi.com/2072-6694/16/22/3831

  The intent is to provide clinicians with a broad and straightforward overview of the effective approach of CS, which requires a combination of biochemical, imaging, and therapeutic strategies to control both the tumor and the hormonal secretion. […] The diagnosis of CS results from a combination of appropriate clinical, biochemical, and radiological evaluations. […] Imaging studies (CT scan, MRI, and somatostatin receptors imaging, including 68Ga-DOTATATE PET, 68-DOTATOC PET, or Cu64-DOTATATE PET) have a key role in the whole assessment of the patients with NET associated with CS to determine the global tumor burden and liver metastasis, the somatostatin receptor status, and to evaluate tumor growth rate and eventual locoregional therapies. […] Carcinoid syndrome typically occurs when NENs, primarily from the small intestine, secrete serotonin and other vasoactive substances.

• #1 Carcinoid-syndrome: recent advances, current status and controversies

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5747542/

  In the recent study of the SEER database, similar data was obtained with relative frequency of small intestine(40%) respiratory system(13%) colon/rectum(10%) cecum/appendix(25%). The molecular basis for this marked heterogeneity in the occurrence of carcinoid-syndrome with carcinoids in different tissues is at present unknown. […] Recent studies demonstrate that assessment of plasma 5-HIAA can be used instead of the urinary 5-HIAA collection. […] It is recommended in most guidelines and expert opinion articles that CHD should be screened for using echocardiography. […] Several biomarkers have been proposed to identify and assess the severity of CHD. These include N-terminal pro B-type natriuretic peptide(NT-proBNP), CgA, TGF, FGF(fibroblast growth factor) and 5-HIAA. […] In the last few years there have been many advances in the management of NETs, including both nonfunctional NETs and NETs causing functional syndromes such as the carcinoid-syndrome, which is the most frequent. There have been advances in many aspects of the carcinoid-syndrome all of which are reviewed in this article, including an increased understanding of its epidemiology demonstrating its increasing frequency: new methods to establish its diagnosis: an increased understanding of its natural history and pathogenesis and important new approaches to its treatment.

• #1 Carcinoid heart disease | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/carcinoid-heart-disease-1?lang=us

  Cardiac lesions are present in approximately 50% of patients with carcinoid syndrome 1. […] Carcinoid heart disease, also known as Hedinger syndrome, is a known complication of neuroendocrine tumors (NETs) and is particularly prevalent in patients who develop carcinoid syndrome.

• #1 Complications from carcinoid syndrome: review of the current evidence – ecancer

  https://ecancer.org/en/journal/article/662-complications-from-carcinoid-syndrome-review-of-the-current-evidence

  In a large case series of 132 patients with CS, echocardiography assessment detected signs of CHD in 74 patients (56%). […] A more recent prospective study followed 252 patients with CS and detected a lower incidence of CHD (19.4%) after a median followup of 29 months. […] The European Neuroendocrine Tumour Society (ENETS) guideline recommends performing annual or more often if medically required, transthoracic echocardiography in patients with known or suspected CHD with a bubble study to exclude a patent foramen ovale. […] Physicians should screen all patients with CS or with asymptomatic 5-HIAA elevation for CHD through an echocardiographic study. […] Collaborative, multicentre prospective studies are key to estimate the incidence, prevalence, and clinical course of CHD, to evaluate whether more modern therapies such as everolimus and telotristat impact on CHD and extracardiac fibrosis progression.

• #1 How to Best Screen For and Manage Carcinoid Heart Disease

  https://www.acc.org/Latest-in-Cardiology/Articles/2023/11/27/12/19/How-to-Best-Screen-For-and-Manage-Carcinoid-Heart-Disease

  Screening for carcinoid heart disease (CHD) using N-terminal proB-type natriuretic peptide levels and echocardiography is recommended in all patients with metastatic carcinoid tumor. […] The primary symptoms of CS can include diarrhea, skin flushing, and bronchospasm. Most NET primaries are intestinal, with gastroenteropancreatic sites accounting for an incidence of 3.6 per 100,000, followed by lung NETs and then those located in the gonads or thymus, or of unknown origin. […] All patients with CS are recommended to have a detailed cardiac history and examination. If signs or symptoms of cardiac involvement are present, N-terminal proB-type natriuretic peptide (NT-proBNP) levels and transthoracic echocardiography (TTE) are recommended for further evaluation. […] The 2017 expert consensus document recommends ongoing follow-up with NT-proBNP levels if TTE findings are unremarkable, and with surveillance TTE every 6 months for mild CHD and every 3 months for moderate or greater CHD. […] Screening for cardiac disease is recommended for all patients with metastatic NET or CS by way of history, physical examination findings, NT-proBNP levels, and TTE findings.

• #1

  https://journals.lww.com/ajg/abstract/2019/10001/931_surveillance_of_duodenal_carcinoids_and_the.931.aspx

  The incidence of gastrointestinal neuroendocrine tumors (GI-NETs) is increasing in recent times reflecting the widespread use of endoscopy, and an increased awareness among clinicians. […] Despite the increase in incidence, the natural history of duodenal carcinoid tumors has not yet been well defined. […] This retrospective study showed that the main predictor of recurrence is the presence of positive tumor margins at initial resection. Patients with negative and margins had no recurrences in the 3 year follow up period, thus alleviating the need for long term surveillance in this patient population. Larger multicenter studies are required to determine the optimal surveillance strategy in patients undergoing endoscopic resection of duodenal carcinoid tumors.

• #1 Management of gastrointestinal carcinoid tumours-10 years experience at a district general hospital – Dronamraju – Journal of Gastrointestinal Oncology

  https://jgo.amegroups.org/article/view/271/html

  Over a period of 10 years there were 35 patients, with an estimated annual incidence of 2.5 per 100,000 population. […] Carcinoid syndrome is typically seen in patients with liver/lung metastases with an overall incidence of 10% in GICTs but 20% in those with jejuno-ileal disease. […] In the current study, after a median follow up of 24 months (range 2-96 months), 22 patients (63%) were alive and disease free and 4 patients (11%) were alive with disease. […] Patients with carcinoid tumours require multiple modalities of treatment and a dedicated multi-disciplinary team (MDT) to co-ordinate the management and follow up is essential. […] Currently there is still a lack of clear guidelines for referral and follow up of patients diagnosed incidentally with GICTs particularly within the setting of district general hospitals within the UK.

• #1 Gastrointestinal neuroendocrine (carcinoid) tumours: current diagnosis and management | The Medical Journal of Australia

  https://www.mja.com.au/journal/2010/193/1/gastrointestinal-neuroendocrine-carcinoid-tumours-current-diagnosis-and

  Neuroendocrine tumours (NETs) are increasing in both incidence and prevalence and, as a group, are more prevalent than either gastric, pancreatic, oesophageal or hepatobiliary adenocarcinomas, or any two of these cancers combined. […] The frequency of NETs in large autopsy series, before the era of increased detection by computed tomography (CT) and endoscopy, indicated that about 85% were undiagnosed during life. US data show that the incidence of gastrointestinal NETs has increased at a rate of 3%10% per year over the past three decades. This most likely reflects: increased detection greater use of abdominal CT and endoscopy, and advances in nuclear medicine and immunohistochemical pathology leading to improved diagnostic classification; increased awareness of the disease among physicians; and a true increase in tumour incidence.

• #1 Carcinoid Tumor: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/986050-overview

  The incidence of carcinoid tumors rose from 36 (5.7%) diagnosed in 2004 to 497 (27.7%) in 2013. Overall survival was significantly longer for carcinoid compared with functional and nonfunctional tumors, with 5-year survival rates of 63.1%, 58.3%, and 52.6%, respectively. […] Overall survival for patients having resection improved significantly for carcinoid tumors (89.2%) compared to functional and non-functional tumors (76.6%, and 78.7%, respectively). […] In 1980-1989, the overall age-standardized incidence rate for male and female populations in England were estimated to be 0.71 (0.68-0.75 and 0.87 (0.83-0.91), respectively. […] In Scotland, the respective rates were 1.17 (0.91-1.44) per 100,000 population and 1.36 (1.09-1.63) per 100,000 population. […] A study by Duess et al in Germany reported that appendiceal carcinoid tumors were found incidentally in 0.11% of children who underwent appendectomy (44 out of 40,499 patients).

• #1 Exploring Carcinoid Syndrome in Neuroendocrine Tumors: Insights from a Multidisciplinary Narrative Review

  https://www.mdpi.com/2072-6694/16/22/3831

  Carcinoid syndrome (CS) is a rare condition associated with neuroendocrine tumors (NETs), particularly those originating in the gastrointestinal tract, which secrete bioactive substances like serotonin. […] CS is predominantly described in about 20% of patients with well-differentiated small intestine NETs, representing 50% of all gastro-entero-pancreatic neuroendocrine tumors (GEP-NETs). […] It is often diagnosed in the fifth and sixth decades of life, with a slightly higher incidence among females than males. […] Patients with CS have an overall survival of 4.7 years, compared to 7.1 years in patients without symptoms of CS, and tumor burden is described as one of the most relevant factors affecting CS-related mortality. […] The complexity of CS necessitates individualized care and continuous coordination among specialists to optimize outcomes and enhance patient well-being.

• #1 Outcome of carcinoid heart syndrome in patients enrolled in the SwissNet cohort | BMC Cancer | Full Text

  https://bmccancer.biomedcentral.com/articles/10.1186/s12885-023-10739-z

  Carcinoid heart disease is a rare disease which develops in patients with functional neuroendocrine tumors in an advanced tumor state. Patients diagnosed with carcinoid heart disease have a poor longtime prognosis with respect to morbidity and mortality and long-term data on patient outcomes are lacking. […] We observed that early diagnosis with echocardiographic surveillance of carcinoid heart disease during the course of the neuroendocrine tumor disease was beneficial to overall survival of patients. […] Through nationwide patient enrollment, the SwissNet registry is a powerful data tool to identify, follow-up and evaluate long-term patient outcomes in patients with rare neuroendocrine tumor driven pathologies including carcinoid heart syndrome with observational methods enabling better therapy optimization to improve patient`s long-term perspectives and survival.

• #1 Outcome of carcinoid heart syndrome in patients enrolled in the SwissNet cohort | BMC Cancer | Full Text

  https://bmccancer.biomedcentral.com/articles/10.1186/s12885-023-10739-z

  Up to 70% of patients with a NET causing carcinoid syndrome will eventually develop CHD which is associated with a poor long-term prognosis with an estimated 3-year survival rate of 31% vs 68% seen in patients with a NET causing carcinoid syndrome without heart involvement. […] Therefore, it is recommended that patients with carcinoid syndrome with documented cardiac involvement receive a TTE screening every 6 months and those without cardiac pathologies once per year. […] Until today, limited data on patient outcomes with NET and CHD is available as most data are derived from small case series. […] This retrospective study of 23 patients with carcinoid heart disease enrolled into the SwissNet database showed that early diagnosis with echocardiographic surveillance of carcinoid heart disease during the course of the NET disease was beneficial to overall survival. […] Further, a thorough laboratory follow-up including markers like NT-proBNP, 5-HIAA and chromogranin A, evaluation of clinical symptoms including abdominal pain, diarrhea, flush, and tumor load is necessary to evaluate disease status and progression.

• #2 Gastrointestinal neuroendocrine (carcinoid) tumours: current diagnosis and management | The Medical Journal of Australia

  https://www.mja.com.au/journal/2010/193/1/gastrointestinal-neuroendocrine-carcinoid-tumours-current-diagnosis-and

  Neuroendocrine tumours (NETs) are increasing in both incidence and prevalence and, as a group, are more prevalent than either gastric, pancreatic, oesophageal or hepatobiliary adenocarcinomas, or any two of these cancers combined. […] The frequency of NETs in large autopsy series, before the era of increased detection by computed tomography (CT) and endoscopy, indicated that about 85% were undiagnosed during life. US data show that the incidence of gastrointestinal NETs has increased at a rate of 3%10% per year over the past three decades. This most likely reflects: increased detection greater use of abdominal CT and endoscopy, and advances in nuclear medicine and immunohistochemical pathology leading to improved diagnostic classification; increased awareness of the disease among physicians; and a true increase in tumour incidence.

• #2 Carcinoid syndrome epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Carcinoid_syndrome_epidemiology_and_demographics

  The incidence of carcinoid syndrome is estimated to be 2 cases per 100,000 individuals worldwide. Carcinoid syndrome is a disease that tends to affect the elderly population. The median age at diagnosis is 60.9 years. Females are more commonly affected with carcinoid syndrome than males. Carcinoid syndrome usually affects individuals of the Caucasian race. African American, Latin American, and Asian individuals are less likely to develop carcinoid syndrome. […] Carcinoid tumors represent about 0.5% of all newly diagnosed malignancies. Carcinoid tumors account for 75% of all gastrointestinal endocrine tumors. Ovarian carcinoid tumors account for 0.3% of all ovarian tumors and 0.5% of carcinoid tumors. […] The annual incidence in the United States is approximately 3.56 per 100,000 population.

• #2 Carcinoid Syndrome Market: Analysis of Epidemiology, Pipeline Products, and Key Companies Working in the Market | Companies – Aquestive Therapeutics, Crinetics Pharmaceuticals – The Globe and Mail

  https://www.theglobeandmail.com/investing/markets/markets-news/GetNews/20849184/carcinoid-syndrome-market-analysis-of-epidemiology-pipeline-products-and-key-companies-working-in-the-market-companies-aquestive-therapeutics-crinetics-pharmaceuticals/

  NETs represent the second most common prevalent gastrointestinal cancers with a prevalence of 171,321 and an annual incidence rate of 6.98 per 100,000 as per the Surveillance, Epidemiology, and End Results (SEER) program of the National Cancer Institute. […] The frequency of CS in the US Surveillance, Epidemiology, and End Results (SEER) database (20002011) was reported to be 19% in patients 65 years, with an increasing incidence over the years mirroring the general increase in frequency of Neuroendocrine neoplasms (NENs). […] As per NHS, About 4,000 people are diagnosed with a neuroendocrine tumor each year in the UK, but not everyone with a tumor will have carcinoid syndrome. […] DelveInsight’s 'Carcinoid Syndrome Epidemiology Forecast to 2032′ report delivers an in-depth understanding of the disease, historical and forecasted Carcinoid Syndrome epidemiology in the 7MM, i.e., the United States, EU5 (Germany, Spain, Italy, France, and the United Kingdom), and Japan.

• #2 Gastrointestinal Neuroendocrine Tumors (PDQ®) – NCI

  https://www.cancer.gov/types/gi-neuroendocrine-tumors/hp/gi-neuroendocrine-treatment-pdq

  The age-adjusted incidence of neuroendocrine (carcinoid) tumors worldwide is approximately 2 per 100,000 people. The average age at diagnosis is 61.4 years. Neuroendocrine tumors (also called NETs) represent about 0.5% of all newly diagnosed malignancies. […] Carcinoid syndrome, which occurs in fewer than 20% of patients with neuroendocrine tumors, is caused by the release of metabolically undergraded vasoactive amines into the systemic circulation. It is associated with flushing, abdominal pain and diarrhea, bronchoconstriction, and carcinoid heart disease. […] Approximately 35% of neuroendocrine tumors of the small intestine are associated with carcinoid syndrome. The relatively common neuroendocrine tumors of the appendix and rectum rarely produce this syndrome, and neuroendocrine tumors from other sites have intermediate risks. Investigations using echocardiographic criteria for carcinoid heart disease found prevalences ranging from 35% to 77% among patients with carcinoid syndrome. […] The presence and severity of carcinoid heart disease, particularly tricuspid valve dysfunction, is associated with shortened survival.

• #2 Carcinoid Syndrome | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/18867

  Neuroendocrine tumors are relatively rare, but their incidence and prevalence have increased, likely due to improved diagnostic techniques and greater clinical awareness. The annual incidence of neuroendocrine tumors is estimated to be approximately 6.98 cases per 100,000 individuals, with a prevalence of approximately 35 cases per 100,000 individuals in the United States. […] These tumors can affect individuals of all racial and ethnic backgrounds, although some studies suggest a slightly higher prevalence among Caucasians. Neuroendocrine tumors are generally more common in females than males, depending on the primary tumor site. For instance, a female predominance is observed in pancreatic and gastrointestinal neuroendocrine tumors. However, the gender distribution can vary across anatomical sites and tumor types. The peak age for diagnosis typically falls between the fifth and seventh decades of life.

• #2 Carcinoid syndrome epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Carcinoid_syndrome_epidemiology_and_demographics

  Carcinoid syndrome is a disease that tends to affect the elderly population. […] The median age at diagnosis is 60.9 years. […] Females are more commonly affected with carcinoid syndrome than males. […] Carcinoid syndrome usually affects individuals of the Caucasian race. African American, Latin American, and Asian individuals are less likely to develop carcinoid syndrome.

• #2 Carcinoid syndrome – Knowledge @ AMBOSS

  https://www.amboss.com/us/knowledge/carcinoid-syndrome/

  Carcinoid syndrome is primarily associated with metastatic well-differentiated NENs, i.e., neuroendocrine tumors (NETs), in the gastrointestinal tract. […] Incidence: 2.7 per 1,000,000 population per year. […] Highest in African-American individuals. […] Epidemiological data refers to the US, unless otherwise specified.

• #2 Outcome of carcinoid heart syndrome in patients enrolled in the SwissNet cohort | BMC Cancer | Full Text

  https://bmccancer.biomedcentral.com/articles/10.1186/s12885-023-10739-z

  Up to 70% of patients with a NET causing carcinoid syndrome will eventually develop CHD which is associated with a poor long-term prognosis with an estimated 3-year survival rate of 31% vs 68% seen in patients with a NET causing carcinoid syndrome without heart involvement. […] Therefore, it is recommended that patients with carcinoid syndrome with documented cardiac involvement receive a TTE screening every 6 months and those without cardiac pathologies once per year. […] Until today, limited data on patient outcomes with NET and CHD is available as most data are derived from small case series. […] This retrospective study of 23 patients with carcinoid heart disease enrolled into the SwissNet database showed that early diagnosis with echocardiographic surveillance of carcinoid heart disease during the course of the NET disease was beneficial to overall survival. […] Further, a thorough laboratory follow-up including markers like NT-proBNP, 5-HIAA and chromogranin A, evaluation of clinical symptoms including abdominal pain, diarrhea, flush, and tumor load is necessary to evaluate disease status and progression.

• #2 Carcinoid Heart Disease: Review of Current Knowledge in: Texas Heart Institute Journal Volume 46: Issue 1 | Texas Heart Institute Journal

  https://doi.org/10.14503/THIJ-17-6562

  Carcinoid tumors are neuroendocrine neoplasms that typically arise from the gastrointestinal tract and bronchopulmonary system. Approximately 2 in 100,000 people are affected annually. Carcinoid tumors, which are typically indolent, secrete myriad vasoactive substances, serotonin chief among them. Patients are often asymptomatic until these tumors metastasize to the liver, which cannot then inactivate the vasoactive substances. Rarely, ovarian and retroperitoneal metastasis occurs when the vasoactive substances bypass the liver and enter the systemic circulation through the caval system, a process that causes the classic symptoms of flushing and diarrhea. […] Carcinoid tumors involve the heart in up to 60% of patients. Cardiac involvement is associated with a poor long-term prognosis: the estimated 3-year survival rate of 31% is half that of patients without cardiac involvement.

• #2 How to Best Screen For and Manage Carcinoid Heart Disease

  https://www.acc.org/Latest-in-Cardiology/Articles/2023/11/27/12/19/How-to-Best-Screen-For-and-Manage-Carcinoid-Heart-Disease

  Screening for carcinoid heart disease (CHD) using N-terminal proB-type natriuretic peptide levels and echocardiography is recommended in all patients with metastatic carcinoid tumor. […] The primary symptoms of CS can include diarrhea, skin flushing, and bronchospasm. Most NET primaries are intestinal, with gastroenteropancreatic sites accounting for an incidence of 3.6 per 100,000, followed by lung NETs and then those located in the gonads or thymus, or of unknown origin. […] All patients with CS are recommended to have a detailed cardiac history and examination. If signs or symptoms of cardiac involvement are present, N-terminal proB-type natriuretic peptide (NT-proBNP) levels and transthoracic echocardiography (TTE) are recommended for further evaluation. […] The 2017 expert consensus document recommends ongoing follow-up with NT-proBNP levels if TTE findings are unremarkable, and with surveillance TTE every 6 months for mild CHD and every 3 months for moderate or greater CHD. […] Screening for cardiac disease is recommended for all patients with metastatic NET or CS by way of history, physical examination findings, NT-proBNP levels, and TTE findings.

• #2 Management of gastrointestinal carcinoid tumours-10 years experience at a district general hospital – Dronamraju – Journal of Gastrointestinal Oncology

  https://jgo.amegroups.org/article/view/271/568

  Over a period of 10 years there were 35 patients, with an estimated annual incidence of 2.5 per 100,000 population. […] The overall incidence of carcinoid tumours has been steadily increasing and they are considered to be more aggressive with a poorer prognosis than previously thought. […] Carcinoid syndrome is typically seen in patients with liver/lung metastases with an overall incidence of 10% in GICTs but 20% in those with jejuno-ileal disease. […] In the current study, after a median follow up of 24 months (range 2-96 months), 22 patients (63%) were alive and disease free and 4 patients (11%) were alive with disease. […] Currently there is still a lack of clear guidelines for referral and follow up of patients diagnosed incidentally with GICTs particularly within the setting of district general hospitals within the UK. […] The hope remains that better and/or modern treatment pathways for carcinoid tumours delivered in a regional setting would be reflected in a difference in survival.

• #2 Key Statistics About Gastrointestinal Carcinoid Tumors | American Cancer Society

  https://www.cancer.org/cancer/types/gastrointestinal-carcinoid-tumor/about/key-statistics.html

  Although the exact number isnt known, about 8,000 carcinoid tumors and cancers that start in the gastrointestinal tract (the stomach, intestine, appendix, colon, or rectum) are diagnosed each year in the United States. […] The number of carcinoid tumors diagnosed has been increasing for many years. […] The most common locations of gastrointestinal (GI) carcinoid tumors are the small intestine and the rectum. Other common sites include , the colon (large intestine), the appendix, and the stomach. […] The average age of people diagnosed with GI carcinoid tumors is early 60s. Carcinoid tumors are more common in African Americans than in White people, and are slightly more common in women than men.

• #2 Neuroendocrine Tumors – Diagnosis & Disease Information for HCPs

  https://www.cancertherapyadvisor.com/ddi/neuroendocrine-tumors/

  In the United States (US), the estimated annual incidence of neuroendocrine tumors is approximately 7 cases per 100,000 individuals. Overall, more than 170,000 people are living with neuroendocrine tumors and approximately 12,000 new cases are diagnosed each year. […] While neuroendocrine tumors are relatively rare, in recent decades, the incidence and prevalence have been increasing. This may be due in part to improved detection methods, heightened awareness among clinicians, and improved survival. […] Patients whose neuroendocrine tumor spreads to the liver or other parts of the body may experience carcinoid syndrome as a result of excessive hormones. Symptoms of carcinoid syndrome include the following: Flushing; Abdominal pain; Bloating; Diarrhea; Wheezing or other trouble breathing; and Tachycardia.

• #3

  https://step2.medbullets.com/oncology/120477/carcinoid-syndrome

  carcinoid syndrome is a rare syndrome that is caused by the metastasis of carcinoid tumors that secrete high levels of serotonin (5-HT) […] Incidence: rare, 1-2 cases per 100,000 individuals […] most frequently in patients 50-70 years.

• #3 Carcinoid Tumor

  https://mobile.fpnotebook.com/GI/HemeOnc/CrcndTmr.htm

  Prevalence: 1-2 per 100,000 […] Age at presentation: 50-60 years […] Carcinoid Syndrome (occurs in 10% of Carcinoid Tumors) […] Late finding for Carcinoid Tumor […] Metastases are usually present when occurs […] Associated with midgut tumors (appendix, ileum) […] Five year survival: 67% (variable based on type)

• #3 Exploring Carcinoid Syndrome in Neuroendocrine Tumors: Insights from a Multidisciplinary Narrative Review

  https://www.mdpi.com/2072-6694/16/22/3831

  Carcinoid syndrome (CS) is a rare condition associated with neuroendocrine tumors (NETs), particularly those originating in the gastrointestinal tract, which secrete bioactive substances like serotonin. […] CS is predominantly described in about 20% of patients with well-differentiated small intestine NETs, representing 50% of all gastro-entero-pancreatic neuroendocrine tumors (GEP-NETs). […] It is often diagnosed in the fifth and sixth decades of life, with a slightly higher incidence among females than males. […] Patients with CS have an overall survival of 4.7 years, compared to 7.1 years in patients without symptoms of CS, and tumor burden is described as one of the most relevant factors affecting CS-related mortality. […] The complexity of CS necessitates individualized care and continuous coordination among specialists to optimize outcomes and enhance patient well-being.

• #3 Carcinoid Tumors and Syndrome | Concise Medical Knowledge

  https://www.lecturio.com/concepts/carcinoid-tumors-and-syndrome/

  Carcinoid syndrome occurs in approximately 5% of carcinoid tumors. […] Rare, but rising incidence, likely due to improved detection. […] The reported annual incidence is 4.7/100,000. […] Black patients have a higher annual rate of incidence than whites. […] Presents in all age groups; greater incidence in the elderly (50-70 years). […] No clear gender predominance.

• #3 Carcinoid Heart Disease: Review of Current Knowledge in: Texas Heart Institute Journal Volume 46: Issue 1 | Texas Heart Institute Journal

  https://doi.org/10.14503/THIJ-17-6562

  The diagnosis of carcinoid HD depends upon strong clinical suspicion, biomarker levels, cardiac imaging results, and possibly the use of cardiac nuclear medicine. […] The European Neuroendocrine Tumor Society (ENTS) recommends TTE screening for patients diagnosed with carcinoid syndrome, annually for patients with no cardiac involvement and semiannually for those with it.

• #3 Carcinoid Tumor: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/986050-overview

  Carcinoids are the most common neuroendocrine tumors, with an estimated 1.5-1.9 clinical cases per 100,000 population. The incidence in autopsy cases is higher at 650 cases per 100,000 population. An estimated 8000 gastrointestinal (GI) tract-related carcinoid tumors are diagnosed each year in the United States. […] Evidence in adults suggests that overall incidence of carcinoid tumors has been steadily increasing. […] The exact incidence in children is not known. Most tumors occur in adults and are rare in children. […] Historically, prior to availability of improved diagnostic techniques, distant metastasis was reported in 12.9% range. […] A retrospective cohort study by Kasumova et al reported that out of 10,752 patients, 12.7% were diagnosed with carcinoid tumors, 84.7% with nonfunctional and 2.6% with functional pancreatic neuroendocrine tumors.

• #3 Carcinoid Heart Disease: Review of Current Knowledge in: Texas Heart Institute Journal Volume 46: Issue 1 | Texas Heart Institute Journal

  https://doi.org/10.14503/THIJ-17-6562

  Carcinoid tumors are neuroendocrine neoplasms that typically arise from the gastrointestinal tract and bronchopulmonary system. Approximately 2 in 100,000 people are affected annually. Carcinoid tumors, which are typically indolent, secrete myriad vasoactive substances, serotonin chief among them. Patients are often asymptomatic until these tumors metastasize to the liver, which cannot then inactivate the vasoactive substances. Rarely, ovarian and retroperitoneal metastasis occurs when the vasoactive substances bypass the liver and enter the systemic circulation through the caval system, a process that causes the classic symptoms of flushing and diarrhea. […] Carcinoid tumors involve the heart in up to 60% of patients. Cardiac involvement is associated with a poor long-term prognosis: the estimated 3-year survival rate of 31% is half that of patients without cardiac involvement.

• #4 Carcinoid syndrome | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/carcinoid-syndrome?lang=us

  Carcinoid syndrome occurs equally between the sexes, most commonly in the 40-70 year age group. […] The presence of the carcinoid syndrome often indicates hepatic metastatic disease, however, extraintestinal neuroendocrine tumor can also cause carcinoid syndrome without hepatic metastasis as serotonin secretion can bypass the enterohepatic circulation.

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