Materiały źródłowe

• #1 Carcinoid Syndrome | UCSF Department of Surgery

  https://surgicaloncology.ucsf.edu/condition/carcinoid-syndrome

  Carcinoid syndrome is a disease defined by a constellation of symptoms, physical manifestations, and abnormal laboratory findings. […] Diagnosis of carcinoid tumors therefore requires collaboration between specialists across multiple disciplines, including surgery, internal medicine and oncology, gastroenterology, radiation oncology, radiology, endocrinology, endocrine surgery, molecular genetics, clinical immunology, pathology, and nuclear medicine. […] Symptoms of carcinoid syndrome are frequently mistaken for other diseases, such as Crohns disease, ulcerative colitis, or irritable bowel syndrome (IBS). As a result, delayed diagnosis or misdiagnosis frequently occurs. Patients with carcinoid syndrome may often wait 5 to 7 years from the initial onset of their symptoms to receive the correct diagnosis. And 60% to 80% of patients are diagnosed with advanced disease and liver metastases at initial presentation.

• #1 Carcinoid Syndrome – StatPearls – NCBI Bookshelf

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

  Carcinoid syndrome is a rare clinical condition caused by metastatic, well-differentiated neuroendocrine tumors, primarily originating in the midgut and associated with liver metastases. […] Diagnosis involves biochemical markers and imaging techniques for tumor localization and staging. […] Carcinoid syndrome is a clinical condition caused by metastatic, well-differentiated neuroendocrine tumors, most commonly arising from the midgut with liver metastases, and is characterized by the secretion of biogenic amines, particularly serotonin, leading to symptoms, eg, flushing, diarrhea, bronchospasm, and systemic complications, like carcinoid heart disease. Diagnosis is primarily achieved through measuring urinary 5-hydroxyindoleacetic acid (5-HIAA), supported by imaging for tumor localization and staging.

• #1 Carcinoid syndrome – Diagnosis & treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/carcinoid-syndrome/diagnosis-treatment/drc-20370672

  Your doctor will assess your signs and symptoms to rule out other causes of skin flushing and diarrhea. If no other causes are found, your doctor may suspect carcinoid syndrome. […] To confirm a diagnosis, your doctor may recommend further tests, including: […] Your urine may contain a substance made when your body breaks down serotonin. An excess amount of this substance could indicate that your body is processing extra serotonin, the chemical most commonly excreted by carcinoid tumors. […] Your blood may contain high levels of certain substances that are released by some carcinoid tumors. […] Imaging tests may be used to locate the primary carcinoid tumor and determine whether it has spread. Your doctor may start with a CT scan of your abdomen, because most carcinoid tumors are found in the gastrointestinal tract. Other scans, such as MRI or nuclear medicine scans, may be helpful in certain situations. […] Your doctor may use a long, thin tube equipped with a lens or camera to examine areas inside your body. […] A sample of tissue from the tumor (biopsy) may be collected to confirm your diagnosis. What type of biopsy you’ll undergo depends on where your tumor is located.

• #1 Carcinoid Syndrome: Symptoms, Causes, Diagnosis, and Treatments

  https://www.webmd.com/cancer/carcinoid-syndrome

  If your doctor thinks you have carcinoid syndrome, theyll do a physical exam and may ask you questions like: […] You may also need tests to look for a carcinoid tumor. […] Urine test. You collect your pee in containers over a 24-hour period. A lab will check it for high levels of hormones or what’s left when your body breaks them down. […] Blood test. This could also show chemicals that the tumors release. […] Imaging tests. A CT scan is a series of X-rays that gives detailed views of the inside of your body. An MRI uses strong magnets and radio waves to make pictures of your organs. For radionuclide scanning, your doctor will inject you with a small amount of radioactive material, and your organs will absorb it. A special camera can spot the material and make pictures that help your doctor find a tumor.

• #1

  https://expertperspectives.com/diagnosis-of-carcinoid-syndrome-and-neuroendocrine-tumors/

  While presentations are quite varied, a delay in diagnosis is common, given that the early symptoms are vague and nonspecific, with early syndromic symptoms typically being flushing and intermittent diarrhea. […] Flushing may be confused with the physiologic flushing of perimenopause, as these NETs often begin to become evident from a patient’s late 40s to their late 60s. Flushing from carcinoid syndrome is often described as dry, whereas many women with perimenopausal or menopausal flushing also experience sweating. […] When the functional symptoms of diarrhea or flushing overlap with the nonfunctional symptoms of pain, weight loss, and nausea, that is going to bring your diagnostic potential for NETs with carcinoid syndrome clearly into focus. […] A less-common but important presentation is that of carcinoid heart disease with signs and symptoms of right heart failure. In retrospect, newly diagnosed patients often have had a steady but, perhaps, modest manifestation of carcinoid syndrome over long periods of time; however, their presenting condition is the right heart failure from carcinoid heart disease.

• #1 Carcinoid Syndrome – StatPearls – NCBI Bookshelf

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

  The diagnostic evaluation of carcinoid syndrome involves initial testing for biochemical markers to confirm the presence of the syndrome, followed by tumor localization and staging through radiographic and endoscopic studies. […] The 24-hour urinary 5-HIAA test is the initial diagnostic test for carcinoid syndrome. 5-HIAA is the primary end product of serotonin metabolism, and this test demonstrates approximately 90% sensitivity and specificity. […] Chromogranin A, a glycoprotein secreted by neuroendocrine tumors, is another essential biochemical marker. […] When biochemical markers confirm the diagnosis of carcinoid syndrome, imaging studies are essential for tumor localization and staging. […] Patients with significant elevation of serum serotonin or urinary 5-HIAA (5 times the upper limit of normal), symptoms of carcinoid heart disease, or those undergoing major surgery should undergo echocardiographic evaluation to assess for cardiac involvement.

• #1 Guidelines for the diagnosis and management of carcinoid tumours. Part 1: The gastrointestinal tract. A statement from a Canadian National Carcinoid Expert Group

  https://pmc.ncbi.nlm.nih.gov/articles/PMC1891174/

  Carcinoid tumours are relatively rare and, in general, slow growing. […] A delay of several years frequently occurs before a diagnosis of carcinoid tumour is made. […] In Canada, carcinoid tumours represent less than 0.25% of the oncology patient load. […] However, in Canada, no consensus or guideline for diagnosing and managing this condition has been established. […] The Canadian National Carcinoid Expert Group, whose members have considerable experience in the field, was convened to study the practices pertaining to carcinoid tumours in Canada and to develop guidelines to facilitate diagnosis and treatment. […] Two markers are primarily used to diagnose and follow carcinoid tumours: 5-hydroxy-indole-acetic acid (5-hiaa) and chromogranin A (CgA). […] When measured in a 24-hour urine sample, 5-hiaa level has a sensitivity of 73% and a specificity of 100% for diagnosing carcinoid.

• #1 Malignant Carcinoid Syndrome Workup: Laboratory Studies, Imaging Studies, Other Tests

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

  Hormones in blood and urine are measured to monitor the growth, activity, and eventual recurrence of the primary tumor. The biochemical diagnosis of carcinoid tumors is based on the measurement of the serotonin metabolite 5-hydroxyindoleacetic acid (5-HIAA) in a 24-hour urine collection (normal value [NV] = 0-8.9 mg/d; plasma serotonin NV is 0.040.2 mg/mL). […] If dietary (or pharmaceutical) 5-hydroxyindoles are excluded, a urinary excretion of 5-HIAA of 25 mg/d or more is diagnostic of carcinoid. With values of 9-25 mg/d, the differential diagnosis includes carcinoid syndrome, nontropical sprue, or acute intestinal obstruction. […] According to one study, chromogranin A measurement with a cutoff range of 84 to 87 U/L yields a specificity of 95% and a sensitivity of 55% for the diagnosis of endocrine tumors.

• #1 SciELO Brazil – Carcinoid syndrome: diagnosis and medical management Carcinoid syndrome: diagnosis and medical management

  https://www.scielo.br/j/abem/a/XsNGMwcFV8D6R6Lk5ZLR78j/?format=html

  Because carcinoid tumors frequently present with obscure clinical manifestations, numerous investigatory procedures are often undertaken prior to establishing the correct diagnosis. Although clinical diagnosis is based on symptoms, biochemical confirmation is necessary. The diagnostic strategies employed usually depend on the individual clinical presentation. […] The measurement of 24-hour urinary excretion of 5-HIAA is useful because it provides a summation of tumor secretory activity that may occasionally be missed by random plasma peptide sampling if secretion is paroxysmal. The test specificity is approximately 88%. Certain serotonin-rich foods (bananas, avocados, plums, eggplant, tomatoes, plantain, pineapples, kiwis and walnuts) can increase urinary 5-HIAA levels and should be avoided during specimen collection.

• #1 Carcinoid Tumours: Causes, Symptoms and Treatment | Doctor

  https://patient.info/doctor/carcinoid-tumours

  False negatives may occur with various drugs – eg, alcohol, aspirin, antidepressants, St. John’s wort. […] Other baseline investigations, considering possible associated neuroendocrine neoplasia or bowel adenocarcinoma, include FBC, LFTs, TFTs, parathyroid hormone, calcium, calcitonin, prolactin. alpha-fetoprotein, carcinoembryonic antigen (CEA) and beta-hCG. […] Provocative tests (eg, pentagastrin test) may be considered if other screening test results are equivocal; however, close monitoring and ready access to intravenous somatostatin are essential in case of a carcinoid crisis. […] Gastric and intestinal tumours may be diagnosed by endoscopy or endoscopic ultrasound; barium studies may demonstrate polyps. […] CT or MRI scanning and laparotomy may be needed for localisation. […] Scintigraphic imaging with labelled somatostatin can provide accurate information on the site and dissemination of the tumour. […] Somatostatin single-photon emission computerised tomography (SPECT) may also be used.

• #1 Carcinoid Tumor Workup: Laboratory Studies, Imaging Studies, Procedures

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

  Fasting plasma 5-HIAA assay is more stable than whole-blood serotonin assay and is more convenient than 24-hour urine collection. […] Numerous imaging modalities have been used to detect carcinoid tumors. These modalities include plain radiography, upper-GI and lower-GI radiography with the use of oral contrast agents, CT, MRI, angiography, positron emission tomography (PET), scintigraphy with metaiodobenzylguanidine (MIBG) and octreotide, radionuclide imaging with somatostatin analogs attached to the radioactive tracer, and technetium-99m bone scanning. […] A study sought to determine the test performance of PET-CT for mediastinal lymph node staging of pulmonary carcinoid tumors. The study found that PET-CT has a poor sensitivity but good specificity to detect the presence of mediastinal lymph node metastases in pulmonary carcinoid tumors.

• #1 Guidelines for the diagnosis and management of carcinoid tumours. Part 1: The gastrointestinal tract. A statement from a Canadian National Carcinoid Expert Group

  https://pmc.ncbi.nlm.nih.gov/articles/PMC1891174/

  Levels of CgA tend to correlate with tumour bulk but not with symptoms. […] Scintigraphy using 111In-pentetreotide is one of the most important imaging investigations for identifying and staging carcinoid tumours of the gastrointestinal tract. […] All patients should have an OctreoScan as a baseline, and patients who have undergone curative surgery should have an annual OctreoScan, unless clinical conditions indicate otherwise. […] The main goals of carcinoid tumour management are symptom control, biochemical control (that is, normalizing the 5-hiaa level), objective tumour control, and quality-of-life improvement. […] Somatostatin analogues for symptom control, frequent debulking, and better management of cardiac complications have improved survival rates. […] Surgery is the only way to obtain a complete cure. […] Patients with carcinoid syndrome should have an echocardiogram at diagnosis. […] All patients should have an annual echocardiogram and follow-up with a cardiologist, particularly those with any cardiac changes or with elevated 5-hiaa.

• #1 Carcinoid Syndrome Tests – NETRF

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

  The sensitivity of this test is good (it often identifies people with a NET), but specificity is low (the test may be positive for a person who does not have a NET). […] Imaging scans help find the location(s) of a neuroendocrine tumor, whether it has spread (metastasized), and where it has spread. […] The triple-phase CT scan, which makes a cross-sectional image of the abdomen, is a useful diagnostic test. […] TTE may be performed in patients with carcinoid syndrome when symptoms suggest there is cardiac involvement. […] Endoscopy of the gut and occasionally lung (bronchoscopy) may be performed to locate the NET and may be combined with a biopsy to diagnose and characterize the tumor based on tissue pathology.

• #1 SciELO Brazil – Carcinoid syndrome: diagnosis and medical management Carcinoid syndrome: diagnosis and medical management

  https://www.scielo.br/j/abem/a/XsNGMwcFV8D6R6Lk5ZLR78j/?format=html

  Chromogranin A (CgA) is a member of the chromogranin family, which is stored in the secretory granules of neuroendocrine cells. Because CgA is a constitutive secretory product of most NETs, its detection in plasma can be utilized as a general tumor marker for carcinoids and even for „non-functioning” tumors. In carcinoid tumors, the highest concentrations of CgA were noted in metastatic midgut lesions with CgA elevation in 87% of lesions, whereas 5-HIAA increases was noted in 76%. CgA concentration correlated with tumor burden. Plasma CgA levels are sensitive but nonspecific markers of carcinoid tumors because they are also elevated in pancreatic NETs, as well as in other types of NETs. False-positive increased CgA concentrations can be seen in renal impairment, liver failure, atrophic gastritis, and inflammatory bowel disease. If biochemical results are equivocal, these tests should be repeated and plasma CgA measured because it is the most sensitive and reliable screening test. […] If one of the peptides/amines or its breakdown products are initially elevated, the precise localization of the primary lesion and its metastases should be undertaken, starting with 111In-labelled pentetreotide scintigraphy.

• #1 Azthena logo with the word Azthena

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

  The early stages of carcinoid syndrome are difficult to diagnose for various reasons. Most symptoms are quite common and wide-ranging. […] The doctor will inquire about lifestyle, general health, symptoms, and risk factors. Patients may be asked to describe the symptoms of carcinoid syndrome and a tumor in intestines, stomach, or rectum. […] Gastrointestinal (GI) carcinoid tumors are diagnosed using this method. […] Tumor marker test: Chromogranin A produced by organs, tumor cells, and tissues is a tumor marker in higher concentration and is measured to quantify its amount by testing the sample of urine, blood, and tissue. […] Abnormal levels of the hormones can be a sign of the disease and used as a diagnostic tool for carcinoid. […] Carcinoid syndrome can be diagnosed by testing the blood and urine of patients. 5-HT or serotonin compounds are formed by the carcinoid tumors which are found in the small intestine and cause symptoms of carcinoid syndrome. […] Measuring the level of serotonin in the blood can be very useful. Normal serotonin levels in the blood with high serotonin and 5-HTP levels in urine are found in patients with these types of tumors.

• #1 Malignant Carcinoid Syndrome Workup: Laboratory Studies, Imaging Studies, Other Tests

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

  Recommended follow-up studies vary according to the location of the carcinoid tumor, as follows: Hindgut carcinoids – Chromogranin A, alpha human chorionic gonadotropin (-HCG), beta human chorioinic gonadotropin (-HCG), and polypeptides are essential assays. […] Apart from measuring daily urinary 5-HIAA secretion, determining the presence of other bioactive amines enables more sensitive detection and also may indicate specific therapeutic measures in particular patients. […] Platelet aggregation testing may show increased aggregation with certain agonists. This test helps to diagnose platelet dysfunction and to distinguish between inherited and acquired bleeding problems (eg, disseminated intravascular coagulation [DIC], which occurs in some patients with malignant carcinoid syndrome).

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

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

  How Is Carcinoid Syndrome Diagnosed? […] In addition to a patient history and physical examination, tests used to diagnose carcinoid syndrome include: […] Urine tests […] Blood tests […] 5-HIAA (5-hydroxyindoleacetic acid) […] Chromogranin A (CgA) […] Imaging tests […] Computerized tomography (CT) scan […] Magnetic resonance imaging (MRI) scan […] Iodine-123-meta-iodobenzylguanidine scan (MIBG) […] Endoscopy […] Ultrasound […] Echocardiogram (echo) […] Pancreastatin Test, Substance P and Neurokinin A Tests […] Full body imaging tests: Somatostatin Receptor Scintigraphy (SRS) Test (Octreoscan)

• #1 Malignant Carcinoid Syndrome Workup: Laboratory Studies, Imaging Studies, Other Tests

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

  N-terminal pro-brain natriuretic peptide (NT-pro-BNP) may serve as a biomarker for the detection of carcinoid heart disease. One study found that at a cut-off level of 260 pg/ml, NT-pro-BNP has a sensitivity of 92% and a specificity of 91% for detection of carcinoid heart disease. […] Modalities that have been evaluated for the diagnosis of carcinoids include the following: Iodine-131 metaiodobenzylguanidine (MIBG) scanning, Octreotide scanning, Computed tomography (CT), Gallium-68 (68Ga)-dotatate positron emission tomography (PET)/CT, Magnetic resonance imaging (MRI), Angiography, Venous blood sampling with radioimmunoassay of tumor products, Echocardiography. […] For imaging studies, current National Comprehensive Cancer Network (NCCN) guidelines recommend multiphasic CT or MRI for the evaluation of carcinoid tumors.

• #1 Carcinoid Syndrome | UCSF Department of Surgery

  https://surgicaloncology.ucsf.edu/condition/carcinoid-syndrome

  Absent alternative causes, tests are then performed to determine if carcinoid syndrome is the cause including: […] Urine Test – The urine tests looks for excess serotonin, the chemical most commonly excreted by carcinoid tumors. […] Blood Test – The blood test looks for high levels of the protein chromogranin A, as well as other substances, known to be secreted by carcinoid tumors. […] Imaging – Imaging tests including computerized tomography (CT) scan of the abdomen, MRI and nuclear medicine scans (OctreoScan, Ga-68 DOTATOC/DOTATE PET-CT) are used to identify the primary tumor and determine the extent of metastatic spread.

• #1 Carcinoid tumors – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/carcinoid-tumors/diagnosis-treatment/drc-20351044

  Tests and procedures used to diagnose carcinoid tumors include: […] If you have a carcinoid tumor, your blood may contain high levels of hormones secreted by a carcinoid tumor or byproducts created when those hormones are broken down by the body. […] People with carcinoid tumors have excess levels of a chemical in their urine that’s produced when the body breaks down hormones secreted by carcinoid tumors. […] Imaging tests, including a computerized tomography (CT) scan, magnetic resonance imaging (MRI), positron emission tomography (PET), X-ray and nuclear medicine scans, may help your doctor pinpoint the carcinoid tumor’s location. […] Your doctor may use a long, thin tube equipped with a lens or camera to examine areas inside your body. […] An endoscopy, which involves passing a scope down your throat, may help your doctor see inside your gastrointestinal tract. A bronchoscopy, using a scope passed down your throat and into your lungs, can help find lung carcinoid tumors. Passing a scope through your rectum (colonoscopy) can help diagnose rectal carcinoid tumors.

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

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

  Imaging modalities for gastrointestinal neuroendocrine tumors include the use of somatostatin scintigraphy with indium In 111 (111In)-octreotide; […] The synthetic radiolabeled SSTR analogue 111In-DTP-d-Phe10-{octreotide} affords an important method, somatostatin receptor scintigraphy (SRS), to localize carcinoid tumors, especially sst(2)-positive and sst(5)-positive tumors; imaging is accomplished in one session, and small primary tumors and metastases are diagnosed more readily than with conventional imaging or imaging techniques requiring multiple sessions. […] Overall sensitivity of the octreotide scan is reported to be as high as 90%; however, failed detection may result from various technical issues, small tumor size, or inadequate expression of SSTRs. […] As might be expected, diagnostic approaches to gastrointestinal neuroendocrine tumors vary according to anatomical location.

• #1 Neuroendocrine Tumor Diagnosis | Roswell Park Comprehensive Cancer Center – Buffalo, NY

  https://www.roswellpark.org/cancer/neuroendocrine-tumors/diagnosis

  As a rare cancer, NETs are commonly misdiagnosed as another condition. About half of NETs are not properly diagnosed until later stages when the cancer has spread to other areas. Many carcinoid tumors are discovered incidentally during gastrointestinal surgery. […] Arriving at a definitive diagnosis may require several types of tests and the expertise of several different specialists. You may undergo some of the following: […] NetSpot Scan (gallium-68 dotatate) is the latest imaging innovation that uses a radiotracer agent instead of glucose for a positron emission tomography (PET) scan and can detect small lesions and early-stage NETs significantly better than other imaging. […] In addition to providing exceptional images, showing whether you have neuroendocrine tumors and where, the NetSpot scan can confirm that your cancer has certain features that make it treatable with a radiopharmaceutical drug, a new therapeutic approach called Theranostics.

• #1 SciELO Brazil – Carcinoid syndrome: diagnosis and medical management Carcinoid syndrome: diagnosis and medical management

  https://www.scielo.br/j/abem/a/XsNGMwcFV8D6R6Lk5ZLR78j/?format=html

  Gastro-intestinal carcinoids are slow growing tumors arising from enterochromaffin or Kulchitsky cells. Their clinical presentation depends on what combination of bioactive substances is secreted. Midgut carcinoid can present with the carcinoid syndrome in the presence of liver metastases. Its most typical clinical manifestations include cutaneous flushing and diarrhea. A nonspecific biochemical tumor marker for carcinoid tumors is serum chromogranin A and a specific marker for the carcinoid syndrome is the increased urinary excretion of 5-hydroxy indole acetic acid (5-HIAA). Localizing studies in carcinoid tumors/syndrome are: transabdominal ultrasonography (US), endoscopy, endoscopic US, videocapsule endoscopy, computerized tomography, magnetic resonance imaging, selective abdominal angiography, 111In-pentetreotide scintigraphy (and intraoperative radionuclide probe), 123I (131I)-metaiodobenzylguanidine (MIBG) scintigraphy, bone scintigraphy and 11C-5-HT positron emission tomography (PET).

• #1 Carcinoid Tumor Workup: Laboratory Studies, Imaging Studies, Procedures

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

  Functional imaging with somatostatin receptor-specific radiotracers with fused functional as well as anatomic imaging have significantly improved the diagnosis and follow up of carcinoid tumors. […] Overall, SRS appears to be the imaging method of choice for localizing and evaluating the extent of carcinoid tumor.

• #1

  https://www.cancervic.org.au/cancer-information/types-of-cancer/carcinoid/carcinoid-tumours-overview.html

  Blood tests a full blood count to measure your white blood cells, red blood cells and platelets and a hormone marker called chromogranin A. […] Urine tests these measure the hormone serotonin (which can be produced by NETs). […] Endoscopy a thin, flexible tube with a light and camera (endoscope) is inserted down the throat to examine the digestive tract. […] Gastroscopy an examination of the large bowel with a camera on a flexible tube (colonoscope) which is passed through the anus. […] Bronchoscopy a flexible tube with a light and camera is inserted down the throat to examine the airways and lungs and take tissue samples. […] imaging scans CT (computerised tomography), PET (positron emission tomography) and MRI (magnetic resonance imaging) scans use special machines to create pictures of the inside of your body while you lie still on an examination table. […] Biopsy this removes a sample of tissue from the affected area for examination under a microscope. […] Echocardiogram an ultrasound scan of the heart to check how it is working.

• #1 Carcinoid Tumors: Causes, Symptoms, Diagnosis, and Treatment

  https://www.webmd.com/cancer/carcinoid-tumors

  Colonoscopy. Your doctor inserts a thin, lighted tube through your behind to get a view of your rectum and colon. They may remove pieces of tissue to check for cancer under a microscope. Just like with an endoscopy, you’ll get medicine to keep you pain-free. […] Capsule endoscopy. For this test, you swallow a pill that has a tiny camera in it. This lets your doctor see all of the small intestine, where many carcinoid tumors begin. […] CT, or computed tomography. This powerful X-ray makes detailed pictures inside your body. It can measure the size of your tumor. It can also see whether it has spread to your liver or lymph nodes, which are small glands that are part of your immune system, your body’s defense against germs. You may get a special dye to drink, or take it in through a vein, to help show a clearer picture of the tumor.

• #1 Carcinoid Syndrome: Symptoms, Diagnosis and Treatment

  https://www.cancercenter.com/risk-factors/carcinoid-syndrome

  Endoscopy and bronchoscopy each use a thin, lighted tube with a camera on the end to peer inside the body. An endoscopy gives doctors a look at the GI tract and a bronchoscopy checks out the lungs. Endoscopic ultrasound may facilitate location and sampling of these tumors. […] Biopsy involves removing a small tissue sample to be examined under a microscope to confirm the diagnosis.

• #1 Carcinoid Tumor | Cedars-Sinai

  https://www.cedars-sinai.org/health-library/diseases-and-conditions/c/carcinoid-tumor.html

  It’s hard to diagnose a small carcinoid tumor thats not causing carcinoid syndrome or other symptoms. These tumors are often found when doing surgery or an exam for another condition. For instance, some carcinoid tumors are found when the appendix is removed due to appendicitis. […] These tests may be used to help diagnose carcinoid tumors: […] A surgical procedure may be needed to take out a tiny piece (sample) of tissue from the carcinoid tumor. This process is called a biopsy. The sample is sent to a lab and tested for cancer cells. A biopsy is important for diagnosis. It’s the only way to know for sure that a tumor is cancer. […] If you have a carcinoid tumor that’s making too much serotonin hormone, it might show up as an acid in your urine called 5-HIAA (5-hydroxyindoleacetic acid).

• #1 Carcinoid tumors – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/carcinoid-tumors/diagnosis-treatment/drc-20351044

  A sample of tissue from the tumor (biopsy) may be collected to confirm your diagnosis. What type of biopsy you’ll undergo depends on where your tumor is located. […] The tissue is sent to a laboratory for testing to determine the types of cells in the tumor and how aggressive those cells appear under the microscope.

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

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

  Carcinoid syndrome, which occurs in fewer than 20% of patients with neuroendocrine tumors, is caused by the release of metabolically undergraded vasoactive amines into the systemic circulation. It is associated with flushing, abdominal pain and diarrhea, bronchoconstriction, and carcinoid heart disease. […] Because vasoactive amines are efficiently metabolized by the liver, carcinoid syndrome rarely occurs in the absence of hepatic metastases. Exceptions include circumstances in which venous blood draining from a tumor enters directly into the systemic circulation (e.g., primary pulmonary or ovarian carcinoids, pelvic or retroperitoneal involvement by metastatic or locally invasive small bowel carcinoids, or extensive bone metastases). […] Carcinoid heart disease develops in more than one-third of patients with carcinoid syndrome. Pathologically, the cardiac valves become thickened because of fibrosis, and the tricuspid and pulmonic valves are affected to a greater extent than the mitral and aortic valves. Symptoms include:

• #1 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. […] Given the complexity involving the origin, diagnosis and management of patients with carcinoid syndrome, we have undertaken a comprehensive review to update information about the pathophysiology, diagnostic tools and treatment sequence of this syndrome, which currently comprises a multidisciplinary approach. […] The diagnosis of CS requires the combination of carcinoid symptoms and evidence of elevated levels of 5-HIAA in a 24-h urine sample. The sensitivity and specificity of this test for CS are higher than 90% in patients with characteristic symptoms. […] The diagnosis of CHD is performed by transthoracic echocardiography, which shows valve thickening with retraction and reduction in the mobility of the tricuspid valve in patients with CS. […] MF is diagnosed with an abdominal imaging exam (usually tomography or NMR) that shows at least a spiculated mass with attenuation in the soft tissue range, with fibrotic bands that radiate outward from the mesenteric fat tissue with a star pattern around a metastatic lymph node.

• #1 Diagnosis – INCA

  https://incalliance.org/diagnosis-and-misdiagnosis/

  Studying tissue under a microscope is often the only way to be absolutely sure of a cancer diagnosis. […] An ECG is used to gather information about your heart. […] An echocardiogram is an ultrasound of the heart. It enables doctors to look in detail at the structure of the heart, and in particular at the heart valves.

• #1 Malignant Carcinoid Syndrome Workup: Laboratory Studies, Imaging Studies, Other Tests

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

  Scintigraphy with indium-111 diethylenetriamine pentaacetic acid (DTPA) octreotide (In-111 DTPA Octr), or OctreoScan, localizes the primary carcinoid and eventual recurrences, as well as other neuroendocrine tumors, with high sensitivity and specificity. […] A positive test result usually predicts a good patient response to treatment with octreotide. […] Several provocation tests have been developed for carcinoid syndrome. Intravenous infusion of pentagastrin appears to provoke flushing and other symptoms more reliably than the traditional test, which uses alcohol (10 mL PO), calcium (10 mg/kg of calcium gluconate in 4 h), or catecholamines (norepinephrine 1-20 mcg). These tests must be performed with caution because they can trigger crises. […] Diagnostic and operative endoscopy of the lower and upper GI tract may be helpful for diagnosis. […] In 1963, Williams and Sandler began to classify the carcinoid tumors anatomically and clinically according to embryologic origin from the foregut, midgut, or hindgut.

• #1

  https://link.springer.com/article/10.1007/s00261-019-01945-0

  To determine the rate of missed CT findings of ileal carcinoid tumor prior to pathologic diagnosis and the resultant diagnostic delay. […] Primary ileal tumors were prospectively missed on at least one CT scan in 64% (32/50) of patients with retrospectively identifiable disease. […] Initial presentation of ileal carcinoid tumor, even with mesenteric involvement, is often missed prospectively at abdominal CT, leading to delay in diagnosis until bowel or mesenteric findings become more obvious, or hepatic metastatic disease manifests. […] Radiologists should make a concerted effort to evaluate the bowel and mesentery in patients with long-standing vague abdominal symptoms.

• #1 Late-stage diagnosis of carcinoid heart disease due to lack of access to health care | Cardio-Oncology | Full Text

  https://cardiooncologyjournal.biomedcentral.com/articles/10.1186/s40959-023-00176-z

  As illustrated in our patients cause, timely diagnosis and treatment of CS are imperative for prevention of CHD. Poor access to the US healthcare system leads to a delayed diagnosis, which resulted in significant morbidity and poor prognosis for this young, 35-year old patient. This case highlights the importance of adequate access to healthcare for timely diagnosis and treatment of CS, appropriate screening for CHD once CS is diagnosed, and appropriate monitoring and therapy when CHD develops.

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

  https://www.unboundmedicine.com/5minute/view/5-Minute-Clinical-Consult/1688569/all/Neuroendocrine_Tumor__Carcinoid_Syndrome_?q=Pruritus

  Due to nonspecific presentation, the average time to diagnosis is 9 years. […] Most commonly misdiagnosed as irritable bowel syndrome (IBS). […] 20% of well-differentiated NETs will have carcinoid symptoms.

• #2 Carcinoid Syndrome: Symptoms, Causes, Diagnosis & Treatment

  https://my.clevelandclinic.org/health/diseases/22103-carcinoid-syndrome

  Carcinoid syndrome may be a complication of neuroendocrine tumors (NETs). […] Sometimes, a carcinoid syndrome diagnosis is the first indication you have a neuroendocrine tumor. […] Healthcare providers may use the following tests to diagnose carcinoid syndrome: […] Carcinoid syndrome is linked to neuroendocrine tumors, and as there isn’t a known way to prevent NETs, there’s no way to prevent its symptoms. […] If you have carcinoid syndrome symptoms, tell your healthcare provider. They’ll be able to assess your situation and recommend tests to determine if you have an NET. […] Carcinoid syndrome might be a sign you have a neuroendocrine tumor. While you’ll have questions about carcinoid syndrome, you’ll probably have more questions about NETs. […] Learning you have carcinoid syndrome is your first step toward finding out if you have cancer and if you do, how to treat it. If you have a carcinoid syndrome diagnosis, ask your healthcare provider about next steps to confirm potential NETs.

• #2 Carcinoid Syndrome Diagnosis – NETRF

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

  How is a carcinoid syndrome diagnosis made? Carcinoid syndrome is a constellation, collection, or group of symptoms experienced by a subset of people who have a neuroendocrine tumor. The diagnosis of carcinoid syndrome is complicated by the fact that common conditions, such as irritable bowel syndrome and severe allergic reactions, can cause symptoms similar to those of carcinoid syndrome. To make a carcinoid syndrome diagnosis, a doctor will likely begin by assessing your symptoms and ruling out or excluding other conditions with similar presentations. The process of considering other conditions that may be responsible for your symptoms is called a “differential diagnosis.” […] If you have not been diagnosed with a neuroendocrine tumor, initial tests involve screening for biochemical markers of neuroendocrine tumors, followed by attempts to localize the tumor by using imaging (MRI, CT, scintigraphy) and/or endoscopic tests. […] If you have been diagnosed with a neuroendocrine tumor, testing will try to learn about any changes that may be going on with the tumor.

• #2 Carcinoid Syndrome | Diagnosis & Treatment | Freedmans Health

  https://freedmanshealth.org/diseases-conditions/diagnosis-treatment/carcinoid-syndrome/

  Healthcare providers may use the following tests to diagnose carcinoid syndrome: […] Urine test: This test checks on certain hormone levels. […] Physical exam and medical history: Many carcinoid syndrome symptoms are similar to other less serious illnesses. Your healthcare provider will ask questions about specific symptoms, how long you’ve had the symptoms and if your symptoms are getting worse. […] Blood tests: These tests check on the amounts of substances that your organs and tissues release into your blood. Substance levels that spike or fall might be signs of disease. […] Neuroendocrine positron emission tomography (PET) scan: This test tracks neuroendocrine tumors with a dye called Gallium 68 dotatate. […] Computed tomography (CT) scan: CT scans use X-rays and a computer to produce 3D images of organs and bones.

• #2 Carcinoid Syndrome Tests – NETRF

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

  Because excessive serotonin is one of the main substances responsible for the most commonly encountered midgut carcinoid syndrome, a 24-hour urine collection to measure 5-HIAA is often the initial diagnostic test a doctor will order to determine if a person has a functional neuroendocrine tumor. […] This test has a sensitivity as high as 90%, meaning that it will correctly diagnose 9 out of 10 people with the condition. […] The test has a 90% specificity for the disease, meaning that the test will rarely be positive for a person who does not have the disease (false positive). […] This test is like the 24-hour urine test for 5-HIAA but detects 5-HIAA in the blood, instead of urine. […] Chromogranin A (CgA) is a substance that is produced by a NET and can be a useful test for detecting NETs that may or may not be producing other active substances (e.g., serotonin, histamine) known to contribute to carcinoid syndrome.

• #2 Malignant Carcinoid Syndrome Workup: Laboratory Studies, Imaging Studies, Other Tests

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

  Hormones in blood and urine are measured to monitor the growth, activity, and eventual recurrence of the primary tumor. The biochemical diagnosis of carcinoid tumors is based on the measurement of the serotonin metabolite 5-hydroxyindoleacetic acid (5-HIAA) in a 24-hour urine collection (normal value [NV] = 0-8.9 mg/d; plasma serotonin NV is 0.040.2 mg/mL). […] If dietary (or pharmaceutical) 5-hydroxyindoles are excluded, a urinary excretion of 5-HIAA of 25 mg/d or more is diagnostic of carcinoid. With values of 9-25 mg/d, the differential diagnosis includes carcinoid syndrome, nontropical sprue, or acute intestinal obstruction. […] According to one study, chromogranin A measurement with a cutoff range of 84 to 87 U/L yields a specificity of 95% and a sensitivity of 55% for the diagnosis of endocrine tumors.

• #2 Carcinoid Tumours: Causes, Symptoms and Treatment | Doctor

  https://patient.info/doctor/carcinoid-tumours

  The diagnosis of gastroenteropancreatic neuroendocrine tumours is based on clinical presentation, pathology, and conventional (CT/MRI) or functional imaging. Patients who present with symptoms of carcinoid syndrome should undergo measurement of 24-hour urinary excretion of 5-hydroxyindoleacetic acid (5-HIAA), which is the breakdown product of serotonin. It has been reported that a plasma 5-HIAA assay is equivalent in accuracy to 24-hour urine 5-HIAA measurement. […] Plasma chromogranin A is an effective screening test because it is very sensitive but is not very specific. […] 24-hour urinary excretion of 5-hydroxyindoleacetic acid (5-HIAA): 24-hour excretion greater than 25 mg provides strong evidence for the diagnosis of carcinoid syndrome: […] False positive results may occur with certain foods (eg, bananas, kiwi fruit, pineapple, plums, tomato products) and some drugs (eg, paracetamol, antipsychotics, some cough remedies, caffeine, diazepam, nicotine, warfarin).

• #2 Carcinoid Syndrome Diarrhea

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

  Diagnosis of carcinoid syndrome diarrhea is based on symptoms of diarrhea and flushing combined with objective findings of carcinoid tumor through biopsy, multiphase abdominal/pelvic CT, MRI, or biomarkers. The biomarkers 5-hydroxyindoleacetic acid (5-HIAA; found in the urine) and chromogranin A (CgA; found in the serum) have acceptable specificity and sensitivity for carcinoid tumors. Serotonin is metabolized by monoamine oxidase to 5-HIAA, and CgA is released from the walls of vesicles that store serotonin. Normal urine 5-HIAA levels range from 3 to 15 mg/24 h; an elevated level has 100% specificity and 73% sensitivity for carcinoid tumors. Serum CgA levels tend to correlate with tumor bulk, but not with symptoms. CgA levels greater than 130 mcg/L are associated with 98.4% specificity and 62.9% sensitivity to carcinoid tumors. Patients should avoid serotonin-rich foods 48 hours prior to urine collection. Urine levels of 5-HIAA can be falsely lowered by levodopa, whereas serum CgA levels can be falsely elevated by proton pump inhibitors, renal insufficiency, or inflammatory conditions.

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

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

  Severe carcinoid heart disease is associated with reduced survival. […] Biochemical investigations in the diagnosis of gastrointestinal neuroendocrine tumors include the use of 24-hour urinary 5-hydroxyindoleacetic acid (5-HIAA) collection, which has a specificity of approximately 88%, although the sensitivity is reported to be as low as 35%. […] Measurement of plasma chromogranin A (CgA) is also useful. […] Although plasma levels of CgA are very sensitive markers of gastrointestinal neuroendocrine tumors, they are nonspecific because they are also elevated in other types of neuroendocrine tumors, such as pancreatic and small cell lung carcinomas. […] Numerous investigations have revealed an association between plasma CgA levels and disease severity. […] However, false-positive plasma levels of CgA may occur in patients on proton pump inhibitors, reported to occur even with short-term, low-dose treatment.

• #2 Carcinoid Heart Disease Guidelines

  https://www.acc.org/latest-in-cardiology/ten-points-to-remember/2017/03/07/15/10/diagnosing-and-managing-carcinoid-heart-disease

  Carcinoid heart disease is a frequent occurrence in patients with carcinoid syndrome and is accountable for substantial morbidity and mortality. […] N-terminal proB-type natriuretic peptide (NT-proBNP) appears to be the best biomarker to date for screening carcinoid syndrome patients for evidence of clinically significant carcinoid heart disease (Evidence Level 2-3, Grade B). […] Measurement of either 24-hour urine 5-hydroxyindoleacetic acid (5-HIAA) or plasma 5-HIAA is mandatory for diagnosis and follow-up of carcinoid syndrome. Furthermore, a 24-hour urinary 5-HIAA level 300 mol/24 hour is a useful marker for identifying those at risk of developing carcinoid heart disease (Evidence Level 2, Grade B). […] Transthoracic echocardiography remains the gold standard for diagnosis and follow-up of carcinoid heart disease. It should be performed in all patients with carcinoid syndrome and high suspicion of carcinoid heart disease, such as clinical features or raised NT-proBNP and/or 5-HIAA levels. For established carcinoid heart disease, echocardiography should be performed if dictated by a change in clinical status; otherwise/thereafter every 3-6 months, depending on the severity of established carcinoid heart disease and clinical status (Evidence Level 2, Grade B).

• #2 Carcinoid Syndrome Tests – NETRF

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

  Testing for carcinoid syndrome […] A variety of tests are used to detect and monitor neuroendocrine tumors and carcinoid syndrome, including laboratory tests, and various imaging tests such as PET/CT, MRI, and increasingly sophisticated nuclear medicine studies. […] Laboratory tests focus on detecting the substances (e.g., serotonin) that a neuroendocrine tumor produces that cause carcinoid syndrome symptoms. […] Imaging tests are used to locate the tumor and evaluate the extent of the disease, for instance, if the cancer has spread. […] Other diagnostic tests include upper and/or lower GI endoscopy, with or without ultrasound, to locate tumors and even remove a small piece for testing (called a biopsy). […] Below you will find more information about the types of tests your doctor may order for carcinoid syndrome.

• #2 Carcinoid syndrome – Symptoms, diagnosis and treatment | BMJ Best Practice

  https://bestpractice.bmj.com/topics/en-gb/296

  Carcinoid syndrome commonly presents with flushing and diarrhoea. Other clinical features include wheeze, palpitations, telangiectasia, and abdominal pain. […] Diagnosed by elevated levels of urinary 5-hydroxyindoleacetic acid. Chromogranin A is often elevated in the presence of liver metastases. […] Key diagnostic factors: diarrhoea, flushing. […] Other diagnostic factors: palpitations, abdominal cramps, telangiectasia, signs of right heart failure, cardiac murmurs, hepatomegaly, wheeze, pellagra, abdominal masses. […] 1st investigations to order: serum chromogranin A/B, urinary 5-hydroxyindoleacetic acid, metabolic panel, liver function tests, full blood count. […] Investigations to consider: CT chest, abdomen, and pelvis with dual-phase liver, bronchoscopy, endoscopy, somatostatin receptor scintigraphy somatostatin single photon emission CT (SPECT), iodine I-123 meta-iodobenzylguanidine (MIBG) scintigraphy, histology, PET scan.

• #2 Carcinoid Tumors: Causes, Symptoms, Diagnosis, and Treatment

  https://www.webmd.com/cancer/carcinoid-tumors

  MRI, or magnetic resonance imaging. It uses powerful magnets and radio waves to make pictures of organs and structures inside your body. An MRI can measure the size of the tumor. Just like with the CT scan, you may need to get a special dye to create a clearer image. […] X-ray. It uses radiation in low doses to let your doctor view structures inside your body. It can look for a tumor in your lungs. Before this test, you may need to swallow a liquid containing a chemical called barium that makes the tumor easier for your doctor to see. […] Radionuclide scanning. Before this test, you’ll take in a small amount of a radioactive substance through one of your veins. This substance is attracted to carcinoid tumors. The test can show where in your body the tumor has spread. […] Many people with carcinoid syndrome develop heart complications. Your doctor may suggest you see a heart doctor, called a cardiologist, or get heart study every 2-3 years to monitor your heart.

• #2 Malignant Carcinoid Syndrome Workup: Laboratory Studies, Imaging Studies, Other Tests

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

  Scintigraphy with indium-111 diethylenetriamine pentaacetic acid (DTPA) octreotide (In-111 DTPA Octr), or OctreoScan, localizes the primary carcinoid and eventual recurrences, as well as other neuroendocrine tumors, with high sensitivity and specificity. […] A positive test result usually predicts a good patient response to treatment with octreotide. […] Several provocation tests have been developed for carcinoid syndrome. Intravenous infusion of pentagastrin appears to provoke flushing and other symptoms more reliably than the traditional test, which uses alcohol (10 mL PO), calcium (10 mg/kg of calcium gluconate in 4 h), or catecholamines (norepinephrine 1-20 mcg). These tests must be performed with caution because they can trigger crises. […] Diagnostic and operative endoscopy of the lower and upper GI tract may be helpful for diagnosis. […] In 1963, Williams and Sandler began to classify the carcinoid tumors anatomically and clinically according to embryologic origin from the foregut, midgut, or hindgut.

• #2 Carcinoid Syndrome | General Surgery

  https://generalsurgery.ucsf.edu/condition/carcinoid-syndrome

  Clinicians first seek to rule out other causes of skin flushing and diarrhea. Absent alternative causes, tests are then performed to determine if carcinoid syndrome is the cause including: […] Urine Test – The urine tests looks for excess serotonin, the chemical most commonly excreted by carcinoid tumors. […] Blood Test – The blood test looks for high levels of the protein chromogranin A, as well as other substances, known to be secreted by carcinoid tumors. […] Imaging – Imaging tests including computerized tomography (CT) scan of the abdomen, MRI and nuclear medicine scans (OctreoScan, Ga-68 DOTATOC/DOTATE PET-CT) are used to identify the primary tumor and determine the extent of metastatic spread.

• #2 Carcinoid Tumor Workup: Laboratory Studies, Imaging Studies, Procedures

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

  Functional imaging with somatostatin receptor-specific radiotracers with fused functional as well as anatomic imaging have significantly improved the diagnosis and follow up of carcinoid tumors. […] Overall, SRS appears to be the imaging method of choice for localizing and evaluating the extent of carcinoid tumor.

• #2 Carcinoid Cancer: Definition, Symptoms, Prognosis, and More

  https://www.verywellhealth.com/what-is-carcinoid-cancer-5092725

  Physical exam and health history: During a general check-up body exam. A healthcare provider can take a history of current and past illnesses and treatments, as well as health habits. […] Biopsy: Involves examining a small sample of tissue from the carcinoid tumor under a microscope. […] Lab tests: Urine and/or blood tests look for signs of hormone level changes. […] Tumor marker test: Checking a sample of blood, urine, or tissue to measure the amounts of certain substances that could suggest the presence of cancer, such as chromogranin A. […] Scope procedures: An endoscope (thin instrument shaped like a tube with a light and lens) is inserted through the mouth and down into the stomach or small intestine to examine tissue and sometimes remove samples. A colonoscopy is a similar procedure, but the scope enters through the rectum. Ultrasound may be used with an endoscope to form pictures of body tissues (endosonography). […] Capsule endoscopy: The person swallows a capsule that contains a small camera in order to take pictures of the gastrointestinal tract.

• #2 Carcinoid Syndrome: Symptoms, Diagnosis and Treatment

  https://www.cancercenter.com/risk-factors/carcinoid-syndrome

  Endoscopy and bronchoscopy each use a thin, lighted tube with a camera on the end to peer inside the body. An endoscopy gives doctors a look at the GI tract and a bronchoscopy checks out the lungs. Endoscopic ultrasound may facilitate location and sampling of these tumors. […] Biopsy involves removing a small tissue sample to be examined under a microscope to confirm the diagnosis.

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

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

  Biochemical tests can play a role in making the diagnosis, particularly for functional neuroendocrine tumors. Measurement of specific biomarkers such as CgA or 5-hydroxyindoleacetic (5-HIAA) in blood or urine can help establish the diagnosis. However, testing for CgA or 5-HIAA is not definitive because elevated CgA levels can be seen in patients who use proton pump inhibitors and have certain health conditions, and many foods and drugs can affect 5-HIAA levels. […] Confirmatory diagnosis is achieved by histopathological examination of biopsy samples, where the tumor is graded and classified based on its cellular characteristics and mitotic activity, which provides critical information for treatment planning.

• #2 Guidelines for the diagnosis and management of carcinoid tumours. Part 1: The gastrointestinal tract. A statement from a Canadian National Carcinoid Expert Group

  https://pmc.ncbi.nlm.nih.gov/articles/PMC1891174/

  Levels of CgA tend to correlate with tumour bulk but not with symptoms. […] Scintigraphy using 111In-pentetreotide is one of the most important imaging investigations for identifying and staging carcinoid tumours of the gastrointestinal tract. […] All patients should have an OctreoScan as a baseline, and patients who have undergone curative surgery should have an annual OctreoScan, unless clinical conditions indicate otherwise. […] The main goals of carcinoid tumour management are symptom control, biochemical control (that is, normalizing the 5-hiaa level), objective tumour control, and quality-of-life improvement. […] Somatostatin analogues for symptom control, frequent debulking, and better management of cardiac complications have improved survival rates. […] Surgery is the only way to obtain a complete cure. […] Patients with carcinoid syndrome should have an echocardiogram at diagnosis. […] All patients should have an annual echocardiogram and follow-up with a cardiologist, particularly those with any cardiac changes or with elevated 5-hiaa.

• #2 Carcinoid Tumours: Causes, Symptoms and Treatment | Doctor

  https://patient.info/doctor/carcinoid-tumours

  False negatives may occur with various drugs – eg, alcohol, aspirin, antidepressants, St. John’s wort. […] Other baseline investigations, considering possible associated neuroendocrine neoplasia or bowel adenocarcinoma, include FBC, LFTs, TFTs, parathyroid hormone, calcium, calcitonin, prolactin. alpha-fetoprotein, carcinoembryonic antigen (CEA) and beta-hCG. […] Provocative tests (eg, pentagastrin test) may be considered if other screening test results are equivocal; however, close monitoring and ready access to intravenous somatostatin are essential in case of a carcinoid crisis. […] Gastric and intestinal tumours may be diagnosed by endoscopy or endoscopic ultrasound; barium studies may demonstrate polyps. […] CT or MRI scanning and laparotomy may be needed for localisation. […] Scintigraphic imaging with labelled somatostatin can provide accurate information on the site and dissemination of the tumour. […] Somatostatin single-photon emission computerised tomography (SPECT) may also be used.

• #2 Late-stage diagnosis of carcinoid heart disease due to lack of access to health care | Cardio-Oncology | Full Text

  https://cardiooncologyjournal.biomedcentral.com/articles/10.1186/s40959-023-00176-z

  Most cases of CS are difficult to diagnose because of the rarity of the condition and the non-specific symptoms it presents with. One study showed that diagnosis of CS can be delayed by up to 7 years. Unfortunately, in our patients case, her diagnosis was even further delayed, potentially by up to 12 years. Though diagnosis of CS is challenging, this patients delayed diagnosis was likely exacerbated by poor access to the US healthcare system due to her uninsured status. Once the diagnosis of CS is made, prompt treatment with somatostatin analogues, most commonly octreotide, should be initiated. Delayed diagnosis of CS is thought to increase chances of developing CHD because it results in prolonged myocardial exposure to vasoactive substances, specifically serotonin, that induce cardiac valvular fibrosis. Regular screening for CHD should also be performed in all patients with CS. Guidelines recommend screening with TTE annually for patients without cardiac involvement and semiannually for those with cardiac involvement. TTE is also the gold standard for diagnosis of CHD. Once CHD is diagnosed, patients should be evaluated every 6-12 months for progression with TTE, NT-pro-BNP, chromogranin A, and urinary 5-HIAA levels to assess for valvular disease, heart failure, and recurrence or worsening of carcinoid malignancy.

• #2

  https://link.springer.com/article/10.1007/s00261-019-01945-0

  To determine the rate of missed CT findings of ileal carcinoid tumor prior to pathologic diagnosis and the resultant diagnostic delay. […] Primary ileal tumors were prospectively missed on at least one CT scan in 64% (32/50) of patients with retrospectively identifiable disease. […] Initial presentation of ileal carcinoid tumor, even with mesenteric involvement, is often missed prospectively at abdominal CT, leading to delay in diagnosis until bowel or mesenteric findings become more obvious, or hepatic metastatic disease manifests. […] Radiologists should make a concerted effort to evaluate the bowel and mesentery in patients with long-standing vague abdominal symptoms.

• #3 SciELO Brazil – Carcinoid syndrome: diagnosis and medical management Carcinoid syndrome: diagnosis and medical management

  https://www.scielo.br/j/abem/a/XsNGMwcFV8D6R6Lk5ZLR78j/?format=html

  Chromogranin A (CgA) is a member of the chromogranin family, which is stored in the secretory granules of neuroendocrine cells. Because CgA is a constitutive secretory product of most NETs, its detection in plasma can be utilized as a general tumor marker for carcinoids and even for „non-functioning” tumors. In carcinoid tumors, the highest concentrations of CgA were noted in metastatic midgut lesions with CgA elevation in 87% of lesions, whereas 5-HIAA increases was noted in 76%. CgA concentration correlated with tumor burden. Plasma CgA levels are sensitive but nonspecific markers of carcinoid tumors because they are also elevated in pancreatic NETs, as well as in other types of NETs. False-positive increased CgA concentrations can be seen in renal impairment, liver failure, atrophic gastritis, and inflammatory bowel disease. If biochemical results are equivocal, these tests should be repeated and plasma CgA measured because it is the most sensitive and reliable screening test. […] If one of the peptides/amines or its breakdown products are initially elevated, the precise localization of the primary lesion and its metastases should be undertaken, starting with 111In-labelled pentetreotide scintigraphy.

• #3 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

  Diagnosing carcinoid syndrome is done by evaluating clinical symptoms, measuring biochemical markers, and using imaging studies to look for neuroendocrine tumors and metastasis. […] If carcinoid syndrome is suspected based on symptoms and clinical presentation, initial testing for biochemical markers is carried out. […] The initial diagnostic test is usually a twenty-four-hour urine test measuring 5-HIAA. […] If these biomarkers are elevated, imaging studies are usually carried out to pinpoint the specific location of the carcinoid tumor. […] There are many challenges in identifying carcinoid syndrome. These types of neuroendocrine tumors can be difficult to locate and recognize since they often result in nonspecific symptoms and a variable course of the disease.

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