Materiały źródłowe

• #1 Neuro-Endocrine/Endocrine system: Carcinoid tumors

  https://atlasgeneticsoncology.org/solid-tumor/5523/neuro-endocrine-endocrine-system-carcinoid-tumors

  Carcinoid tumors are the most common type of neuroendocrine (NE) tumor and comprise approximately half of all NE tumors of the gastrointestinal (GI) tract. […] The incidence of carcinoid tumors is estimated at 1 to 2 cases per 100,000 people per annum. Because carcinoids often have an indolent clinical course, they are often misdiagnosed, and their true incidence is likely higher. A Swedish study, in which the incidence of carcinoid tumors was evaluated in surgical specimens and autopsies, estimated the true incidence of carcinoids to be 8.4 cases per 100,000. […] Lung and bronchial carcinoids make up about 28% of carcinoids, and 2% of all primary lung tumors. […] Gastric carcinoid tumors account for less than 1% of all gastric cancers. They comprise 5% of all GI carcinoids. […] Small intestinal carcinoid tumors account for approximately one third of all small bowel tumors. They comprise 29% of GI carcinoids.

• #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. […] 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. […] The most common site of primary NETs is the gastrointestinal tract (about 60% of all cases), followed by the bronchopulmonary tree (27%).

• #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. […] While not entirely clear, it is speculated that this increase is due to more universal utilization of proton pump inhibitors. 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. 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).

• #4 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.

• #5 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. […] While not entirely clear, it is speculated that this increase is due to more universal utilization of proton pump inhibitors. 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. 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).

• #6 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. […] Tumors that are smaller than 1 cm in diameter rarely metastasize, while lesions larger than 2 cm often metastasize. […] The presence of a few small metastases to the liver is associated with a longer life expectancy.

• #7 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.

• #8 Updated Population-Based Review of Carcinoid Tumors

  https://pmc.ncbi.nlm.nih.gov/articles/PMC1356383/

  An updated, population-based evaluation of carcinoid tumors was performed using a nationwide database (n = 11,427). Incidence rates have increased for many carcinoid tumors, and the small intestine is the most common site for these tumors to develop in the gastrointestinal tract. […] The overall incidence of carcinoid tumors was 38.4 per one million individuals in the year 1997 (age-adjusted), as determined from the population-based data. […] The incidence of carcinoids found in the rectum, stomach, and small intestine increased significantly over the time period (P 0.05), while the incidences of tumors in the appendix and colon have remained constant (P = NS). […] The overall cancer-specific 5-year survival for all sites was 69.7% (relative survival, 56.2%). […] Survival rates for carcinoid tumors varied significantly depending on their site of origin and tumor stage.

• #9 Carcinoid Syndrome – StatPearls – NCBI Bookshelf

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

  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. […] 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.

• #10 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. […] While not entirely clear, it is speculated that this increase is due to more universal utilization of proton pump inhibitors. 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. 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).

• #11 Neuro-Endocrine/Endocrine system: Carcinoid tumors

  https://atlasgeneticsoncology.org/solid-tumor/5523/neuro-endocrine-endocrine-system-carcinoid-tumors

  Carcinoid tumors are the most common type of neuroendocrine (NE) tumor and comprise approximately half of all NE tumors of the gastrointestinal (GI) tract. […] The incidence of carcinoid tumors is estimated at 1 to 2 cases per 100,000 people per annum. Because carcinoids often have an indolent clinical course, they are often misdiagnosed, and their true incidence is likely higher. A Swedish study, in which the incidence of carcinoid tumors was evaluated in surgical specimens and autopsies, estimated the true incidence of carcinoids to be 8.4 cases per 100,000. […] Lung and bronchial carcinoids make up about 28% of carcinoids, and 2% of all primary lung tumors. […] Gastric carcinoid tumors account for less than 1% of all gastric cancers. They comprise 5% of all GI carcinoids. […] Small intestinal carcinoid tumors account for approximately one third of all small bowel tumors. They comprise 29% of GI carcinoids.

• #12 Neuro-Endocrine/Endocrine system: Carcinoid tumors

  https://atlasgeneticsoncology.org/solid-tumor/5523/neuro-endocrine-endocrine-system-carcinoid-tumors

  Carcinoid tumors are the most common type of neuroendocrine (NE) tumor and comprise approximately half of all NE tumors of the gastrointestinal (GI) tract. […] The incidence of carcinoid tumors is estimated at 1 to 2 cases per 100,000 people per annum. Because carcinoids often have an indolent clinical course, they are often misdiagnosed, and their true incidence is likely higher. A Swedish study, in which the incidence of carcinoid tumors was evaluated in surgical specimens and autopsies, estimated the true incidence of carcinoids to be 8.4 cases per 100,000. […] Lung and bronchial carcinoids make up about 28% of carcinoids, and 2% of all primary lung tumors. […] Gastric carcinoid tumors account for less than 1% of all gastric cancers. They comprise 5% of all GI carcinoids. […] Small intestinal carcinoid tumors account for approximately one third of all small bowel tumors. They comprise 29% of GI carcinoids.

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

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

  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).

• #14 The epidemiology of carcinoid tumours in England and Scotland | British Journal of Cancer

  https://www.nature.com/articles/bjc1994424

  Relatively little is known about the epidemiology of carcinoid tumours in contrast to the extensive information available on their biochemical effects and natural history. Accordingly, we have used cancer registrations in England from 1979 to 1987, and in Scotland from 1980 to 1989, to estimate the incidence of carcinoid tumours in Britain. Age-standardised incidence rates for England, based on 3,382 registrations, were 0.71 (0.68-0.75) for men and 0.87 (0.83-0.91) for women, per 100,000 per year. The equivalent rates for Scotland, based on 639 registrations, were 1.17 (0.91-1.44) for men and 1.36 (1.09-1.63) for women. There was a consistent female excess of carcinoid tumours in the reproductive years, which was reversed after the age of 50. The female excess was most striking for gastrointestinal carcinoid tumours in women aged 15-19 years (F:M ratio = 2.14). The sex differences are probably due in part to incidental diagnosis of carcinoid tumours during abdominal procedures, which are more common in women than men at ages 15-49 years. However, there is some evidence to suggest a true sex difference in incidence, particularly the fact that the sex ratio for thoracic tumours varies with age in a similar way to that for gastrointestinal tumours. Hormonal factors may, therefore, be important in the aetiology of carcinoid tumours.

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

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

  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).

• #16 The epidemiology of carcinoid tumours in England and Scotland | British Journal of Cancer

  https://www.nature.com/articles/bjc1994424

  Relatively little is known about the epidemiology of carcinoid tumours in contrast to the extensive information available on their biochemical effects and natural history. Accordingly, we have used cancer registrations in England from 1979 to 1987, and in Scotland from 1980 to 1989, to estimate the incidence of carcinoid tumours in Britain. Age-standardised incidence rates for England, based on 3,382 registrations, were 0.71 (0.68-0.75) for men and 0.87 (0.83-0.91) for women, per 100,000 per year. The equivalent rates for Scotland, based on 639 registrations, were 1.17 (0.91-1.44) for men and 1.36 (1.09-1.63) for women. There was a consistent female excess of carcinoid tumours in the reproductive years, which was reversed after the age of 50. The female excess was most striking for gastrointestinal carcinoid tumours in women aged 15-19 years (F:M ratio = 2.14). The sex differences are probably due in part to incidental diagnosis of carcinoid tumours during abdominal procedures, which are more common in women than men at ages 15-49 years. However, there is some evidence to suggest a true sex difference in incidence, particularly the fact that the sex ratio for thoracic tumours varies with age in a similar way to that for gastrointestinal tumours. Hormonal factors may, therefore, be important in the aetiology of carcinoid tumours.

• #17

  https://link.springer.com/article/10.1023/A:1007334217762

  Between 1985 and 1991, 83 carcinoid tumours were diagnosed in the province of Florence where the Tuscany Tumour Registry is active. There were 44 males and 39 females. The age-adjusted incidence was 0.65 cases/100,000 population/year. The most common location was the lung (30.1%), followed by colorectum (25.2%) and small intestine (22.8%). The 5-year observed and relative survival rates were 70.8% and 78.9% respectively. The relative risk of developing multiple primary cancers was 0.79 (CI 95% 0.288.33).

• #18 Epidemiology of carcinoid neoplasms in Vaud, Switzerland, 1974–97 | British Journal of Cancer

  https://www.nature.com/articles/6691394

  In Vaud, Switzerland, the incidence of carcinoids based on 218 malignant and 215 benign cases rose from 19.6/106 in 1974-85 to 28.2/106 in 1986-97, more so among males and malignant neoplasms. […] Lung was the commonest site for malignant and large intestine for benign carcinoids. […] Sixty-eight (16%) carcinoids had another neoplasm.

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

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

  Morbidity is related to vasoactive amine production. […] The survival rate usually correlates inversely with the levels of daily urinary 5-HIAA excretion. […] The incidence of metastasis is estimated at 1-2 cases per 100,000 affected people. […] Carcinoids occur most frequently in patients aged 50-70 years. […] 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.

• #20

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

  Epidemiology […] Incidence […] rare, 1-2 cases per 100,000 individuals […] […] […] Demographics […] most frequently in patients 50-70 years

• #21 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.

• #22 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.

• #23 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. […] While not entirely clear, it is speculated that this increase is due to more universal utilization of proton pump inhibitors. 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. 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).

• #24 Bronchial carcinoid tumor | Radiology Reference Article | Radiopaedia.org

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

  Typically affects patients from the 3rd to 7th decades with the mean age around 45 years 7,10. […] multiple endocrine neoplasia type 1 13 […] Cushing syndrome: due to ACTH-producing carcinoid tumor types 1.

• #25 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.

• #26 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.

• #27 The epidemiology of carcinoid tumours in England and Scotland | British Journal of Cancer

  https://www.nature.com/articles/bjc1994424

  Relatively little is known about the epidemiology of carcinoid tumours in contrast to the extensive information available on their biochemical effects and natural history. Accordingly, we have used cancer registrations in England from 1979 to 1987, and in Scotland from 1980 to 1989, to estimate the incidence of carcinoid tumours in Britain. Age-standardised incidence rates for England, based on 3,382 registrations, were 0.71 (0.68-0.75) for men and 0.87 (0.83-0.91) for women, per 100,000 per year. The equivalent rates for Scotland, based on 639 registrations, were 1.17 (0.91-1.44) for men and 1.36 (1.09-1.63) for women. There was a consistent female excess of carcinoid tumours in the reproductive years, which was reversed after the age of 50. The female excess was most striking for gastrointestinal carcinoid tumours in women aged 15-19 years (F:M ratio = 2.14). The sex differences are probably due in part to incidental diagnosis of carcinoid tumours during abdominal procedures, which are more common in women than men at ages 15-49 years. However, there is some evidence to suggest a true sex difference in incidence, particularly the fact that the sex ratio for thoracic tumours varies with age in a similar way to that for gastrointestinal tumours. Hormonal factors may, therefore, be important in the aetiology of carcinoid tumours.

• #28 The epidemiology of carcinoid tumours in England and Scotland | British Journal of Cancer

  https://www.nature.com/articles/bjc1994424

  Relatively little is known about the epidemiology of carcinoid tumours in contrast to the extensive information available on their biochemical effects and natural history. Accordingly, we have used cancer registrations in England from 1979 to 1987, and in Scotland from 1980 to 1989, to estimate the incidence of carcinoid tumours in Britain. Age-standardised incidence rates for England, based on 3,382 registrations, were 0.71 (0.68-0.75) for men and 0.87 (0.83-0.91) for women, per 100,000 per year. The equivalent rates for Scotland, based on 639 registrations, were 1.17 (0.91-1.44) for men and 1.36 (1.09-1.63) for women. There was a consistent female excess of carcinoid tumours in the reproductive years, which was reversed after the age of 50. The female excess was most striking for gastrointestinal carcinoid tumours in women aged 15-19 years (F:M ratio = 2.14). The sex differences are probably due in part to incidental diagnosis of carcinoid tumours during abdominal procedures, which are more common in women than men at ages 15-49 years. However, there is some evidence to suggest a true sex difference in incidence, particularly the fact that the sex ratio for thoracic tumours varies with age in a similar way to that for gastrointestinal tumours. Hormonal factors may, therefore, be important in the aetiology of carcinoid tumours.

• #29 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.

• #30 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.

• #31 Carcinoid Tumors and Syndrome | Concise Medical Knowledge

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

  Carcinoid tumors are most commonly found in the GI and bronchopulmonary tracts. […] 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). […] Carcinoid syndrome occurs in approximately 5% of carcinoid tumors. […] Rare, but rising incidence, likely due to improved detection.

• #32 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.

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

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

  Morbidity is related to vasoactive amine production. […] The survival rate usually correlates inversely with the levels of daily urinary 5-HIAA excretion. […] The incidence of metastasis is estimated at 1-2 cases per 100,000 affected people. […] Carcinoids occur most frequently in patients aged 50-70 years. […] 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.

• #34 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. […] 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. […] The most common site of primary NETs is the gastrointestinal tract (about 60% of all cases), followed by the bronchopulmonary tree (27%).

• #35 Updated Population-Based Review of Carcinoid Tumors

  https://pmc.ncbi.nlm.nih.gov/articles/PMC1356383/

  To determine the population-based incidence, anatomic distribution, and survival rates of gastrointestinal carcinoid tumors. […] The overall incidence rates for carcinoid tumors have increased significantly over the past 25 years, although rates for some sites have decreased (eg, appendix). […] The gastrointestinal tract accounted for 54.5% of the tumors. […] The 5-year survival rates for the most common gastrointestinal sites were stomach (75.1%), small intestine (76.1%), appendix (76.3%), and rectum (87.5%). […] Using national, population-based cancer registry data, this study demonstrates that (1) incidence rates for carcinoid tumors have changed, (2) the most common gastrointestinal site is not the appendix (as is often quoted), but the small intestine, followed in frequency by the rectum, and (3) survival rates differ between individual anatomic sites.

• #36 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. […] 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. […] The most common site of primary NETs is the gastrointestinal tract (about 60% of all cases), followed by the bronchopulmonary tree (27%).

• #37 Updated Population-Based Review of Carcinoid Tumors

  https://pmc.ncbi.nlm.nih.gov/articles/PMC1356383/

  To determine the population-based incidence, anatomic distribution, and survival rates of gastrointestinal carcinoid tumors. […] The overall incidence rates for carcinoid tumors have increased significantly over the past 25 years, although rates for some sites have decreased (eg, appendix). […] The gastrointestinal tract accounted for 54.5% of the tumors. […] The 5-year survival rates for the most common gastrointestinal sites were stomach (75.1%), small intestine (76.1%), appendix (76.3%), and rectum (87.5%). […] Using national, population-based cancer registry data, this study demonstrates that (1) incidence rates for carcinoid tumors have changed, (2) the most common gastrointestinal site is not the appendix (as is often quoted), but the small intestine, followed in frequency by the rectum, and (3) survival rates differ between individual anatomic sites.

• #38 Updated Population-Based Review of Carcinoid Tumors

  https://pmc.ncbi.nlm.nih.gov/articles/PMC1356383/

  Local stage tumors have relatively high survival rates, regardless of site of origin. […] Survival rates are substantially lower for those with regional disease, particularly for stomach, rectal, and small intestine tumors. […] The SEER database allows for a longitudinal examination of population-based cancer data, but it has limitations. […] In summary, our work shows that the distribution of carcinoid tumors appears to have changed significantly over the 25-year study period. The incidence rate for appendiceal carcinoids has remained stable, while the rates for small intestine, rectum, and stomach have increased. The relative distribution of carcinoids in these organs has changed. The appendix may no longer be the most common site for gastrointestinal carcinoids to develop; the small intestine and rectum appear to be the more frequent sites.

• #39 Neuro-Endocrine/Endocrine system: Carcinoid tumors

  https://atlasgeneticsoncology.org/solid-tumor/5523/neuro-endocrine-endocrine-system-carcinoid-tumors

  Carcinoid tumors are the most common type of neuroendocrine (NE) tumor and comprise approximately half of all NE tumors of the gastrointestinal (GI) tract. […] The incidence of carcinoid tumors is estimated at 1 to 2 cases per 100,000 people per annum. Because carcinoids often have an indolent clinical course, they are often misdiagnosed, and their true incidence is likely higher. A Swedish study, in which the incidence of carcinoid tumors was evaluated in surgical specimens and autopsies, estimated the true incidence of carcinoids to be 8.4 cases per 100,000. […] Lung and bronchial carcinoids make up about 28% of carcinoids, and 2% of all primary lung tumors. […] Gastric carcinoid tumors account for less than 1% of all gastric cancers. They comprise 5% of all GI carcinoids. […] Small intestinal carcinoid tumors account for approximately one third of all small bowel tumors. They comprise 29% of GI carcinoids.

• #40 Neuro-Endocrine/Endocrine system: Carcinoid tumors

  https://atlasgeneticsoncology.org/solid-tumor/5523/neuro-endocrine-endocrine-system-carcinoid-tumors

  Carcinoid tumors are the most common tumor of the appendix, accounting for more than half of all appendiceal malignancies and discovered in seven of every 1,000 appendectomy specimens. They account for 5% of GI carcinoids and are more common in women than men. […] Rectal carcinoids comprise 1-2% of all rectal cancers, and 14% of all GI carcinoids.

• #41 Neuro-Endocrine/Endocrine system: Carcinoid tumors

  https://atlasgeneticsoncology.org/solid-tumor/5523/neuro-endocrine-endocrine-system-carcinoid-tumors

  Carcinoid tumors are the most common tumor of the appendix, accounting for more than half of all appendiceal malignancies and discovered in seven of every 1,000 appendectomy specimens. They account for 5% of GI carcinoids and are more common in women than men. […] Rectal carcinoids comprise 1-2% of all rectal cancers, and 14% of all GI carcinoids.

• #42 Neuro-Endocrine/Endocrine system: Carcinoid tumors

  https://atlasgeneticsoncology.org/solid-tumor/5523/neuro-endocrine-endocrine-system-carcinoid-tumors

  Carcinoid tumors are the most common type of neuroendocrine (NE) tumor and comprise approximately half of all NE tumors of the gastrointestinal (GI) tract. […] The incidence of carcinoid tumors is estimated at 1 to 2 cases per 100,000 people per annum. Because carcinoids often have an indolent clinical course, they are often misdiagnosed, and their true incidence is likely higher. A Swedish study, in which the incidence of carcinoid tumors was evaluated in surgical specimens and autopsies, estimated the true incidence of carcinoids to be 8.4 cases per 100,000. […] Lung and bronchial carcinoids make up about 28% of carcinoids, and 2% of all primary lung tumors. […] Gastric carcinoid tumors account for less than 1% of all gastric cancers. They comprise 5% of all GI carcinoids. […] Small intestinal carcinoid tumors account for approximately one third of all small bowel tumors. They comprise 29% of GI carcinoids.

• #43 Updated Population-Based Review of Carcinoid Tumors

  https://pmc.ncbi.nlm.nih.gov/articles/PMC1356383/

  An updated, population-based evaluation of carcinoid tumors was performed using a nationwide database (n = 11,427). Incidence rates have increased for many carcinoid tumors, and the small intestine is the most common site for these tumors to develop in the gastrointestinal tract. […] The overall incidence of carcinoid tumors was 38.4 per one million individuals in the year 1997 (age-adjusted), as determined from the population-based data. […] The incidence of carcinoids found in the rectum, stomach, and small intestine increased significantly over the time period (P 0.05), while the incidences of tumors in the appendix and colon have remained constant (P = NS). […] The overall cancer-specific 5-year survival for all sites was 69.7% (relative survival, 56.2%). […] Survival rates for carcinoid tumors varied significantly depending on their site of origin and tumor stage.

• #44

  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. […] Duodenal carcinoids are rare and accounting for 1%-3% of primary duodenal tumors. […] Despite the increase in incidence, the natural history of duodenal carcinoid tumors has not yet been well defined. […] Endoscopic resection appears to be a safe and effective treatment for duodenal carcinoid tumors measuring 10 mm in diameter and confined to the submucosal layer. […] This retrospective study showed that the main predictor of recurrence is the presence of positive tumor margins at initial resection. […] Patients with negative 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.

• #45 Neuro-Endocrine/Endocrine system: Carcinoid tumors

  https://atlasgeneticsoncology.org/solid-tumor/5523/neuro-endocrine-endocrine-system-carcinoid-tumors

  Carcinoid tumors are the most common type of neuroendocrine (NE) tumor and comprise approximately half of all NE tumors of the gastrointestinal (GI) tract. […] The incidence of carcinoid tumors is estimated at 1 to 2 cases per 100,000 people per annum. Because carcinoids often have an indolent clinical course, they are often misdiagnosed, and their true incidence is likely higher. A Swedish study, in which the incidence of carcinoid tumors was evaluated in surgical specimens and autopsies, estimated the true incidence of carcinoids to be 8.4 cases per 100,000. […] Lung and bronchial carcinoids make up about 28% of carcinoids, and 2% of all primary lung tumors. […] Gastric carcinoid tumors account for less than 1% of all gastric cancers. They comprise 5% of all GI carcinoids. […] Small intestinal carcinoid tumors account for approximately one third of all small bowel tumors. They comprise 29% of GI carcinoids.

• #46 Lung neuroendocrine (carcinoid) tumors: Epidemiology, risk factors, classification, histology, diagnosis, and staging – UpToDate

  https://www.uptodate.com/contents/lung-neuroendocrine-carcinoid-tumors-epidemiology-risk-factors-classification-histology-diagnosis-and-staging

  Lung neuroendocrine tumors (NETs) account for approximately 1 to 2 percent of all lung malignancies in adults and roughly 20 to 30 percent of all NETs. […] Globally, incidence rates range from 0.2 to 2 per 100,000 population per year, and most series suggest a higher incidence in females as compared with males and in White as compared with Black populations.

• #47 Updated Population-Based Review of Carcinoid Tumors

  https://pmc.ncbi.nlm.nih.gov/articles/PMC1356383/

  To determine the population-based incidence, anatomic distribution, and survival rates of gastrointestinal carcinoid tumors. […] The overall incidence rates for carcinoid tumors have increased significantly over the past 25 years, although rates for some sites have decreased (eg, appendix). […] The gastrointestinal tract accounted for 54.5% of the tumors. […] The 5-year survival rates for the most common gastrointestinal sites were stomach (75.1%), small intestine (76.1%), appendix (76.3%), and rectum (87.5%). […] Using national, population-based cancer registry data, this study demonstrates that (1) incidence rates for carcinoid tumors have changed, (2) the most common gastrointestinal site is not the appendix (as is often quoted), but the small intestine, followed in frequency by the rectum, and (3) survival rates differ between individual anatomic sites.

• #48 Updated Population-Based Review of Carcinoid Tumors

  https://pmc.ncbi.nlm.nih.gov/articles/PMC1356383/

  An updated, population-based evaluation of carcinoid tumors was performed using a nationwide database (n = 11,427). Incidence rates have increased for many carcinoid tumors, and the small intestine is the most common site for these tumors to develop in the gastrointestinal tract. […] The overall incidence of carcinoid tumors was 38.4 per one million individuals in the year 1997 (age-adjusted), as determined from the population-based data. […] The incidence of carcinoids found in the rectum, stomach, and small intestine increased significantly over the time period (P 0.05), while the incidences of tumors in the appendix and colon have remained constant (P = NS). […] The overall cancer-specific 5-year survival for all sites was 69.7% (relative survival, 56.2%). […] Survival rates for carcinoid tumors varied significantly depending on their site of origin and tumor stage.

• #49 Updated Population-Based Review of Carcinoid Tumors

  https://pmc.ncbi.nlm.nih.gov/articles/PMC1356383/

  To determine the population-based incidence, anatomic distribution, and survival rates of gastrointestinal carcinoid tumors. […] The overall incidence rates for carcinoid tumors have increased significantly over the past 25 years, although rates for some sites have decreased (eg, appendix). […] The gastrointestinal tract accounted for 54.5% of the tumors. […] The 5-year survival rates for the most common gastrointestinal sites were stomach (75.1%), small intestine (76.1%), appendix (76.3%), and rectum (87.5%). […] Using national, population-based cancer registry data, this study demonstrates that (1) incidence rates for carcinoid tumors have changed, (2) the most common gastrointestinal site is not the appendix (as is often quoted), but the small intestine, followed in frequency by the rectum, and (3) survival rates differ between individual anatomic sites.

• #50 Updated Population-Based Review of Carcinoid Tumors

  https://pmc.ncbi.nlm.nih.gov/articles/PMC1356383/

  An updated, population-based evaluation of carcinoid tumors was performed using a nationwide database (n = 11,427). Incidence rates have increased for many carcinoid tumors, and the small intestine is the most common site for these tumors to develop in the gastrointestinal tract. […] The overall incidence of carcinoid tumors was 38.4 per one million individuals in the year 1997 (age-adjusted), as determined from the population-based data. […] The incidence of carcinoids found in the rectum, stomach, and small intestine increased significantly over the time period (P 0.05), while the incidences of tumors in the appendix and colon have remained constant (P = NS). […] The overall cancer-specific 5-year survival for all sites was 69.7% (relative survival, 56.2%). […] Survival rates for carcinoid tumors varied significantly depending on their site of origin and tumor stage.

• #51 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. […] While not entirely clear, it is speculated that this increase is due to more universal utilization of proton pump inhibitors. 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. 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).

• #52 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. […] 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. […] The most common site of primary NETs is the gastrointestinal tract (about 60% of all cases), followed by the bronchopulmonary tree (27%).

• #53 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.

• #54

  https://journals.lww.com/md-journal/fulltext/2018/01120/subcutaneous_metastasis_of_a_pulmonary_carcinoid.9.aspx

  Carcinoid tumors are derived from neuroendocrine cells and are most frequently found in the gastrointestinal tract and bronchopulmonary system. They are generally characterized by an indolent clinical course but may in some instances spread to regional lymph nodes or to distant sites. […] Pulmonary carcinoids (PCs) are rare tumors with an age-adjusted incidence rate ranging from 0.2 to 2/100,000 population/year in both United States and European countries and comprise approximately 2% of all primary lung cancers. […] Despite the indolent nature of carcinoid tumors, up to 26% of patients with atypical carcinoids may still experience recurrences. […] Surveillance for recurrences after surgical resection remains an important component of cancer care with a significant impact on survival, quality of life, and health care costs.

• #55

  https://journals.lww.com/md-journal/fulltext/2018/01120/subcutaneous_metastasis_of_a_pulmonary_carcinoid.9.aspx

  Carcinoid tumors are derived from neuroendocrine cells and are most frequently found in the gastrointestinal tract and bronchopulmonary system. They are generally characterized by an indolent clinical course but may in some instances spread to regional lymph nodes or to distant sites. […] Pulmonary carcinoids (PCs) are rare tumors with an age-adjusted incidence rate ranging from 0.2 to 2/100,000 population/year in both United States and European countries and comprise approximately 2% of all primary lung cancers. […] Despite the indolent nature of carcinoid tumors, up to 26% of patients with atypical carcinoids may still experience recurrences. […] Surveillance for recurrences after surgical resection remains an important component of cancer care with a significant impact on survival, quality of life, and health care costs.

• #56

  https://journals.lww.com/md-journal/fulltext/2018/01120/subcutaneous_metastasis_of_a_pulmonary_carcinoid.9.aspx

  Recently, European Neuroendocrine Tumour Society (ENTS) guidelines on best practice for PC tumors recommended for ACs a close monitoring with CT imaging carried out 3 months post-surgery, then 6 monthly for 5 years and after 5 years yearly. […] However, if recurrences are rare, the utility of repeated imaging for such an extended period could be questionable. […] This case highlights the value of surveillance after atypical carcinoid tumor surgery and the usefulness of PET/CT and immunohistochemistry to make a diagnosis.

• #57 Consensus Statements and Guidelines – Carcinoid Cancer Foundation

  https://www.carcinoid.org/for-doctors/other-resources/consensus-statements-and-guidelines/

  The publication of the first North American consensus guidelines for NET disease management was a major achievement for the carcinoid/NET community in 2010. These guidelines include early detection procedures for diagnostic purposes, imaging, histopathology, biochemical evaluation, surgical procedures, and evidence-based treatments emphasizing a multidisciplinary team-based approach to patient care. The NANETS Guidelines Working Group consists of 38 carcinoid/NET specialists writing guidelines in the areas of Epidemiology and Genetics, Pathology, Radionuclide Therapies, Biochemistry, Symptomatic Control, Surgery, Imaging and Liver-Directed Therapies, Systemic Treatments, and Follow-up. […] The incidence of neuroendocrine tumours of the gastroenteropancreatic system seems to have increased during the past decade. New diagnostic and therapeutic procedures have aroused the interest of physicians, though most see very few cases of such diseases. A group of members of the Nordic Neuroendocrine Tumour Group decided to compile some guidelines to facilitate the diagnosis and treatment of patients with these tumours. Part I of these guidelines discusses the principles of histopathology, biochemical and radiological diagnosis as well as therapeutic options. Part II-specific NE tumour types contains a description of epidemiology, histopathology, clinical presentation, diagnostic procedure, treatment, and survival for each type of neuroendocrine tumour. We have, however, tried to give more updated information about the epidemiology and histopathology, which is essential for the clinical management of these tumours.

• #58 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

  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.

• #59 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.

• #60 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

  Patients with well-differentiated neuroendocrine tumours may develop carcinoid syndrome (CS), which is characterised by flushing, abdominal cramps, diarrhoea, and bronchospasms. […] Between 2000 and 2004, well-differentiated neuroendocrine tumours (NET) occurred in 5 per 100,000 people per year in the US according to the Surveillance, Epidemiology, and End Results (SEER) 17. […] One of the most common NET is the well-differentiated midgut NET presenting with carcinoid syndrome (CS). […] The aim of this review is to critically describe the current state of the art in respect to epidemiology, pathogenesis, clinical features, diagnosis, and treatment options for complications associated with CS resulting from neuroendocrine tumours. […] In a large case series of 132 patients with CS, echocardiography assessment detected signs of CHD in 74 patients (56%).

• #61 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

  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.

• #62 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

  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.

• #63

  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. […] Duodenal carcinoids are rare and accounting for 1%-3% of primary duodenal tumors. […] Despite the increase in incidence, the natural history of duodenal carcinoid tumors has not yet been well defined. […] Endoscopic resection appears to be a safe and effective treatment for duodenal carcinoid tumors measuring 10 mm in diameter and confined to the submucosal layer. […] This retrospective study showed that the main predictor of recurrence is the presence of positive tumor margins at initial resection. […] Patients with negative 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.

• #64

  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. […] Duodenal carcinoids are rare and accounting for 1%-3% of primary duodenal tumors. […] Despite the increase in incidence, the natural history of duodenal carcinoid tumors has not yet been well defined. […] Endoscopic resection appears to be a safe and effective treatment for duodenal carcinoid tumors measuring 10 mm in diameter and confined to the submucosal layer. […] This retrospective study showed that the main predictor of recurrence is the presence of positive tumor margins at initial resection. […] Patients with negative 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.

• #65

  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. […] Duodenal carcinoids are rare and accounting for 1%-3% of primary duodenal tumors. […] Despite the increase in incidence, the natural history of duodenal carcinoid tumors has not yet been well defined. […] Endoscopic resection appears to be a safe and effective treatment for duodenal carcinoid tumors measuring 10 mm in diameter and confined to the submucosal layer. […] This retrospective study showed that the main predictor of recurrence is the presence of positive tumor margins at initial resection. […] Patients with negative 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.

• #66

  https://journals.lww.com/md-journal/fulltext/2018/01120/subcutaneous_metastasis_of_a_pulmonary_carcinoid.9.aspx

  Recently, European Neuroendocrine Tumour Society (ENTS) guidelines on best practice for PC tumors recommended for ACs a close monitoring with CT imaging carried out 3 months post-surgery, then 6 monthly for 5 years and after 5 years yearly. […] However, if recurrences are rare, the utility of repeated imaging for such an extended period could be questionable. […] This case highlights the value of surveillance after atypical carcinoid tumor surgery and the usefulness of PET/CT and immunohistochemistry to make a diagnosis.

• #67 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. […] Until recently there were no clear guidelines regarding diagnosis, referral, management and follow up of patients with GICTs. […] Referral to regional neuroendocrine multidisciplinary team (NET-MDT) services started in 2006 and of the 18 patients who were diagnosed with GICTs post 2006, 10 (56%) patients were referred to this service. […] Prognosis of patients with GICTs is largely determined by age, race and sex of patients, site and size of the primary lesion, stage of the disease, histologic grade and extent of the disease.

• #68 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

  The incidence of nodal and distant metastasis is rare if the primary tumour size is less than 1 cm, however this increases significantly once the primary lesion is over 2 cm in size. […] 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. Hence, there is a need for more NET-MDTs nationwide in order to provide a co-ordinated approach in the management of this rare condition.

• #69 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

  As a result of the substantial (~ 60%) 5-year survival, the prevalence of NETs (about 35 per 100 000) is considerably higher than the incidence and, as a group, NETs are more prevalent than either gastric, pancreatic, oesophageal or hepatobiliary adenocarcinomas, or any two of these cancers combined. […] National and international efforts are now underway to develop practice guidelines for diagnosing and treating this heterogeneous disease, and to establish multidisciplinary management groups. In Australia, the number of gastrointestinal NETs treated on a per capita basis is far lower than the predicted incidence (15002000 new cases per year United States National Cancer Institute, Surveillance Epidemiology and End Results database), suggesting significant under-recognition of a disease entity whose predicted prevalence is 50007000 cases.

• #70 Updated Population-Based Review of Carcinoid Tumors

  https://pmc.ncbi.nlm.nih.gov/articles/PMC1356383/

  An updated, population-based evaluation of carcinoid tumors was performed using a nationwide database (n = 11,427). Incidence rates have increased for many carcinoid tumors, and the small intestine is the most common site for these tumors to develop in the gastrointestinal tract. […] The overall incidence of carcinoid tumors was 38.4 per one million individuals in the year 1997 (age-adjusted), as determined from the population-based data. […] The incidence of carcinoids found in the rectum, stomach, and small intestine increased significantly over the time period (P 0.05), while the incidences of tumors in the appendix and colon have remained constant (P = NS). […] The overall cancer-specific 5-year survival for all sites was 69.7% (relative survival, 56.2%). […] Survival rates for carcinoid tumors varied significantly depending on their site of origin and tumor stage.

• #71 Updated Population-Based Review of Carcinoid Tumors

  https://pmc.ncbi.nlm.nih.gov/articles/PMC1356383/

  An updated, population-based evaluation of carcinoid tumors was performed using a nationwide database (n = 11,427). Incidence rates have increased for many carcinoid tumors, and the small intestine is the most common site for these tumors to develop in the gastrointestinal tract. […] The overall incidence of carcinoid tumors was 38.4 per one million individuals in the year 1997 (age-adjusted), as determined from the population-based data. […] The incidence of carcinoids found in the rectum, stomach, and small intestine increased significantly over the time period (P 0.05), while the incidences of tumors in the appendix and colon have remained constant (P = NS). […] The overall cancer-specific 5-year survival for all sites was 69.7% (relative survival, 56.2%). […] Survival rates for carcinoid tumors varied significantly depending on their site of origin and tumor stage.

• #72 Updated Population-Based Review of Carcinoid Tumors

  https://pmc.ncbi.nlm.nih.gov/articles/PMC1356383/

  To determine the population-based incidence, anatomic distribution, and survival rates of gastrointestinal carcinoid tumors. […] The overall incidence rates for carcinoid tumors have increased significantly over the past 25 years, although rates for some sites have decreased (eg, appendix). […] The gastrointestinal tract accounted for 54.5% of the tumors. […] The 5-year survival rates for the most common gastrointestinal sites were stomach (75.1%), small intestine (76.1%), appendix (76.3%), and rectum (87.5%). […] Using national, population-based cancer registry data, this study demonstrates that (1) incidence rates for carcinoid tumors have changed, (2) the most common gastrointestinal site is not the appendix (as is often quoted), but the small intestine, followed in frequency by the rectum, and (3) survival rates differ between individual anatomic sites.

• #73 Updated Population-Based Review of Carcinoid Tumors

  https://pmc.ncbi.nlm.nih.gov/articles/PMC1356383/

  To determine the population-based incidence, anatomic distribution, and survival rates of gastrointestinal carcinoid tumors. […] The overall incidence rates for carcinoid tumors have increased significantly over the past 25 years, although rates for some sites have decreased (eg, appendix). […] The gastrointestinal tract accounted for 54.5% of the tumors. […] The 5-year survival rates for the most common gastrointestinal sites were stomach (75.1%), small intestine (76.1%), appendix (76.3%), and rectum (87.5%). […] Using national, population-based cancer registry data, this study demonstrates that (1) incidence rates for carcinoid tumors have changed, (2) the most common gastrointestinal site is not the appendix (as is often quoted), but the small intestine, followed in frequency by the rectum, and (3) survival rates differ between individual anatomic sites.

• #74 Updated Population-Based Review of Carcinoid Tumors

  https://pmc.ncbi.nlm.nih.gov/articles/PMC1356383/

  To determine the population-based incidence, anatomic distribution, and survival rates of gastrointestinal carcinoid tumors. […] The overall incidence rates for carcinoid tumors have increased significantly over the past 25 years, although rates for some sites have decreased (eg, appendix). […] The gastrointestinal tract accounted for 54.5% of the tumors. […] The 5-year survival rates for the most common gastrointestinal sites were stomach (75.1%), small intestine (76.1%), appendix (76.3%), and rectum (87.5%). […] Using national, population-based cancer registry data, this study demonstrates that (1) incidence rates for carcinoid tumors have changed, (2) the most common gastrointestinal site is not the appendix (as is often quoted), but the small intestine, followed in frequency by the rectum, and (3) survival rates differ between individual anatomic sites.

• #75 Updated Population-Based Review of Carcinoid Tumors

  https://pmc.ncbi.nlm.nih.gov/articles/PMC1356383/

  To determine the population-based incidence, anatomic distribution, and survival rates of gastrointestinal carcinoid tumors. […] The overall incidence rates for carcinoid tumors have increased significantly over the past 25 years, although rates for some sites have decreased (eg, appendix). […] The gastrointestinal tract accounted for 54.5% of the tumors. […] The 5-year survival rates for the most common gastrointestinal sites were stomach (75.1%), small intestine (76.1%), appendix (76.3%), and rectum (87.5%). […] Using national, population-based cancer registry data, this study demonstrates that (1) incidence rates for carcinoid tumors have changed, (2) the most common gastrointestinal site is not the appendix (as is often quoted), but the small intestine, followed in frequency by the rectum, and (3) survival rates differ between individual anatomic sites.

• #76 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. […] While not entirely clear, it is speculated that this increase is due to more universal utilization of proton pump inhibitors. 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. 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).

• #77 Updated Population-Based Review of Carcinoid Tumors

  https://pmc.ncbi.nlm.nih.gov/articles/PMC1356383/

  Local stage tumors have relatively high survival rates, regardless of site of origin. […] Survival rates are substantially lower for those with regional disease, particularly for stomach, rectal, and small intestine tumors. […] The SEER database allows for a longitudinal examination of population-based cancer data, but it has limitations. […] In summary, our work shows that the distribution of carcinoid tumors appears to have changed significantly over the 25-year study period. The incidence rate for appendiceal carcinoids has remained stable, while the rates for small intestine, rectum, and stomach have increased. The relative distribution of carcinoids in these organs has changed. The appendix may no longer be the most common site for gastrointestinal carcinoids to develop; the small intestine and rectum appear to be the more frequent sites.

• #78 Updated Population-Based Review of Carcinoid Tumors

  https://pmc.ncbi.nlm.nih.gov/articles/PMC1356383/

  Local stage tumors have relatively high survival rates, regardless of site of origin. […] Survival rates are substantially lower for those with regional disease, particularly for stomach, rectal, and small intestine tumors. […] The SEER database allows for a longitudinal examination of population-based cancer data, but it has limitations. […] In summary, our work shows that the distribution of carcinoid tumors appears to have changed significantly over the 25-year study period. The incidence rate for appendiceal carcinoids has remained stable, while the rates for small intestine, rectum, and stomach have increased. The relative distribution of carcinoids in these organs has changed. The appendix may no longer be the most common site for gastrointestinal carcinoids to develop; the small intestine and rectum appear to be the more frequent sites.

• #79 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

  The incidence of nodal and distant metastasis is rare if the primary tumour size is less than 1 cm, however this increases significantly once the primary lesion is over 2 cm in size. […] 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. Hence, there is a need for more NET-MDTs nationwide in order to provide a co-ordinated approach in the management of this rare condition.

• #80 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. […] Tumors that are smaller than 1 cm in diameter rarely metastasize, while lesions larger than 2 cm often metastasize. […] The presence of a few small metastases to the liver is associated with a longer life expectancy.

• #81 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. […] Tumors that are smaller than 1 cm in diameter rarely metastasize, while lesions larger than 2 cm often metastasize. […] The presence of a few small metastases to the liver is associated with a longer life expectancy.

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

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

  Morbidity is related to vasoactive amine production. […] The survival rate usually correlates inversely with the levels of daily urinary 5-HIAA excretion. […] The incidence of metastasis is estimated at 1-2 cases per 100,000 affected people. […] Carcinoids occur most frequently in patients aged 50-70 years. […] 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.

• #83 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. […] While not entirely clear, it is speculated that this increase is due to more universal utilization of proton pump inhibitors. 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. 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).

• #84 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. […] Until recently there were no clear guidelines regarding diagnosis, referral, management and follow up of patients with GICTs. […] Referral to regional neuroendocrine multidisciplinary team (NET-MDT) services started in 2006 and of the 18 patients who were diagnosed with GICTs post 2006, 10 (56%) patients were referred to this service. […] Prognosis of patients with GICTs is largely determined by age, race and sex of patients, site and size of the primary lesion, stage of the disease, histologic grade and extent of the disease.

• #85 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

  Prognosis of patients with GICTs is largely determined by age, race and sex of patients, site and size of the primary lesion, stage of the disease, histologic grade and extent of the disease. […] The current study is a small series of abdominal carcinoid tumours treated at a single institution but it does represent a modest experience of midgut carcinoids. […] 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.

• #86 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.

• #87 Prognostic nomogram for predicting long-term cancer-specific survival in patients with lung carcinoid tumors | BMC Cancer | Full Text

  https://bmccancer.biomedcentral.com/articles/10.1186/s12885-021-07832-6

  Lung carcinoid is a rare malignant tumor with poor survival. The current study established a nomogram model for predicting cancer-specific survival (CSS) in patients with lung carcinoid tumors. A total of 1956 patients diagnosed with primary lung carcinoid tumors were extracted from the Surveillance, Epidemiology, and End Results database. Age at diagnosis, grade, histological type, N stage, M stage, surgery of the primary site, radiation of the primary site, and tumor size were independent prognostic factors of CSS. The incidence of lung carcinoid tumor has increased sharply in the past three decades, partly due to the broad application of cross-sectional imaging and bronchoscopy in population screening. The tumor-node-metastasis (TNM) classification system has been utilized for tumor staging in lung carcinoids since 2010, which is also now recommended by the North American Neuroendocrine Tumor Society and European Neuroendocrine Tumor Society. However, TNM staging is not a perfect predictor of lung carcinoid tumor prognosis, because it does not take into account the effect of tumor size, metastasis sites/patterns, pathological, genetic, and therapeutic factors, which are significantly associated with prognosis. Detection of patients under high risk with homogenous prognosis especially at an early stage could be helpful to improve clinical practice and decision-making. The established nomogram model exhibited high discriminative accuracy and good concordance in the prediction of 3-, 5- and 10-year CSS. This nomogram may help clinicians with prognostic evaluations and with the development of individualized therapy for this aggressive disease.

• #88 Prognostic nomogram for predicting long-term cancer-specific survival in patients with lung carcinoid tumors | BMC Cancer | Full Text

  https://bmccancer.biomedcentral.com/articles/10.1186/s12885-021-07832-6

  Lung carcinoid is a rare malignant tumor with poor survival. The current study established a nomogram model for predicting cancer-specific survival (CSS) in patients with lung carcinoid tumors. A total of 1956 patients diagnosed with primary lung carcinoid tumors were extracted from the Surveillance, Epidemiology, and End Results database. Age at diagnosis, grade, histological type, N stage, M stage, surgery of the primary site, radiation of the primary site, and tumor size were independent prognostic factors of CSS. The incidence of lung carcinoid tumor has increased sharply in the past three decades, partly due to the broad application of cross-sectional imaging and bronchoscopy in population screening. The tumor-node-metastasis (TNM) classification system has been utilized for tumor staging in lung carcinoids since 2010, which is also now recommended by the North American Neuroendocrine Tumor Society and European Neuroendocrine Tumor Society. However, TNM staging is not a perfect predictor of lung carcinoid tumor prognosis, because it does not take into account the effect of tumor size, metastasis sites/patterns, pathological, genetic, and therapeutic factors, which are significantly associated with prognosis. Detection of patients under high risk with homogenous prognosis especially at an early stage could be helpful to improve clinical practice and decision-making. The established nomogram model exhibited high discriminative accuracy and good concordance in the prediction of 3-, 5- and 10-year CSS. This nomogram may help clinicians with prognostic evaluations and with the development of individualized therapy for this aggressive disease.

• #89 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

  As a result of the substantial (~ 60%) 5-year survival, the prevalence of NETs (about 35 per 100 000) is considerably higher than the incidence and, as a group, NETs are more prevalent than either gastric, pancreatic, oesophageal or hepatobiliary adenocarcinomas, or any two of these cancers combined. […] National and international efforts are now underway to develop practice guidelines for diagnosing and treating this heterogeneous disease, and to establish multidisciplinary management groups. In Australia, the number of gastrointestinal NETs treated on a per capita basis is far lower than the predicted incidence (15002000 new cases per year United States National Cancer Institute, Surveillance Epidemiology and End Results database), suggesting significant under-recognition of a disease entity whose predicted prevalence is 50007000 cases.

• #90 Carcinoid Syndrome – StatPearls – NCBI Bookshelf

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

  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. […] 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.

• #91 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.

• #92 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

  As a result of the substantial (~ 60%) 5-year survival, the prevalence of NETs (about 35 per 100 000) is considerably higher than the incidence and, as a group, NETs are more prevalent than either gastric, pancreatic, oesophageal or hepatobiliary adenocarcinomas, or any two of these cancers combined. […] National and international efforts are now underway to develop practice guidelines for diagnosing and treating this heterogeneous disease, and to establish multidisciplinary management groups. In Australia, the number of gastrointestinal NETs treated on a per capita basis is far lower than the predicted incidence (15002000 new cases per year United States National Cancer Institute, Surveillance Epidemiology and End Results database), suggesting significant under-recognition of a disease entity whose predicted prevalence is 50007000 cases.

• #93 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

  As a result of the substantial (~ 60%) 5-year survival, the prevalence of NETs (about 35 per 100 000) is considerably higher than the incidence and, as a group, NETs are more prevalent than either gastric, pancreatic, oesophageal or hepatobiliary adenocarcinomas, or any two of these cancers combined. […] National and international efforts are now underway to develop practice guidelines for diagnosing and treating this heterogeneous disease, and to establish multidisciplinary management groups. In Australia, the number of gastrointestinal NETs treated on a per capita basis is far lower than the predicted incidence (15002000 new cases per year United States National Cancer Institute, Surveillance Epidemiology and End Results database), suggesting significant under-recognition of a disease entity whose predicted prevalence is 50007000 cases.

• #94 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. […] 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. […] Until recently there were no clear guidelines regarding diagnosis, referral, management and follow up of patients with GICTs. […] Management of patients with carcinoid tumours in district hospitals needs streamlining with increased utilisation of regional neuroendocrine multidisciplinary teams. […] GICTs account for approximately 75% of all neuro-endocrine tumours and according to a recent large population based survey there has been a significant increase in the annual age adjusted incidence rates of carcinoids tumours.

• #95 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

  The incidence of nodal and distant metastasis is rare if the primary tumour size is less than 1 cm, however this increases significantly once the primary lesion is over 2 cm in size. […] 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. Hence, there is a need for more NET-MDTs nationwide in order to provide a co-ordinated approach in the management of this rare condition.

• #96 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

  As a result of the substantial (~ 60%) 5-year survival, the prevalence of NETs (about 35 per 100 000) is considerably higher than the incidence and, as a group, NETs are more prevalent than either gastric, pancreatic, oesophageal or hepatobiliary adenocarcinomas, or any two of these cancers combined. […] National and international efforts are now underway to develop practice guidelines for diagnosing and treating this heterogeneous disease, and to establish multidisciplinary management groups. In Australia, the number of gastrointestinal NETs treated on a per capita basis is far lower than the predicted incidence (15002000 new cases per year United States National Cancer Institute, Surveillance Epidemiology and End Results database), suggesting significant under-recognition of a disease entity whose predicted prevalence is 50007000 cases.

• #97 Carcinoid Tumors | CancerIndex

  http://www.cancerindex.org/clinks6d.htm

  Carcinoid tumors are rare, with incidence estimated at less than 3 per 100,000 people. The most common sites of disease are the gastrointestinal tract or lung, but they can occur in many parts of the body. […] Carcinoid tumours arise in cells of the diffuse neuroendocrine system and can develop in a number of anatomical sites including the lungs and the gastrointestinal tract. There has been a move away from the use of the term carcinoid tumour to the more appropriate use of neuroendocrine tumour (NET) to highlight the potential for invasion and metastasis associated with some NETs. […] Close surveillance of at-risk individuals is important to detect NETs early when curative surgery can be offered and the morbidity and mortality of metastatic NETs can be avoided. […] Nonexcisional management of small, localized rectal carcinoids is becoming more common and may offer equivalent survival to excision or resection. […] This survey provides the largest international overview of the current practice in the management of bronchial carcinoids and identifies discrepancies that could be the focus of future investigations.

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