Materiały źródłowe

• #1 Carcinoid Syndrome – NETRF

  https://netrf.org/old-for-patients/living-with-nets/carcinoid-syndrome/

  Carcinoid syndrome can develop when a neuroendocrine tumor produces sufficient quantities of substances that cause the symptoms associated with the condition. NETs occur in about 7 out of every 100,000 people. Among those living with NETs, researchers estimate 19% develop carcinoid syndrome. NET tumors occur in both men and women, occurring slightly more frequently in women, and the typical age (median age) of a person at NET diagnosis is between 55 and 60. NETs arise most frequently (approximately 70% of the time) in the gastrointestinal tract, followed by occurrences in the respiratory tract (approximately 25% of the time), and more rarely arise in many other sites. Regardless of where they start (so-called primary site), NETs can subsequently spread extensively to places such as liver, bone, and other sites. Carcinoid syndrome is most commonly caused by NETs that occur in the midgut and spread (metastasize) to the liver; however, in rare instances, carcinoid syndrome may be caused by NETs originating in the foregut and hindgut.

• #2 Carcinoid Syndrome: An Overview, Symptoms, Causes & Treatment

  https://www.ganeshdiagnostic.com/blog/carcinoid-syndrome-an-overview-symptoms-causes-and-treatment

  The most frequent cause of carcinoid syndrome is liver-metastasising neuroendocrine tumours of the midgut. Rarely, foregut and hind-gut neuroendocrine tumours can also lead to carcinoid syndrome. […] About 70% of neuroendocrine tumours develop in the GI system, with the respiratory tract accounting for the remaining 25%. Rarely, neuroendocrine tumours can develop in other organs like the kidneys, sperm, or ovaries. […] Biologically active amines and peptides that reach the systemic circulation and avoid the liver’s first-pass metabolism are the basis for the pathophysiology of the carcinoid syndrome. […] Less commonly, conditions like primary gut tumour with extensive retroperitoneal nodal metastases, ovarian tumour, or bronchial carcinoid, which release bioactive amines straight into the systemic circulation, can cause carcinoid syndrome without liver metastasis.

• #3 Carcinoid Syndrome – StatPearls – NCBI Bookshelf

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

  Carcinoid syndrome is caused by neuroendocrine tumors that metastasize to the liver, with the majority of these tumors originating from the midgut, particularly the appendix and small intestine. Neuroendocrine tumors without liver metastases typically do not produce carcinoid syndrome, as the liver metabolizes the biogenic amines produced by the primary tumor. Less commonly, tumors arising from the foregut or thorax can also result in carcinoid syndrome.[3][5] […] Carcinoid syndrome arises from the release of biologically active amines and peptides into the systemic circulation, bypassing the liver’s first-pass metabolism. The liver normally inactivates these substances, but liver metastases from neuroendocrine tumors can allow these products to enter the circulation and exert systemic effects.[8][9]

• #4 Carcinoid Syndrome Diarrhea

  https://www.uspharmacist.com/article/carcinoid-syndrome-diarrhea

  Diarrhea is often the revealing symptom in patients suffering from carcinoid syndrome secondary to a carcinoid tumor. The etiology of this burdensome diarrhea is thought to be hypersecretion of serotonin from neuroendocrine cells. […] Carcinoid tumors are NETs that primarily arise from enterochromaffin cells located throughout the gastrointestinal (GI) and bronchial tracts. These cells are responsible for the production, storage, and secretion of many biologically active substances including serotonin, norepinephrine, dopamine, histamine, bradykinin, prostaglandins, substance P, and others that may lead to the development of carcinoid syndrome, a collective term encompassing a variety of symptoms mediated by the hormones secreted by the carcinoid tumor. […] The pathophysiology of carcinoid syndrome diarrhea involves the presence of excess serotonin, which stimulates secretion of colonic mucus, increases peristalsis, and inhibits absorption in the GI tract all of which lead to diarrhea.

• #5 Clinical features of carcinoid syndrome – UpToDate

  https://www.uptodate.com/contents/clinical-features-of-carcinoid-syndrome

  „Carcinoid syndrome” is the term applied to a constellation of symptoms mediated by various humoral factors elaborated by some well-differentiated neuroendocrine tumors (NETs) of the digestive tract and lungs, which synthesize, store, and release a variety of polypeptides, biogenic amines, and prostaglandins (table 1). Some of these tumor products are responsible for carcinoid syndrome, but the relative contributions of each and the specificity of any for particular components of the syndrome are uncertain (table 2). […] Carcinoid syndrome is most common in the setting of disseminated disease, particularly liver metastases. […] The liver inactivates bioactive products secreted into the portal circulation. This may explain why patients with gastrointestinal NETs most often develop carcinoid syndrome if they have hepatic metastases, resulting in the secretion of tumor products into the systemic circulation.

• #6 Carcinoid syndrome: Symptoms, causes, treatments, and more

  https://www.medicalnewstoday.com/articles/carcinoid-syndrome

  Carcinoid syndrome affects certain people who have neuroendocrine tumors (NETs). Carcinoid syndrome can occur when an NET secretes hormones into the bloodstream. This can cause symptoms such as facial flushing and diarrhea. […] The American Cancer Society notes that around 10% of NETs cause carcinoid syndrome. Medical professionals previously referred to NETs as carcinoid tumors. […] Carcinoid syndrome occurs when NETs release a high enough level of hormones to cause symptoms. […] The liver usually inactivates hormones that the body does not use. However, carcinoid syndrome can occur if the hormones directly enter the systemic circulation or if something affects the liver’s function. […] Carcinoid syndrome is most likely to occur as a result of NETs that develop in the midgut. The midgut includes the appendix, small intestine, cecum, and ascending colon. […] Additionally, certain NETs, such as those that develop in the ovaries and lungs, may release hormones directly into the bloodstream without spreading to the liver. […] Carcinoid syndrome develops as a result of the NET releasing high levels of hormones.

• #7 Gastrointestinal Neuroendocrine Tumors and Carcinoid Syndrome – Endocrine and Metabolic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/endocrine-and-metabolic-disorders/gastrointestinal-and-pancreatic-neuroendocrine-tumors-nets/gastrointestinal-neuroendocrine-tumors-and-carcinoid-syndrome

  Carcinoid syndrome is usually due to endocrinologically active NETs that develop from neuroendocrine cells (mostly in the ileum) and produce serotonin. […] It can, however, occur as a result of neuroendocrine tumors elsewhere in the gastrointestinal tract (particularly the appendix and rectum), pancreas, bronchi, or, rarely, the gonads. Rarely, certain highly malignant tumors (eg, oat cell carcinoma of the lung, pancreatic islet cell carcinoma, medullary thyroid carcinoma) are responsible. […] An intestinal serotonin-producing NET does not usually cause carcinoid syndrome unless hepatic metastases have occurred because metabolic products released by the tumor are rapidly destroyed by blood and liver enzymes in the portal circulation (eg, serotonin by hepatic monoamine oxidase). […] Hepatic metastases, however, release metabolic products via the hepatic veins directly into the systemic circulation. […] Metabolic products released by primary pulmonary and ovarian carcinoids bypass the portal route and may similarly induce symptoms. […] Rare intestinal NETs with only intra-abdominal spread can drain directly into the systemic circulation or the lymphatics and cause symptoms.

• #8 Carcinoid Syndrome: An Overview, Symptoms, Causes & Treatment

  https://www.ganeshdiagnostic.com/blog/carcinoid-syndrome-an-overview-symptoms-causes-and-treatment

  The most frequent cause of carcinoid syndrome is liver-metastasising neuroendocrine tumours of the midgut. Rarely, foregut and hind-gut neuroendocrine tumours can also lead to carcinoid syndrome. […] About 70% of neuroendocrine tumours develop in the GI system, with the respiratory tract accounting for the remaining 25%. Rarely, neuroendocrine tumours can develop in other organs like the kidneys, sperm, or ovaries. […] Biologically active amines and peptides that reach the systemic circulation and avoid the liver’s first-pass metabolism are the basis for the pathophysiology of the carcinoid syndrome. […] Less commonly, conditions like primary gut tumour with extensive retroperitoneal nodal metastases, ovarian tumour, or bronchial carcinoid, which release bioactive amines straight into the systemic circulation, can cause carcinoid syndrome without liver metastasis.

• #9 Carcinoid Syndrome – NETRF

  https://netrf.org/old-for-patients/living-with-nets/carcinoid-syndrome/

  Carcinoid syndrome can develop when a neuroendocrine tumor produces sufficient quantities of substances that cause the symptoms associated with the condition. NETs occur in about 7 out of every 100,000 people. Among those living with NETs, researchers estimate 19% develop carcinoid syndrome. NET tumors occur in both men and women, occurring slightly more frequently in women, and the typical age (median age) of a person at NET diagnosis is between 55 and 60. NETs arise most frequently (approximately 70% of the time) in the gastrointestinal tract, followed by occurrences in the respiratory tract (approximately 25% of the time), and more rarely arise in many other sites. Regardless of where they start (so-called primary site), NETs can subsequently spread extensively to places such as liver, bone, and other sites. Carcinoid syndrome is most commonly caused by NETs that occur in the midgut and spread (metastasize) to the liver; however, in rare instances, carcinoid syndrome may be caused by NETs originating in the foregut and hindgut.

• #10 Carcinoid syndrome: update on the pathophysiology and treatment | Clinics

  https://www.elsevier.es/en-revista-clinics-22-articulo-carcinoid-syndrome-update-on-pathophysiology-S1807593222011310

  Considering all types of NETs and all stages, among patients 65 years old or older, a large epidemiological study from the US reported that 19% of this patient population had CS. […] For several decades, patients with NETs and CS have been first treated with somatostatin analogues. […] Given the complexity involving the origin, diagnosis and management of patients with CS, we have undertaken a comprehensive review to update our knowledge about the pathophysiology, diagnostic tools and treatment options for this syndrome. […] The exact mechanism underlying the development of CHD remains unclear. However, there is strong evidence for the role of 5-HT in the fibrogenesis and stimulation of fibroblast growth in CHD. […] A recent study demonstrated a significant association of MF with elevated urinary 5-HIAA levels, suggesting a potential causal relationship.

• #11 Carcinoid syndrome: Causes and Management – DermNet

  https://dermnetnz.org/topics/carcinoid-syndrome

  Carcinoid syndrome is the result of a combination of peptides and amines secreted by advanced neuroendocrine tumours (NETs) into the bloodstream. […] Neuroendocrine tumours can secrete bioactive amines into the bloodstream, causing symptoms of carcinoid syndrome. The most common amines and peptides are: Serotonin (key molecule associated with carcinoid syndrome), Histamine, Kallikrein, Prostaglandins, Tachykinins.

• #12 Carcinoid syndrome – Wikipedia

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

  Carcinoid syndrome is a paraneoplastic syndrome comprising the signs and symptoms that occur secondary to neuroendocrine tumors (formerly known as carcinoid tumors). The syndrome is caused by neuroendocrine tumors most often found in the gut releasing biologically active substances into the blood causing symptoms such as flushing and diarrhea, and less frequently, heart failure, vomiting and bronchoconstriction. […] The carcinoid syndrome occurs secondary to neuroendocrine tumors. These tumors occur mostly in the gut and less commonly in the lungs, but may also occur in other places in the body such as the pancreas, kidneys, and other organs. Neuroendocrine tumors produce several biologically active substances, mainly amines and peptides. There are over 40 substances known to be secreted by these tumors but the exact effect of each and their contribution to the carcinoid syndrome is unknown.

• #13 Carcinoid Syndrome – NETRF

  https://netrf.org/old-for-patients/living-with-nets/carcinoid-syndrome/

  Carcinoid syndrome is caused by the release or secretion of excessive amounts of bioactive substances (peptides and other hormones) from a neuroendocrine tumor (NET) into the bloodstream. NETs that secrete such substances are considered to be functional NETs. The body’s normal neuroendocrine cells also produce these active substances but in the case of carcinoid syndrome, the neuroendocrine tumor cells produce abnormally high levels of the substances. Once secreted by the NET, these active substances enter the bloodstream, and if they are not metabolized/deactivated by the body, for instance, in the liver, they may produce the pattern of symptoms referred to as carcinoid syndrome. […] NETs can release a multitude of bioactive substances, many of which are largely unknown, that create symptoms. More commonly encountered chemical substances associated with carcinoid syndrome, include serotonin, tachykinins, histamine, kallikrein, and prostaglandins. Serotonin appears to the primary substance associated with the so-called “midgut” carcinoid syndrome; however, the contribution of individual substances known to cause the condition is largely unknown.

• #14 Carcinoid syndrome: update on the pathophysiology and treatment | Clinics

  https://www.elsevier.es/en-revista-clinics-22-articulo-carcinoid-syndrome-update-on-pathophysiology-S1807593222011310

  Approximately 30-40% of patients with well-differentiated neuroendocrine tumors present with carcinoid syndrome, which is a paraneoplastic syndrome associated with the secretion of several humoral factors. […] More than 40 substances have been identified as being potentially related to carcinoid syndrome; however, their individual contributions in triggering different carcinoid symptoms or complications, such as carcinoid heart disease, remain unclear. These substances include serotonin (5-HT), which appears to be the primary marker associated with the syndrome, as well as histamine, kallikrein, prostaglandins, and tachykinins. […] Carcinoid syndrome (CS) is a paraneoplastic syndrome associated with the secretion of several humoral factors, such as polypeptides, vasoactive amines, and prostaglandins.

• #15 Carcinoid syndrome of neuroendocrine neoplasia | Deranged Physiology

  https://derangedphysiology.com/main/required-reading/endocrine-intensive-care/Chapter-228/carcinoid-syndrome-neuroendocrine-neoplasia

  When the tumours are intestinal, carcinoid syndrome develops only after liver metastases appear: the liver usually removes the bioactive mediators from the portal circulation, and they never make it into the systemic circulation. […] In actual fact, there are up to 40 different possible mediators; but generally everybody blames the serotonin. […] Increased metabolism of dietary tryptophan into serotonin (70% conversion rate, instead of the normal 1%). […] Serotonin causes cardiac fibrosis by stimulating fibroblasts (right side is more affected because the lung filters the bioactive mediators, protecting the left sided chambers). […] Bradykinins and prostaglandins cause facial flushing. […] Diversion of tryptophan metabolism may result in niacin deficiency. […] Twenty-four-hour measurement of urinary 5-hydroxyindole-3-acetic acid (5-HIAA), which is the degradation product of serotonin, is apparently 88% specific for serotonin-producing carcinoid tumours.

• #16 Carcinoid Syndrome: An Overview, Symptoms, Causes & Treatment

  https://www.ganeshdiagnostic.com/blog/carcinoid-syndrome-an-overview-symptoms-causes-and-treatment

  About 40 different kinds of biologically active amines and peptides are released by neuroendocrine tumours. […] Most of the clinical characteristics are caused by serotonin, which is a by-product of the breakdown of tryptophan. […] However, up to 70% of tryptophan is converted into serotonin in neuroendocrine tumours. […] Niacin insufficiency consequently causes Pellagra, which presents as a trifecta of diarrhoea, dementia, and dermatitis. […] Additionally, prostaglandins contribute to the gastrointestinal tract’s higher fluid secretion and intestinal motility, which results in diarrhoea.

• #17 Clinical features of carcinoid syndrome – UpToDate

  https://www.uptodate.com/contents/clinical-features-of-carcinoid-syndrome

  „Carcinoid syndrome” is the term applied to a constellation of symptoms mediated by various humoral factors elaborated by some well-differentiated neuroendocrine tumors (NETs) of the digestive tract and lungs, which synthesize, store, and release a variety of polypeptides, biogenic amines, and prostaglandins (table 1). Some of these tumor products are responsible for carcinoid syndrome, but the relative contributions of each and the specificity of any for particular components of the syndrome are uncertain (table 2). […] Carcinoid syndrome is most common in the setting of disseminated disease, particularly liver metastases. […] The liver inactivates bioactive products secreted into the portal circulation. This may explain why patients with gastrointestinal NETs most often develop carcinoid syndrome if they have hepatic metastases, resulting in the secretion of tumor products into the systemic circulation.

• #18 Clinical features of carcinoid syndrome – UpToDate

  https://www.uptodate.com/contents/clinical-features-of-carcinoid-syndrome

  In the large majority of cases, carcinoid syndrome is associated with metastatic tumors originating in the midgut (jejunum, ileum, and cecum); however, the expression is variable in individual patients. […] Less often, carcinoid syndrome is caused by a NET arising in the lung or in the distal colon or rectum (foregut and hindgut embryologic origin, respectively). […] Approximately 1 percent of pancreatic NETs secrete excess serotonin and other vasoactive substances that produce carcinoid syndrome.

• #19 What Gastroenterologists Should Know about Carcinoid Syndrome

  https://www.mdpi.com/2036-7422/13/1/14

  Carcinoid syndrome (CS) is the most common functional syndrome associated with neuroendocrine neoplasia (NEN), particularly in intestinal NEN with extensive liver metastases. […] Carcinoid syndrome (CS) is the most common functional syndrome associated with NEN due to the release of serotonin and vasoactive substances in the systemic circulation. […] More than 40 substances are known to be involved in the pathogenesis of CS, but the main mediator is serotonin (5-HT), which is considered the primary marker associated with the syndrome, and histamine, prostaglandins, and tachykinins. […] In the majority of cases, CS occurs in patients affected by NEN of the small intestine with extensive liver metastases due to the blood bypassing hepatic inactivation when entering the systemic circulation.

• #20 What Gastroenterologists Should Know about Carcinoid Syndrome

  https://www.mdpi.com/2036-7422/13/1/14

  Since serotonin and other vasoactive substances produced by the tumor are usually inactivated by the liver, CS usually occurs when liver metastases are present (87–100% of patients), especially in NEN of the small intestine. […] CHD is a severe complication of CS reported in 20–40% of these patients. It is caused by fibrotic damage to the heart, especially involving the right section. […] Carcinoid syndrome is the most common NEN functional syndrome and is caused by the release of serotonin and other vasoactive substances. It commonly occurs in small intestine NEN with extensive liver metastases.

• #21 Carcinoid Tumor: Treatment, Symptoms, Meaning

  https://www.medicinenet.com/carcinoid_syndrome/article.htm

  Carcinoid syndrome is a combination of symptoms caused by the hormones and other chemical substances released by the tumors into the bloodstream. […] The prevalence of carcinoid tumors is difficult to determine since many carcinoid tumors are not detected because they produce no symptoms. […] Carcinoid syndrome is very rare. This is because many carcinoid tumors are non-secretory — that is, they do not produce the hormones responsible for the carcinoid syndrome. […] The chemicals released by carcinoid tumors, particularly from tumors within the abdomen, often are destroyed by the liver before they reach the general circulation to cause symptoms. […] The presence of carcinoid syndrome usually means that the tumor is malignant and has spread to the liver. […] Approximately 10% of small intestinal carcinoids cause carcinoid syndrome. […] Carcinoid syndrome is rare with rectal carcinoid tumors. […] Carcinoid syndrome is rare with colonic carcinoid tumors.

• #22 Clinical features of carcinoid syndrome – UpToDate

  https://www.uptodate.com/contents/clinical-features-of-carcinoid-syndrome

  In the large majority of cases, carcinoid syndrome is associated with metastatic tumors originating in the midgut (jejunum, ileum, and cecum); however, the expression is variable in individual patients. […] Less often, carcinoid syndrome is caused by a NET arising in the lung or in the distal colon or rectum (foregut and hindgut embryologic origin, respectively). […] Approximately 1 percent of pancreatic NETs secrete excess serotonin and other vasoactive substances that produce carcinoid syndrome.

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

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

  Malignant carcinoid syndrome is characterized by an array of signs and symptoms in particular, hot, red flushing of the face; severe and debilitating diarrhea; and asthma attacks caused by vasoactive hormones secreted by metastases from carcinoid tumors. […] Carcinoid tumors arise from neuroendocrine cells, which are widespread in the human body, especially in the organs derived from the primitive intestine. […] As with many other cancers, the exact cause is unknown. Malignant carcinoid syndrome does not generally appear to be hereditary. […] A study of genetic alterations in small bowel carcinoid tumors found that loss of all or most of chromosome 18 was the most common finding. Heterozygosity was also lost on chromosome arms 9p and 16q.

• #24 Gastrointestinal Neuroendocrine Tumors and Carcinoid Syndrome – Endocrine and Metabolic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/endocrine-and-metabolic-disorders/gastrointestinal-and-pancreatic-neuroendocrine-tumors-nets/gastrointestinal-neuroendocrine-tumors-and-carcinoid-syndrome

  Carcinoid syndrome is usually due to endocrinologically active NETs that develop from neuroendocrine cells (mostly in the ileum) and produce serotonin. […] It can, however, occur as a result of neuroendocrine tumors elsewhere in the gastrointestinal tract (particularly the appendix and rectum), pancreas, bronchi, or, rarely, the gonads. Rarely, certain highly malignant tumors (eg, oat cell carcinoma of the lung, pancreatic islet cell carcinoma, medullary thyroid carcinoma) are responsible. […] An intestinal serotonin-producing NET does not usually cause carcinoid syndrome unless hepatic metastases have occurred because metabolic products released by the tumor are rapidly destroyed by blood and liver enzymes in the portal circulation (eg, serotonin by hepatic monoamine oxidase). […] Hepatic metastases, however, release metabolic products via the hepatic veins directly into the systemic circulation. […] Metabolic products released by primary pulmonary and ovarian carcinoids bypass the portal route and may similarly induce symptoms. […] Rare intestinal NETs with only intra-abdominal spread can drain directly into the systemic circulation or the lymphatics and cause symptoms.

• #25 Carcinoid Syndrome due to Neuroendocrine Tumors – Hormonal and Metabolic Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/hormonal-and-metabolic-disorders/gastrointestinal-and-pancreatic-neuroendocrine-tumors/carcinoid-syndrome-due-to-neuroendocrine-tumors

  Neuroendocrine tumors are noncancerous (benign) or cancerous (malignant) growths that sometimes produce excessive amounts of hormone-like substances (such as serotonin), sometimes resulting in carcinoid syndrome. Carcinoid syndrome is a group of specific symptoms that occur as a result of these excessive hormones. […] Neuroendocrine tumors can produce an excess of hormone-like substances, such as serotonin, bradykinin, histamine, and prostaglandins. Excess levels of these substances can sometimes result in a diverse set of symptoms called carcinoid syndrome. Neuroendocrine tumors use the amino acid tryptophan to produce the excess serotonin. […] When neuroendocrine tumors occur in the digestive tract or pancreas, the substances they produce are released into a blood vessel that flows directly to the liver (portal vein), where enzymes destroy them. Therefore, neuroendocrine tumors that originate in the digestive tract generally do not cause symptoms of carcinoid syndrome unless the tumors have spread to the liver.

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

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

  Carcinoid syndrome refers to a spectrum of symptoms that result from excessive hormone (mainly serotonin) secretion. […] Carcinoid syndrome can occur as a paraneoplastic syndrome in the context of a neuroendocrine tumor. […] The presence of the carcinoid syndrome often indicates hepatic metastatic disease, however, extraintestinal neuroendocrine tumor can also cause carcinoid syndrome without hepatic metastasis as serotonin secretion can bypass the enterohepatic circulation.

• #27 Gastrointestinal Neuroendocrine Tumors and Carcinoid Syndrome – Endocrine and Metabolic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/endocrine-and-metabolic-disorders/gastrointestinal-and-pancreatic-neuroendocrine-tumors-nets/gastrointestinal-neuroendocrine-tumors-and-carcinoid-syndrome

  Carcinoid syndrome is usually due to endocrinologically active NETs that develop from neuroendocrine cells (mostly in the ileum) and produce serotonin. […] It can, however, occur as a result of neuroendocrine tumors elsewhere in the gastrointestinal tract (particularly the appendix and rectum), pancreas, bronchi, or, rarely, the gonads. Rarely, certain highly malignant tumors (eg, oat cell carcinoma of the lung, pancreatic islet cell carcinoma, medullary thyroid carcinoma) are responsible. […] An intestinal serotonin-producing NET does not usually cause carcinoid syndrome unless hepatic metastases have occurred because metabolic products released by the tumor are rapidly destroyed by blood and liver enzymes in the portal circulation (eg, serotonin by hepatic monoamine oxidase). […] Hepatic metastases, however, release metabolic products via the hepatic veins directly into the systemic circulation. […] Metabolic products released by primary pulmonary and ovarian carcinoids bypass the portal route and may similarly induce symptoms. […] Rare intestinal NETs with only intra-abdominal spread can drain directly into the systemic circulation or the lymphatics and cause symptoms.

• #28 Genetic Causes of Neuroendocrine Tumors by Dr. Run Yu – Carcinoid Cancer Foundation

  https://www.carcinoid.org/genetic-causes-neuroendocrine-tumors-dr-run-yu/

  A common question that patients with neuroendocrine tumors (NETs) ask is whether their NETs are caused by inheriting a defective gene. […] Most patients with pancreatic NETs do not have a clear genetic cause. […] The most common genetic syndrome that causes pancreatic NETs is the multiple endocrine neoplasia type 1 (MEN1). […] The second most common genetic syndrome is the von Hippel-Lindau disease (VHL). […] Two other genetic syndromes, neurofibromatosis type 1 (NF1) and tuberous sclerosis complex (TSC) occasionally cause pancreatic NETs. […] The fifth genetic syndrome, Mahvash disease, which we discovered several years ago based on our clinical practice, causes pancreatic NETs and very high glucagon levels. […] In contrast to pancreatic NETs, small bowel carcinoids are not associated with any known genetic syndromes.

• #29 Neuroendocrine Tumors (NETs): Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/22006-neuroendocrine-tumors-net

  Multiple endocrine neoplasia (MEN): This condition causes overactive tumors to form in various organs and endocrine system glands. Type 1 (MEN1) is the most common cancer syndrome associated with NETs. Its especially common in people with pancreatic NETs (P-NETs). […] Von Hippel-Lindau syndrome: This condition increases your risk of benign and cancerous tumor growth in various parts of your body. […] Neurofibromatosis type 1: This condition causes tumors to grow on your skin and nerves, affecting your eyes and other parts of your body. […] Tuberous sclerosis complex: This condition causes benign tumors that can form anywhere on your body but most commonly in your brain. […] Cowden syndrome: This condition causes benign tumor-like growths and increases your risk of some cancers.

• #30 Explore Carcinoid Tumor Symptoms And Causes Symptoms and Causes at HCG Oncology

  https://www.hcgoncology.com/types-of-cancers/carcinoid-tumours-symptoms-and-causes/

  Carcinoid tumor causes include the presence of genetic diseases. […] Some of the causes of carcinoid tumors are: […] It is a genetic condition and is one of the carcinoid tumor causes. The condition is characterized by tumors in pancreatic islet cells, parathyroid glands, or the pituitary gland. […] It is also a genetic condition and is one of the carcinoid tumor causes. It involves the development of carcinoid tumors in the parathyroid, thyroid, and adrenal glands. […] It is a rare genetic condition and could serve as a carcinoid tumor cause. Patients with this condition may develop cysts or tumors in various organs, such as the spinal cord, brain, eyes, pancreas, and kidneys. […] It is also known as von Recklinghausen disease and is one of the carcinoid tumor causes. People with this condition may develop carcinoid tumors. […] The carcinoid tumor causes include MEN1, MEN2, Von Hippel-Lindau syndrome, and neurofibromatosis type 1. […] Genetic causes of carcinoid tumors include MEN1, MEN2, Von Hippel-Lindau syndrome, and neurofibromatosis type 1.

• #31 Gastrointestinal Carcinoid Tumor Risk Factors | American Cancer Society

  https://www.cancer.org/cancer/types/gastrointestinal-carcinoid-tumor/causes-risks-prevention/risk-factors.html

  Carcinoid tumors are also more common among people with tuberous sclerosis complex, von Hippel Lindau disease and familial small intestinal neuroendocrine tumor. […] Some studies estimate that inherited mutations of the MEN1 gene are responsible for about 5% to 10% of carcinoid tumors. […] People with certain diseases that damage the stomach and reduce the amount of acid it makes (such as atrophic gastritis or Zollinger-Ellison syndrome) have a greater risk of developing stomach carcinoid tumors, but their risk for carcinoid tumors of other organs is not affected. […] Having a family history of any cancer, specifically in a first-degree relative (sibling, parent, or child), or a family history of a neuroendocrine tumor or carcinoid tumor seems to raise the risk of developing a carcinoid tumor. […] It is not clear if smoking increases the risk of getting a carcinoid tumor. Further studies are needed.

• #32 Genetic Causes of Neuroendocrine Tumors by Dr. Run Yu – Carcinoid Cancer Foundation

  https://www.carcinoid.org/genetic-causes-neuroendocrine-tumors-dr-run-yu/

  A common question that patients with neuroendocrine tumors (NETs) ask is whether their NETs are caused by inheriting a defective gene. […] Most patients with pancreatic NETs do not have a clear genetic cause. […] The most common genetic syndrome that causes pancreatic NETs is the multiple endocrine neoplasia type 1 (MEN1). […] The second most common genetic syndrome is the von Hippel-Lindau disease (VHL). […] Two other genetic syndromes, neurofibromatosis type 1 (NF1) and tuberous sclerosis complex (TSC) occasionally cause pancreatic NETs. […] The fifth genetic syndrome, Mahvash disease, which we discovered several years ago based on our clinical practice, causes pancreatic NETs and very high glucagon levels. […] In contrast to pancreatic NETs, small bowel carcinoids are not associated with any known genetic syndromes.

• #33 Carcinoid Tumors

  https://www.health.ny.gov/statistics/cancer/registry/abouts/carcinoid.htm

  Carcinoid tumors, or carcinoids, are typically slow-growing tumors of neuroendocrine cells, specialized cells in the body that release hormones into the bloodstream when acted upon by nerve cells. […] Sometimes, particularly when a malignant tumor has spread, the hormone-producing cells that make up the tumor release enough hormone-like substances into the bloodstream to cause symptoms. This is called the carcinoid syndrome. Symptoms of carcinoid syndrome most often include hot, red flushing of the face, diarrhea, and wheezing, as well as others that depend on which hormone-like substances the tumor is releasing. […] Very few risk factors have been identified for carcinoid tumors. Carcinoid tumors are more likely to develop in people who have a family history of certain rare genetic syndromes, including multiple endocrine neoplasia type 1 (MEN1), and neurofibromatosis. […] Some studies suggest that smoking may be associated with a greater risk of carcinoids of the small intestine or lungs, but more research needs to be done to confirm this.

• #34 Genetic Causes of Neuroendocrine Tumors by Dr. Run Yu – Carcinoid Cancer Foundation

  https://www.carcinoid.org/genetic-causes-neuroendocrine-tumors-dr-run-yu/

  Small bowel carcinoids very rarely happen in the same family but the genetic basis of this phenomenon has not been clearly worked out. […] Important clues to a genetic syndrome are young age of diagnosis, one patient having multiple NETs (or other tumors) in one or more organs, family history of similar or different NETs, and endocrine cell hyperplasia and microadenomas in the surgical specimen.

• #35 Azthena logo with the word Azthena

  https://www.news-medical.net/health/Causes-of-Carcinoid-Tumors.aspx

  The causes for carcinoid tumors are obscure. Genetic factors are thought to be implicated in addition to a myriad of other factors. Anomalies in chromosomes comprise differences such as loss of heterogeneity and numerical imbalances. […] Many carcinoid tumors occur together with MEN 1. Despite the fact that neuroendocrine tumors depend on the site of the MEN 1 gene mutation, the genetic events underlying the neoplastic process are mostly unfamiliar. Genetic cases except those related to MEN 1 are uncommon, but they usually take place. […] Various studies have shown a loss of heterozygosity (LOH) with the MEN1 gene. It has been found that abnormalities related to this gene are connected to chromosome 11. These are a part of or independent of MEN 1. Among the 5 of 9 exemplary carcinoid tumors of the lung, three apparent regions of allelic loss have been diagnosed at bands 11q13.1 (D11S1883), 11q14.3-11q21 (D11S906), and 11q25 (D11S910).

• #36 Azthena logo with the word Azthena

  https://www.news-medical.net/health/Causes-of-Carcinoid-Tumors.aspx

  A few abnormal carcinoids have LOH at band 11q13, between markers PYGM and D11S937, and at bands 11q14.3-11q21 (D11S906), 11q23.2-23.3 (D11S939), and 11q25 (D11S910). The region at band 11q13 with MEN 1 may be more affected in abnormal carcinoid tumors than that in normal carcinoid tumors. Thus, band 11q13 is considered significant among these tumors. Destructive abnormal carcinoid tumors, characterized by high mitosis, vascular invasion and organ metastasis, also seem to possess more allelic losses than other types of tumors. […] Band 11q13 with the MEN 1 gene possibly acts as a tumor-suppressor gene. In the investigation of 46 sporadically occurring tumors, 78% had LOH at this site, with five patients lacking nearly all alleles. The rest had an unbroken pattern in genetic heterozygosity. Some studies have found that inactivation of a tumor-suppressor gene on chromosome 11 and genetic mutations provoking DNA-mismatch repair result in sporadically occurring carcinoid tumors.

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

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

  Malignant carcinoid syndrome is characterized by an array of signs and symptoms in particular, hot, red flushing of the face; severe and debilitating diarrhea; and asthma attacks caused by vasoactive hormones secreted by metastases from carcinoid tumors. […] Carcinoid tumors arise from neuroendocrine cells, which are widespread in the human body, especially in the organs derived from the primitive intestine. […] As with many other cancers, the exact cause is unknown. Malignant carcinoid syndrome does not generally appear to be hereditary. […] A study of genetic alterations in small bowel carcinoid tumors found that loss of all or most of chromosome 18 was the most common finding. Heterozygosity was also lost on chromosome arms 9p and 16q.

• #38 Azthena logo with the word Azthena

  https://www.news-medical.net/health/Causes-of-Carcinoid-Tumors.aspx

  A few abnormal carcinoids have LOH at band 11q13, between markers PYGM and D11S937, and at bands 11q14.3-11q21 (D11S906), 11q23.2-23.3 (D11S939), and 11q25 (D11S910). The region at band 11q13 with MEN 1 may be more affected in abnormal carcinoid tumors than that in normal carcinoid tumors. Thus, band 11q13 is considered significant among these tumors. Destructive abnormal carcinoid tumors, characterized by high mitosis, vascular invasion and organ metastasis, also seem to possess more allelic losses than other types of tumors. […] Band 11q13 with the MEN 1 gene possibly acts as a tumor-suppressor gene. In the investigation of 46 sporadically occurring tumors, 78% had LOH at this site, with five patients lacking nearly all alleles. The rest had an unbroken pattern in genetic heterozygosity. Some studies have found that inactivation of a tumor-suppressor gene on chromosome 11 and genetic mutations provoking DNA-mismatch repair result in sporadically occurring carcinoid tumors.

• #39 Azthena logo with the word Azthena

  https://www.news-medical.net/health/Causes-of-Carcinoid-Tumors.aspx

  The formation of carcinoid tumors is infrequent. There is not much knowledge about the factors that lead to these tumors, and physicians consider that, in most cases, they occur unexpectedly. A risk factor is that which leads to acquiring a disease. Risk factors depend on the type of cancer. It is not absolute that one or more risk factors will cause cancer. It can be seen that a person with no risk factors may get cancer, whereas a person with many risk factors may not get cancer. […] African descendants are a little more exposed to carcinoids than are people of Caucasian descent. […] In general, relatively more women develop carcinoids than men. […] These tumors are more common in long-term diabetic patients. […] An inflammation in the stomach lining for a long period (called chronic atrophic gastritis) increases the risk in the stomach. The presence of MEN 1 or a syndrome called Zollinger Ellison, a condition where the stomach secretes more acid than usual, creates a higher risk of stomach carcinoid.

• #40 Azthena logo with the word Azthena

  https://www.news-medical.net/health/Causes-of-Carcinoid-Tumors.aspx

  The formation of carcinoid tumors is infrequent. There is not much knowledge about the factors that lead to these tumors, and physicians consider that, in most cases, they occur unexpectedly. A risk factor is that which leads to acquiring a disease. Risk factors depend on the type of cancer. It is not absolute that one or more risk factors will cause cancer. It can be seen that a person with no risk factors may get cancer, whereas a person with many risk factors may not get cancer. […] African descendants are a little more exposed to carcinoids than are people of Caucasian descent. […] In general, relatively more women develop carcinoids than men. […] These tumors are more common in long-term diabetic patients. […] An inflammation in the stomach lining for a long period (called chronic atrophic gastritis) increases the risk in the stomach. The presence of MEN 1 or a syndrome called Zollinger Ellison, a condition where the stomach secretes more acid than usual, creates a higher risk of stomach carcinoid.

• #41 Carcinoid Syndrome – CRCAZ

  https://crcaz.com/areas-of-specialty/abdominal-condition-surgery/carcinoid-syndrome/

  A group of symptoms caused by malignant tumors (carcinoids) in the wall of the intestine. Carcinoids secrete serotonin, histamine, and prostaglandins (powerful chemicals that cause carcinoid symptoms). Primary tumors appear in the appendix, ileum, rectum, ovaries or stomach. The malignancy may spread and cause symptoms that affect the skin, blood vessels, kidney, gastrointestinal tract, liver, heart and lungs. […] As of now the exact causes of carcinoid syndrome are unknown. However, we do know that the factors the increase risk of getting carcinoid syndrome are: Adults over 60. Obesity. Smoking. Excess alcohol consumption.

• #42 Carcinoid Tumors and Syndrome | Concise Medical Knowledge

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

  Carcinoid syndrome describes the signs and symptoms associated with unregulated vasoactive hormone production by neuroendocrine tumors. […] Vasoactive substances produced by NET of the GI tract do not cause carcinoid syndrome until the tumors metastasize to the liver. […] Carcinoid syndrome occurs in approximately 5% of carcinoid tumors. […] Exact cause is unknown. […] Several risk factors have been identified: Approximately 10% of GI carcinoids are associated with multiple endocrine neoplasia. […] Other associations: neurofibromatosis type 1, Von-Hippel-Lindau (VHL) syndrome, tuberous sclerosis. […] GI carcinoids are more common in Blacks. […] Lung carcinoids are more common in whites. […] Classic carcinoid syndrome is associated with midgut tumors metastatic to liver. […] Associated mostly with serotonin production. […] No symptoms appear while the tumor is confined to the gut, as serotonin is metabolized by the liver. […] Other substances contributing to clinical symptoms: Histamine, Kallikrein, Tachykinins, Prostaglandins.

• #43 Azthena logo with the word Azthena

  https://www.news-medical.net/health/Causes-of-Carcinoid-Tumors.aspx

  It is not quite clear whether the risk factors increase the chance of tumors. […] The age for examining GI carcinoid tumors is 55-65 and for lung carcinoid tumors, between 45-55. There is less chance of cancers affecting children. […] Some studies suggest that the intake of more saturated fat increases the risk for carcinoid in the small intestine in comparison with intake of low saturated fat. However, more research has to be done in this area. […] These increase ones chances of developing carcinoids. […] Unnaturally obese people, having body mass index greater than 35, have twice the risk of cancer in the small intestine in comparison with those of normal weight. […] This is another risk factor and is believed to be dependent on the type and duration of the therapy.

• #44 Azthena logo with the word Azthena

  https://www.news-medical.net/health/Causes-of-Carcinoid-Tumors.aspx

  The formation of carcinoid tumors is infrequent. There is not much knowledge about the factors that lead to these tumors, and physicians consider that, in most cases, they occur unexpectedly. A risk factor is that which leads to acquiring a disease. Risk factors depend on the type of cancer. It is not absolute that one or more risk factors will cause cancer. It can be seen that a person with no risk factors may get cancer, whereas a person with many risk factors may not get cancer. […] African descendants are a little more exposed to carcinoids than are people of Caucasian descent. […] In general, relatively more women develop carcinoids than men. […] These tumors are more common in long-term diabetic patients. […] An inflammation in the stomach lining for a long period (called chronic atrophic gastritis) increases the risk in the stomach. The presence of MEN 1 or a syndrome called Zollinger Ellison, a condition where the stomach secretes more acid than usual, creates a higher risk of stomach carcinoid.

• #45 Gastrointestinal Carcinoid Tumor Risk Factors | American Cancer Society

  https://www.cancer.org/cancer/types/gastrointestinal-carcinoid-tumor/causes-risks-prevention/risk-factors.html

  Carcinoid tumors are also more common among people with tuberous sclerosis complex, von Hippel Lindau disease and familial small intestinal neuroendocrine tumor. […] Some studies estimate that inherited mutations of the MEN1 gene are responsible for about 5% to 10% of carcinoid tumors. […] People with certain diseases that damage the stomach and reduce the amount of acid it makes (such as atrophic gastritis or Zollinger-Ellison syndrome) have a greater risk of developing stomach carcinoid tumors, but their risk for carcinoid tumors of other organs is not affected. […] Having a family history of any cancer, specifically in a first-degree relative (sibling, parent, or child), or a family history of a neuroendocrine tumor or carcinoid tumor seems to raise the risk of developing a carcinoid tumor. […] It is not clear if smoking increases the risk of getting a carcinoid tumor. Further studies are needed.

• #46 Gastrointestinal Carcinoid Tumor Risk Factors | American Cancer Society

  https://www.cancer.org/cancer/types/gastrointestinal-carcinoid-tumor/causes-risks-prevention/risk-factors.html

  Carcinoid tumors are also more common among people with tuberous sclerosis complex, von Hippel Lindau disease and familial small intestinal neuroendocrine tumor. […] Some studies estimate that inherited mutations of the MEN1 gene are responsible for about 5% to 10% of carcinoid tumors. […] People with certain diseases that damage the stomach and reduce the amount of acid it makes (such as atrophic gastritis or Zollinger-Ellison syndrome) have a greater risk of developing stomach carcinoid tumors, but their risk for carcinoid tumors of other organs is not affected. […] Having a family history of any cancer, specifically in a first-degree relative (sibling, parent, or child), or a family history of a neuroendocrine tumor or carcinoid tumor seems to raise the risk of developing a carcinoid tumor. […] It is not clear if smoking increases the risk of getting a carcinoid tumor. Further studies are needed.

• #47 Azthena logo with the word Azthena

  https://www.news-medical.net/health/Causes-of-Carcinoid-Tumors.aspx

  It is not quite clear whether the risk factors increase the chance of tumors. […] The age for examining GI carcinoid tumors is 55-65 and for lung carcinoid tumors, between 45-55. There is less chance of cancers affecting children. […] Some studies suggest that the intake of more saturated fat increases the risk for carcinoid in the small intestine in comparison with intake of low saturated fat. However, more research has to be done in this area. […] These increase ones chances of developing carcinoids. […] Unnaturally obese people, having body mass index greater than 35, have twice the risk of cancer in the small intestine in comparison with those of normal weight. […] This is another risk factor and is believed to be dependent on the type and duration of the therapy.

• #48 Gastrointestinal Carcinoid Tumor Risk Factors | American Cancer Society

  https://www.cancer.org/cancer/types/gastrointestinal-carcinoid-tumor/causes-risks-prevention/risk-factors.html

  Carcinoid tumors are also more common among people with tuberous sclerosis complex, von Hippel Lindau disease and familial small intestinal neuroendocrine tumor. […] Some studies estimate that inherited mutations of the MEN1 gene are responsible for about 5% to 10% of carcinoid tumors. […] People with certain diseases that damage the stomach and reduce the amount of acid it makes (such as atrophic gastritis or Zollinger-Ellison syndrome) have a greater risk of developing stomach carcinoid tumors, but their risk for carcinoid tumors of other organs is not affected. […] Having a family history of any cancer, specifically in a first-degree relative (sibling, parent, or child), or a family history of a neuroendocrine tumor or carcinoid tumor seems to raise the risk of developing a carcinoid tumor. […] It is not clear if smoking increases the risk of getting a carcinoid tumor. Further studies are needed.

• #49 Carcinoid Tumors: Symptoms, Diagnosis, Treatment, and Outlook

  https://www.healthline.com/health/cancer/carcinoid-tumor

  According to the ACS, most lung carcinoid tumors dont have a link to smoking, but some atypical types may have a connection. […] The risk of carcinoid tumors spreading to other organs seems to be related to their size. Experts state that only about 15% of tumors smaller than 1 centimeter (cm), or 0.4 inches (in) in diameter spread to distant tissues. With tumors larger than 2 cm, or 0.8 in, 95% of them spread.

• #50 Carcinoid Syndrome – CRCAZ

  https://crcaz.com/areas-of-specialty/abdominal-condition-surgery/carcinoid-syndrome/

  A group of symptoms caused by malignant tumors (carcinoids) in the wall of the intestine. Carcinoids secrete serotonin, histamine, and prostaglandins (powerful chemicals that cause carcinoid symptoms). Primary tumors appear in the appendix, ileum, rectum, ovaries or stomach. The malignancy may spread and cause symptoms that affect the skin, blood vessels, kidney, gastrointestinal tract, liver, heart and lungs. […] As of now the exact causes of carcinoid syndrome are unknown. However, we do know that the factors the increase risk of getting carcinoid syndrome are: Adults over 60. Obesity. Smoking. Excess alcohol consumption.

• #51 What is Carcinoid Syndrome, and How Can Functional Medicine Help?

  https://www.rupahealth.com/post/what-is-carcinoid-syndrome-and-how-can-functional-medicine-help

  Some complications of carcinoid syndrome can be life-threatening, including carcinoid heart disease that can lead to heart failure and mesenteric fibrosis when inflammation and scarring of the mesentery occurs, causing scarring of the fold of tissue that attaches your intestines to the wall of your abdomen. […] In rare cases, a carcinoid crisis can occur due to surgery, other medical procedures, or traumatic events. This is an emergency that causes significant changes in blood pressure, including low blood pressure (hypotension), shortness of breath, confusion, and severe flushing.

• #52 What Gastroenterologists Should Know about Carcinoid Syndrome

  https://www.mdpi.com/2036-7422/13/1/14

  Since serotonin and other vasoactive substances produced by the tumor are usually inactivated by the liver, CS usually occurs when liver metastases are present (87–100% of patients), especially in NEN of the small intestine. […] CHD is a severe complication of CS reported in 20–40% of these patients. It is caused by fibrotic damage to the heart, especially involving the right section. […] Carcinoid syndrome is the most common NEN functional syndrome and is caused by the release of serotonin and other vasoactive substances. It commonly occurs in small intestine NEN with extensive liver metastases.

• #53 What Gastroenterologists Should Know about Carcinoid Syndrome

  https://www.mdpi.com/2036-7422/13/1/14

  Since serotonin and other vasoactive substances produced by the tumor are usually inactivated by the liver, CS usually occurs when liver metastases are present (87–100% of patients), especially in NEN of the small intestine. […] CHD is a severe complication of CS reported in 20–40% of these patients. It is caused by fibrotic damage to the heart, especially involving the right section. […] Carcinoid syndrome is the most common NEN functional syndrome and is caused by the release of serotonin and other vasoactive substances. It commonly occurs in small intestine NEN with extensive liver metastases.

• #54 Carcinoid syndrome of neuroendocrine neoplasia | Deranged Physiology

  https://derangedphysiology.com/main/required-reading/endocrine-intensive-care/Chapter-228/carcinoid-syndrome-neuroendocrine-neoplasia

  When the tumours are intestinal, carcinoid syndrome develops only after liver metastases appear: the liver usually removes the bioactive mediators from the portal circulation, and they never make it into the systemic circulation. […] In actual fact, there are up to 40 different possible mediators; but generally everybody blames the serotonin. […] Increased metabolism of dietary tryptophan into serotonin (70% conversion rate, instead of the normal 1%). […] Serotonin causes cardiac fibrosis by stimulating fibroblasts (right side is more affected because the lung filters the bioactive mediators, protecting the left sided chambers). […] Bradykinins and prostaglandins cause facial flushing. […] Diversion of tryptophan metabolism may result in niacin deficiency. […] Twenty-four-hour measurement of urinary 5-hydroxyindole-3-acetic acid (5-HIAA), which is the degradation product of serotonin, is apparently 88% specific for serotonin-producing carcinoid tumours.

• #55 Carcinoid syndrome: update on the pathophysiology and treatment | Clinics

  https://www.elsevier.es/en-revista-clinics-22-articulo-carcinoid-syndrome-update-on-pathophysiology-S1807593222011310

  Considering all types of NETs and all stages, among patients 65 years old or older, a large epidemiological study from the US reported that 19% of this patient population had CS. […] For several decades, patients with NETs and CS have been first treated with somatostatin analogues. […] Given the complexity involving the origin, diagnosis and management of patients with CS, we have undertaken a comprehensive review to update our knowledge about the pathophysiology, diagnostic tools and treatment options for this syndrome. […] The exact mechanism underlying the development of CHD remains unclear. However, there is strong evidence for the role of 5-HT in the fibrogenesis and stimulation of fibroblast growth in CHD. […] A recent study demonstrated a significant association of MF with elevated urinary 5-HIAA levels, suggesting a potential causal relationship.

• #56 What is Carcinoid Syndrome, and How Can Functional Medicine Help?

  https://www.rupahealth.com/post/what-is-carcinoid-syndrome-and-how-can-functional-medicine-help

  Some complications of carcinoid syndrome can be life-threatening, including carcinoid heart disease that can lead to heart failure and mesenteric fibrosis when inflammation and scarring of the mesentery occurs, causing scarring of the fold of tissue that attaches your intestines to the wall of your abdomen. […] In rare cases, a carcinoid crisis can occur due to surgery, other medical procedures, or traumatic events. This is an emergency that causes significant changes in blood pressure, including low blood pressure (hypotension), shortness of breath, confusion, and severe flushing.

• #57 Carcinoid syndrome: update on the pathophysiology and treatment | Clinics

  https://www.elsevier.es/en-revista-clinics-22-articulo-carcinoid-syndrome-update-on-pathophysiology-S1807593222011310

  Considering all types of NETs and all stages, among patients 65 years old or older, a large epidemiological study from the US reported that 19% of this patient population had CS. […] For several decades, patients with NETs and CS have been first treated with somatostatin analogues. […] Given the complexity involving the origin, diagnosis and management of patients with CS, we have undertaken a comprehensive review to update our knowledge about the pathophysiology, diagnostic tools and treatment options for this syndrome. […] The exact mechanism underlying the development of CHD remains unclear. However, there is strong evidence for the role of 5-HT in the fibrogenesis and stimulation of fibroblast growth in CHD. […] A recent study demonstrated a significant association of MF with elevated urinary 5-HIAA levels, suggesting a potential causal relationship.

• #58 What is Carcinoid Syndrome, and How Can Functional Medicine Help?

  https://www.rupahealth.com/post/what-is-carcinoid-syndrome-and-how-can-functional-medicine-help

  Some complications of carcinoid syndrome can be life-threatening, including carcinoid heart disease that can lead to heart failure and mesenteric fibrosis when inflammation and scarring of the mesentery occurs, causing scarring of the fold of tissue that attaches your intestines to the wall of your abdomen. […] In rare cases, a carcinoid crisis can occur due to surgery, other medical procedures, or traumatic events. This is an emergency that causes significant changes in blood pressure, including low blood pressure (hypotension), shortness of breath, confusion, and severe flushing.

• #59 Symptoms of neuroendocrine tumours (NETs) | Canadian Cancer Society

  https://cancer.ca/en/cancer-information/cancer-types/neuroendocrine-tumours/signs-and-symptoms

  Carcinoid syndrome is a group of symptoms caused by a NET releasing large amounts of serotonin and other chemicals into the blood. Carcinoid syndrome may happen in people with any type of NET. It most commonly occurs with NETs of the small intestine that have spread to the liver (liver metastases). […] Carcinoid crisis is a severe case of flushing, low blood pressure, difficulty breathing and an irregular heartbeat. It may be triggered by anesthesia, surgery or other treatments. Carcinoid crisis is a serious and possibly life-threatening problem that needs to be treated right away.

• #60 Carcinoid Crisis in the Intensive Care Unit | SpringerLink

  https://link.springer.com/10.1007/978-3-319-74698-2_82-1

  Carcinoid tumors are slow-growing, usually asymptomatic neuroendocrine tumors of enterochromaffin cell origin. […] Functional neuroendocrine tumors secrete many different mediators such as histamine, 5-hydroxytryptamine (serotonin), and 5-hydroxytryptophan. […] Carcinoid syndrome is characterized by episodic flushing, diarrhea, wheezing, and right heart valve disease due to these mediators. […] There are many factors triggering the carcinoid crisis. The most common cause is anesthesia and surgery. […] However there are some risk factors such as presence of carcinoid syndrome, elevated 5-HIAA, high tumor burden, metastatic disease, carcinoid heart disease, liver metastases, duration of anesthesia, and advanced patient age.

• #61 Carcinoid Syndrome: An Overview, Symptoms, Causes & Treatment

  https://www.ganeshdiagnostic.com/blog/carcinoid-syndrome-an-overview-symptoms-causes-and-treatment

  About 40 different kinds of biologically active amines and peptides are released by neuroendocrine tumours. […] Most of the clinical characteristics are caused by serotonin, which is a by-product of the breakdown of tryptophan. […] However, up to 70% of tryptophan is converted into serotonin in neuroendocrine tumours. […] Niacin insufficiency consequently causes Pellagra, which presents as a trifecta of diarrhoea, dementia, and dermatitis. […] Additionally, prostaglandins contribute to the gastrointestinal tract’s higher fluid secretion and intestinal motility, which results in diarrhoea.

• #62 Confusion in a patient with carcinoid syndrome | SFEBES2015 | Society for Endocrinology BES 2015 | Endocrine Abstracts

  https://www.endocrine-abstracts.org/ea/0038/ea0038p175

  A 64 year old lady was admitted with a 2 day history of feeling generally unwell. […] She had recently been diagnosed with carcinoid syndrome; primary tumour situated in the terminal ileum with liver metastasis and had been deemed unfit for intervention by the specialist carcinoid team in the tertiary centre. […] Tryptophan is an amino acid which is a precursor for both Niacin and Serotonin. There is diversion of tryptophan to making serotonin in patients with Carcinoid syndrome. This diversion causes decreased protein synthesis and Niacin deficiency leading to clinical manifestations of Pellagra. […] Pellagra should be considered in patients with Carcinoid syndrome. Pellagra is due to deficiency of Niacin and if left untreated can lead to death.

• #63 Confusion in a patient with carcinoid syndrome | SFEBES2015 | Society for Endocrinology BES 2015 | Endocrine Abstracts

  https://www.endocrine-abstracts.org/ea/0038/ea0038p175

  A 64 year old lady was admitted with a 2 day history of feeling generally unwell. […] She had recently been diagnosed with carcinoid syndrome; primary tumour situated in the terminal ileum with liver metastasis and had been deemed unfit for intervention by the specialist carcinoid team in the tertiary centre. […] Tryptophan is an amino acid which is a precursor for both Niacin and Serotonin. There is diversion of tryptophan to making serotonin in patients with Carcinoid syndrome. This diversion causes decreased protein synthesis and Niacin deficiency leading to clinical manifestations of Pellagra. […] Pellagra should be considered in patients with Carcinoid syndrome. Pellagra is due to deficiency of Niacin and if left untreated can lead to death.

• #64 Carcinoid syndrome: MedlinePlus Medical EncyclopediaLock

  https://medlineplus.gov/ency/article/000347.htm

  Carcinoid syndrome is a group of symptoms associated with carcinoid tumors. These tumors release too much of the hormone serotonin, as well as several other chemicals. These hormones cause the blood vessels to open (dilate). This causes carcinoid syndrome. […] Carcinoid syndrome occurs in very few people with carcinoid tumors, typically after the tumor has spread to the liver or lung. […] Treating the tumor reduces the risk of carcinoid syndrome.

• #65 Carcinoid syndrome and carcinoid tumours | NHS inform

  https://www.nhsinform.scot/illnesses-and-conditions/cancer/cancer-types-in-adults/carcinoid-syndrome-and-carcinoid-tumours

  Carcinoid syndrome is the collection of symptoms some people get when a carcinoid tumour usually one that has spread to the liver releases hormones such as serotonin into the bloodstream. […] Its not known exactly why carcinoid tumours develop, but its thought that most occur by chance. […] Your chances of developing a carcinoid tumour may be increased if you have a rare family syndrome called multiple endocrine neoplasia type 1 (MEN1). […] Symptoms of carcinoid syndrome can be treated with injections of octreotide and lanreotide.

• #66 Carcinoid Syndrome – Living with NETS

  https://www.livingwithnets.com/about-neuroendocrine-tumours-nets/learn-about-nets/carcinoid-syndrome/

  Carcinoid syndrome is a distinct group of symptoms that some people with neuroendocrine tumours (NETs) may get when NETS from the small intestine (and sometimes other sites) spread to other parts of the body, usually the liver. […] Carcinoid syndrome affects approximately 20% of people with gastrointestinal NETs. […] One key symptom of carcinoid syndrome is diarrhoea which can be lessened by taking a medication that reduces the activity of TPH (a TPH inhibitor). […] An excess of serotonin can cause carcinoid syndrome. […] Liver metastases are the main source of hormones released into the bloodstream in some patients with NETs. Thus treatments that are directed at the liver may help to reduce symptoms of carcinoid syndrome if other methods have not worked.

• #67 Carcinoid syndrome in neuroendocrine tumours (NETs)| Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/neuroendocrine-tumours-nets/about-carcinoid-syndrome

  Some neuroendocrine tumours (NETs) make large amounts of hormones and proteins. The most common substance they make is serotonin. […] They sometimes release serotonin and the other proteins into the bloodstream. This can cause a collection of symptoms called carcinoid syndrome. […] Carcinoid syndrome is most common in people with a small bowel NET which has spread to the liver. It can also happen in people with lung and pancreatic NETs. But this is less common. […] Doctors dont fully understand why this happens. They think its because some NETs make large amounts of hormones and proteins. And these can build up around the heart. […] Carcinoid crisis is a more serious version of carcinoid syndrome. It happens when NETs make large amounts of hormones and proteins. […] To prevent carcinoid crisis, you might have a somatostatin analogue called octreotide. You can have it as an injection, or as a continuous drip into your vein.

• #68 Can You Die from Carcinoid Syndrome? Life Expectancy

  https://www.emedicinehealth.com/can_you_die_from_carcinoid_syndrome/article_em.htm

  Carcinoid syndrome is caused by a neuroendocrine (carcinoid) tumor, and that may lead to liver dysfunction and death in cases where the cancer has spread (metastasized). […] Carcinoid syndrome is caused by an overproduction of hormones such as serotonin by a neuroendocrine tumor, resulting in the characteristic symptoms. […] Carcinoid syndrome commonly occurs when a neuroendocrine tumor that starts in the small intestine spreads to the liver, where the hormones produced by the tumor can enter the bloodstream, which can result in symptoms.

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