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Duodenal Carcinoid Syndrome

Duodenal carcinoid syndrome (DCS) refers to a complex of cardiovascular, respiratory, and gastrointestinal symptoms triggered by a functional neuroendocrine tumor developing in the proximal part of the small intestine. These symptoms may be associated with gastrointestinal complaints suggestive of local mass effects. The complete resection of the duodenal carcinoid and existing metastases is the preferred approach to therapy, but it may not be feasible in advanced stages of the disease. Here, palliative treatment and alleviation of DCS-related symptoms may be achieved with somatostatin analogs.


Presentation

DCS refers to the sum of systemic symptoms provoked by the mediators released by tumor cells in the duodenum. These symptoms may include gastrointestinal complaints, but they don't allow for the localization of the pathological process to the duodenum. Indeed, DCS patients frequently report diarrhea. Numerous episodes of watery stools are not uncommon and may cause dehydration and weight loss [1]. Additionally, patients may describe episodic hot flushes. These may or may not involve the whole body, but frequently affect the face, neck, and thorax [2]. Mediators released by tumor cells may induce cardiac damage, namely tricuspid and pulmonary insufficiency, as well as endomyocardial fibrosis. Patients may thus present with heart murmurs and signs of right-sided heart failure [3]. Respiratory symptoms may also be observed. Bronchoconstriction is often induced and may lead to a dry cough [2].

Besides DCS, affected individuals may describe upper abdominal pain, presumably attributable to the primary tumor in the duodenum or any of its metastases [4]. Duodenal carcinoids may also lead to gastrointestinal hemorrhages, melena, and anemia [5]. Non-specific gastrointestinal symptoms related to duodenal carcinoids comprise nausea, vomiting, gastroesophageal reflux, bloating, and dyspepsia [1] [6]. If the bile duct is obstructed, patients may develop jaundice.

Pain
  • The right upper quadrant abdominal pains accompanied by symptoms that resemble even part of the carcinoid syndrome should initiate the investigational process including proximal gastrointestinal endoscopy to avoid delay in diagnosis of duodenal carcinoid[ncbi.nlm.nih.gov]
  • We report a rare case of duodenal carcinoid presenting as a carcinoid syndrome in a middle-aged man with upper abdominal pain, hot flushes, diarrhoea and dry cough.[ncbi.nlm.nih.gov]
  • Carcinoids are slow-growing neoplasms and although they rarely interfere with the intestinal transit, they may induce epigastric pain.[symptoma.com]
  • Chest Pain Chest pain is a common complaint by a patient in the ER. Causes of chest pain include broken or bruised ribs, pleurisy,...[medicinenet.com]
  • Dyspepsia refers to acute, chronic, or recurrent pain or discomfort centered in the upper abdomen.[accessmedicine.mhmedical.com]
Weight Loss
  • Numerous episodes of watery stools are not uncommon and may cause dehydration and weight loss. Additionally, patients may describe episodic hot flushes. These may or may not involve the whole body, but frequently affect the face, neck, and thorax.[symptoma.com]
  • Before undergoing clinical work-up and diagnosing the NET, the patient related an almost 12 month history of increasing diarrhea (up to 15 bowel movements per day) with weight loss of 11 kg.[bmcinfectdis.biomedcentral.com]
  • Enteral feedings should be considered in patients with repeated hospitalizations for refractory symptoms or patients with greater than 10% weight loss in 3-6 months.[endocrinologyadvisor.com]
  • The symptoms vary from vague abdominal discomfort or severe abdominal pain, diarrhoea and weight loss.[intechopen.com]
Asymptomatic
  • Most patients remain asymptomatic, though, and their tumors are detected incidentally during endoscopic examinations realized for other reasons. Few patients with duodenal carcinoids develop what is referred to as duodenal carcinoid syndrome (DCS).[symptoma.com]
  • Infection with Giardia duodenalis may remain asymptomatic or cause acute or chronic diarrhoea.[intechopen.com]
  • They can be asymptomatic or cause nonspecific symptoms, which usually leads to a delayed diagnosis. Imaging modalities can aid diagnosis and surgery remains the mainstay of treatment.[ncbi.nlm.nih.gov]
  • Hepatic: Most patients with non-alcoholic fatty liver disease are asymptomatic until the end stages of the disease.[endocrinologyadvisor.com]
  • Patients may also be asymptomatic, and the diagnosis can be accidental ( 5, 8, 15 ). The neuroendocrine markers, chromogranin A and 5-HIAA, in serum and urine, respectively, may be useful for the diagnosis ( 2, 15, 18 ).[scielo.br]
Swelling
  • Hereditary Angioedema (HAE) Hereditary angioedema or HAE is a genetic disease that causes swelling of the skin and tissues beneath it. Symptoms of HAE...[medicinenet.com]
  • Those features cause heart failure with swelling of the feet and distention of the neck veins, which pulsate.[carcinoid.org]
  • .  In clinical trials, the most frequent adverse event was injection-site reaction, consisting of mild or moderate swelling, redness, and itching.[slideshare.net]
Congestive Heart Failure
  • In later stages, carcinoid syndrome may damage the heart valves, resulting in symptoms of congestive heart failure.[rarediseases.org]
  • Caution in interpreting TE measurements should be paid in patients with aminotransferases flares, with congestive heart failure and with extrahepatic cholestasis. reviews VĂŠrszegĂŠnysĂŠg ĂŠs kezelĂŠse gyulladĂĄsos bĂŠlbetegekben GyulladĂĄsos bĂŠlbetegsĂŠgben[ceu-jgh.org]
Cough
  • We report a rare case of duodenal carcinoid presenting as a carcinoid syndrome in a middle-aged man with upper abdominal pain, hot flushes, diarrhoea and dry cough.[ncbi.nlm.nih.gov]
  • Bronchoconstriction is often induced and may lead to a dry cough. Besides DCS, affected individuals may describe upper abdominal pain, presumably attributable to the primary tumor in the duodenum or any of its metastases.[symptoma.com]
  • Those caused by the tumour may include abdominal pain, anemia, pneumonia, cough and haemoptysis (cough productive of blood). Carcinoid tumors can also be present without producing any symptoms and may often go undetected for a long period of time.[rarediseases.org]
  • Q Fever Q fever is a highly infectious disease that causes high fever, diarrhea, cough, and sweating. Infected animals may transmit Q...[medicinenet.com]
  • Patients with pulmonary infections may have cough, tachypnea, dyspnea, retraction, accessory muscle use, hypoxia, or abnormal breath sound on auscultation. Lymphadenopathy may be present. Jaundice, pruritus, and hepatomegaly may be present.[slideshare.net]
Cough
  • We report a rare case of duodenal carcinoid presenting as a carcinoid syndrome in a middle-aged man with upper abdominal pain, hot flushes, diarrhoea and dry cough.[ncbi.nlm.nih.gov]
  • Bronchoconstriction is often induced and may lead to a dry cough. Besides DCS, affected individuals may describe upper abdominal pain, presumably attributable to the primary tumor in the duodenum or any of its metastases.[symptoma.com]
  • Those caused by the tumour may include abdominal pain, anemia, pneumonia, cough and haemoptysis (cough productive of blood). Carcinoid tumors can also be present without producing any symptoms and may often go undetected for a long period of time.[rarediseases.org]
  • Q Fever Q fever is a highly infectious disease that causes high fever, diarrhea, cough, and sweating. Infected animals may transmit Q...[medicinenet.com]
  • Patients with pulmonary infections may have cough, tachypnea, dyspnea, retraction, accessory muscle use, hypoxia, or abnormal breath sound on auscultation. Lymphadenopathy may be present. Jaundice, pruritus, and hepatomegaly may be present.[slideshare.net]
Dyspnea
  • Headache, chills, flushing, chest tightness, dyspnea, fever, myalgia, nausea, and fatigue may begin at the end of the infusion and continue for several hours.[slideshare.net]
  • […] lesions, fibrosis of the endocardium; may lead to heart failure with tachycardia and hypertension Wheezing or asthmalike syndrome (25%): Due to bronchial constriction; some tremors are relatively indolent and result in chronic symptoms such as cough and dyspnea[emedicine.medscape.com]
Dry Cough
  • We report a rare case of duodenal carcinoid presenting as a carcinoid syndrome in a middle-aged man with upper abdominal pain, hot flushes, diarrhoea and dry cough.[ncbi.nlm.nih.gov]
  • Bronchoconstriction is often induced and may lead to a dry cough. Besides DCS, affected individuals may describe upper abdominal pain, presumably attributable to the primary tumor in the duodenum or any of its metastases.[symptoma.com]
Dyspnea
  • Headache, chills, flushing, chest tightness, dyspnea, fever, myalgia, nausea, and fatigue may begin at the end of the infusion and continue for several hours.[slideshare.net]
  • […] lesions, fibrosis of the endocardium; may lead to heart failure with tachycardia and hypertension Wheezing or asthmalike syndrome (25%): Due to bronchial constriction; some tremors are relatively indolent and result in chronic symptoms such as cough and dyspnea[emedicine.medscape.com]
Diarrhea
  • Indeed, DCS patients frequently report diarrhea. Numerous episodes of watery stools are not uncommon and may cause dehydration and weight loss. Additionally, patients may describe episodic hot flushes.[symptoma.com]
  • Diarrhea, Chronic is a topic covered in the 5-Minute Clinical Consult.[unboundmedicine.com]
  • Factors Contributing to Diabetic Diarrhea Symptoms and Signs: Diabetics with diarrhea report passage of loose and/or frequent stools. Nocturnal symptoms may occur depending on the underlying etiology for the patient’s diarrhea.[endocrinologyadvisor.com]
  • Carcinoid syndrome causes redness or a feeling of warmth in your face and neck (skin flushing), chronic diarrhea, and difficulty breathing, among other signs and symptoms. Carcinoid heart disease.[mayoclinic.org]
  • Interestingly this patient developed mainly watery diarrhea with a little mucus but without blood.[bmcinfectdis.biomedcentral.com]
Abdominal Pain
  • The right upper quadrant abdominal pains accompanied by symptoms that resemble even part of the carcinoid syndrome should initiate the investigational process including proximal gastrointestinal endoscopy to avoid delay in diagnosis of duodenal carcinoid[ncbi.nlm.nih.gov]
  • We report a rare case of duodenal carcinoid presenting as a carcinoid syndrome in a middle-aged man with upper abdominal pain, hot flushes, diarrhoea and dry cough.[ncbi.nlm.nih.gov]
  • Besides DCS, affected individuals may describe upper abdominal pain, presumably attributable to the primary tumor in the duodenum or any of its metastases. Duodenal carcinoids may also lead to gastrointestinal hemorrhages, melena, and anemia.[symptoma.com]
  • Persisting abdominal pain should be evaluated by a physician.[medicinenet.com]
  • Miscellaneous GI Complications of Diabetes Abdominal Pain: Some diabetics present with upper abdominal pain due to neuropathy or radiculopathy of nerves exiting from the thoracic spinal cord.[endocrinologyadvisor.com]
Abdominal Pain
  • The right upper quadrant abdominal pains accompanied by symptoms that resemble even part of the carcinoid syndrome should initiate the investigational process including proximal gastrointestinal endoscopy to avoid delay in diagnosis of duodenal carcinoid[ncbi.nlm.nih.gov]
  • We report a rare case of duodenal carcinoid presenting as a carcinoid syndrome in a middle-aged man with upper abdominal pain, hot flushes, diarrhoea and dry cough.[ncbi.nlm.nih.gov]
  • Besides DCS, affected individuals may describe upper abdominal pain, presumably attributable to the primary tumor in the duodenum or any of its metastases. Duodenal carcinoids may also lead to gastrointestinal hemorrhages, melena, and anemia.[symptoma.com]
  • Persisting abdominal pain should be evaluated by a physician.[medicinenet.com]
  • Miscellaneous GI Complications of Diabetes Abdominal Pain: Some diabetics present with upper abdominal pain due to neuropathy or radiculopathy of nerves exiting from the thoracic spinal cord.[endocrinologyadvisor.com]
Vomiting
  • Non-specific gastrointestinal symptoms related to duodenal carcinoids comprise nausea, vomiting, gastroesophageal reflux, bloating, and dyspepsia. If the bile duct is obstructed, patients may develop jaundice.[symptoma.com]
  • May be associated epigastric fullness, nausea, heartburn, or vomiting. Endoscopy is warranted in all patients age 60 years or older and selected younger patients with alarm features.[accessmedicine.mhmedical.com]
  • Vomiting typically occurs 30-60 minutes after eating but may occur up to 8 hours after oral intake.[endocrinologyadvisor.com]
  • ) Cholelithiasis ( English Spanish ) Cirrhosis of the Liver Cirrhosis, Primary Biliary Clostridium Difficile Infection Colitis, Ischemic Colonic Polyps Congenital Megacolon (Hirschsprung Disease) Constipation ( English Spanish ) Crohn Disease Cyclic Vomiting[5minuteconsult.com]
  • Nausea and vomiting after embolization can arise from the emetogenic potential of pain or from adynamic ileus, which may be compounded by concurrent opioid use.[ncbi.nlm.nih.gov]
Dyspepsia
  • Functional dyspepsia refers to dyspepsia for which no organic etiology has been determined by endoscopy or other testing. This is the most common cause of chronic dyspepsia, accounting for the majority of patients.[accessmedicine.mhmedical.com]
  • Non-specific gastrointestinal symptoms related to duodenal carcinoids comprise nausea, vomiting, gastroesophageal reflux, bloating, and dyspepsia. If the bile duct is obstructed, patients may develop jaundice.[symptoma.com]
  • Food and Digestion Aerophagy Nervous Dyspepsia Anorexia Nervosa X-ray Negative Dyspepsia (Non-Ulcer Dyspepsia) Chapter X The Gall-Bladder and Pancreas Incidence of Gall-Stones Biliary Colic Acute Cholecystitis Chronic Cholecystitis Motility Disturbances[elsevier.com]
  • Ischemic Colonic Polyps Congenital Megacolon (Hirschsprung Disease) Constipation ( English Spanish ) Crohn Disease Cyclic Vomiting Syndrome Diarrhea, Acute ( English Spanish ) Diarrhea, Chronic Diverticular Disease ( English Spanish ) Dumping Syndrome Dyspepsia[5minuteconsult.com]
  • The most common symptoms are abdominal pain, gastroesophageal reflux, dyspepsia, and gastrointestinal bleeding ( 8, 12 ). The predominant symptoms in the present case series were dyspepsia and epigastric pain.[scielo.br]
Jaundice
  • If the bile duct is obstructed, patients may develop jaundice. In the absence of upper gastrointestinal symptoms, laboratory analyses of blood and urine samples may yield first hints at the primary disorder.[symptoma.com]
  • Jaundice, pruritus, and hepatomegaly may be present. Oral mucosal and perirectal ulcerations may be present, especially in patients with concomitant neutropenia. Prof Ariyanto Harsono MD PhD SpA(K) 23 24.[slideshare.net]
  • A Konferencia elnĂśki feladatait HerszĂŠnyi LĂĄszlĂł lĂĄtta el. reviews Autoimmune pancreatitis - Recent advances Autoimmune pancreatitis (AIP) is a form of chronic pancreatitis, with frequent and typical clinical symptoms, including obstructive jaundice[ceu-jgh.org]
Heart Failure
  • Patients may thus present with heart murmurs and signs of right-sided heart failure. Respiratory symptoms may also be observed. Bronchoconstriction is often induced and may lead to a dry cough.[symptoma.com]
  • This can lead to leaky heart valves and heart failure that may require valve-replacement surgery. Carcinoid heart disease can usually be controlled with medications. Cushing syndrome.[mayoclinic.org]
  • In later stages, carcinoid syndrome may damage the heart valves, resulting in symptoms of congestive heart failure.[rarediseases.org]
  • Those features cause heart failure with swelling of the feet and distention of the neck veins, which pulsate.[carcinoid.org]
  • Read More BNP (Blood) This test measures the amount of a protein that builds up in your blood when you have heart failure. It's an important tool for doctors to diagnose heart failure quickly.[hackensackumc.org]
Heart Disease
  • Patients diagnosed with duodenal carcinoids should undergo a thorough cardiological examination to evaluate the degree of DCS-related heart disease. In case of a single, resectable duodenal carcinoid, the complete removal of the tumor is sought.[symptoma.com]
  • Carcinoid heart disease can usually be controlled with medications. Cushing syndrome. A lung carcinoid tumor can produce an excess of a hormone that can cause your body to produce too much of the hormone cortisol. Dec. 12, 2018[mayoclinic.org]
  • A high level of this cholesterol may mean you have heart disease or another cholesterol-related condition.[hackensackumc.org]
  • Surgical pathology of carcinoid heart disease: a study of 139 valves from 75 patients spanning 20 years. Mayo Clin Proc. 2002;77:139-47. Kulaksiz H, Eissele R, Rossler D, et al.[rarediseases.org]
  • Or, in the heart, the valves of the heart become scarred by the deposits of this fibrous tissue, causing carcinoid valvular heart disease.[carcinoid.org]
Hypotension
  • Furthermore, it is possible that our definition of carcinoid crisis as hypotension was too narrow during the initial review of periembolization hemodynamic parameters, in that some of the observed postprocedural hypertensive episodes may have signified[ncbi.nlm.nih.gov]
  • The symptoms of carcinoid syndrome include episodes of warmth and redness of the face, head and upper chest; diarrhea; marked changes in blood pressure (usually hypotension, a decrease in blood pressure); asthmatic-like wheezing; weight loss or gain;[rarediseases.org]
  • Prof Ariyanto Harsono MD PhD SpA(K) 47 48. o Prekallikrein activator has been associated with hypotension and circulatory collapse, and IgG aggregates may result in anaphylaxis via complement activation. o In patients with XHIGM, production of IgE antibodies[slideshare.net]
Heart Murmur
  • Patients may thus present with heart murmurs and signs of right-sided heart failure. Respiratory symptoms may also be observed. Bronchoconstriction is often induced and may lead to a dry cough.[symptoma.com]
  • We said that suspicion comes first, then examining the patient, feeling a lump or hearing a heart murmur, or seeing a flush. Then, after that, we look at the markers.[carcinoid.org]
Flushing
  • We report a rare case of duodenal carcinoid presenting as a carcinoid syndrome in a middle-aged man with upper abdominal pain, hot flushes, diarrhoea and dry cough.[ncbi.nlm.nih.gov]
  • Additionally, patients may describe episodic hot flushes. These may or may not involve the whole body, but frequently affect the face, neck, and thorax.[symptoma.com]
  • Carcinoid syndrome causes redness or a feeling of warmth in your face and neck (skin flushing), chronic diarrhea, and difficulty breathing, among other signs and symptoms. Carcinoid heart disease.[mayoclinic.org]
  • This is an acute carcinoid flush. It disappeared within a matter of a minute or two. And here he is, now, feeling obviously much better, but you see residual mask-like flush of which he is not even aware.[carcinoid.org]
  • The occurrence of episodic facial flushing and/or chronic diarrhea not diagnosed by standard tests as being a result of more common causes should lead to suspicion of carcinoid syndrome.[rarediseases.org]
Flushing
  • We report a rare case of duodenal carcinoid presenting as a carcinoid syndrome in a middle-aged man with upper abdominal pain, hot flushes, diarrhoea and dry cough.[ncbi.nlm.nih.gov]
  • Additionally, patients may describe episodic hot flushes. These may or may not involve the whole body, but frequently affect the face, neck, and thorax.[symptoma.com]
  • Carcinoid syndrome causes redness or a feeling of warmth in your face and neck (skin flushing), chronic diarrhea, and difficulty breathing, among other signs and symptoms. Carcinoid heart disease.[mayoclinic.org]
  • This is an acute carcinoid flush. It disappeared within a matter of a minute or two. And here he is, now, feeling obviously much better, but you see residual mask-like flush of which he is not even aware.[carcinoid.org]
  • The occurrence of episodic facial flushing and/or chronic diarrhea not diagnosed by standard tests as being a result of more common causes should lead to suspicion of carcinoid syndrome.[rarediseases.org]
Suggestibility
  • These symptoms may be associated with gastrointestinal complaints suggestive of local mass effects.[symptoma.com]
  • Recent report also suggests that this parasite alters chloride secretory response in human colonic cells in vitro, as well as in murine models [ 7 ].[intechopen.com]
  • Symptoms suggestive of an organic disease include a history of diarrhoea of 18 and a normal physical examination, are suggestive of a functional bowel disturbance, but only with a specificity of approximately 52–74%. 19 Unfortunately, these criteria do[gut.bmj.com]
  • Suggested algorithm for postembolization management. Abbreviations: p.o., orally; prn, as needed.[ncbi.nlm.nih.gov]
  • The gastroparesis diet requires 4-6 small, low fat, low fiber meals each day (an inclusive list of suggested foods for patients can be found at ).[endocrinologyadvisor.com]
Hot Flushes
  • We report a rare case of duodenal carcinoid presenting as a carcinoid syndrome in a middle-aged man with upper abdominal pain, hot flushes, diarrhoea and dry cough.[ncbi.nlm.nih.gov]
  • Additionally, patients may describe episodic hot flushes. These may or may not involve the whole body, but frequently affect the face, neck, and thorax.[symptoma.com]

Workup

In the absence of upper gastrointestinal symptoms, laboratory analyses of blood and urine samples may yield first hints at the primary disorder. If mediators are released by tumor cells, these mediators (or their metabolites) can be detected in body fluids. Their concentrations are likely to exceed the respective reference ranges. 5-hydroxyindoleacetic acid, for instance, is the end product of serotonin metabolism and is excreted by DCS patients if the primary tumor produces serotonin. Increased levels of urinary 5-hydroxyindoleacetic acid have a 100% specificity for carcinoid tumors [3]. Of note, alterations of hepatic parameters may be due to metastases in the liver or may be caused by obstructive cholestasis.

Diagnostic imaging is the most important tool to confirm the tentative diagnosis of a gastrointestinal carcinoid, to assess whether there is a single or multiple tumors, whether there metastases in the liver or other organs. Computed tomography is most commonly used to this end, but sonography may yield conclusive findings, too. Scintigraphy using isotopes that bind to the receptor of neuroendocrine cells is another technique with high sensitivity for the detection of duodenal carcinoids, regardless of their functionality [3]. In symptomatic patients, multiple tumors are a rather common finding [2]. They are typically spread through the duodenum and more distal parts of the intestine. In one of eight patients, distant metastases are present at the time of diagnosis, and the share of patients with advanced-stage disease may be even higher among those presenting with DCS: Metastases most commonly form in the liver and bones, so biologically active substances that had previously been inactivated in the liver are now released into the systemic circulation without prior metabolization [3].

After the tumor has been localized to the duodenum, it may be visualized and accessed endoscopically. Surgeons may opt for a standard biopsy or choose to perform an excisional biopsy to obtain tissue samples [1]. These specimens are subjected to a histopathological examination. Tumor cells are monomorphic, have rounded nuclei and abundant cytoplasm. They are arranged in a trabecular or cribriform pattern. Necrotic foci are not usually seen, and the mitotic rate is low [5]. Carcinoids test positive for chromogranin A, the most valuable marker of neuroendocrine tumors [7]. Staining for neuron-specific enolase and synaptophysin [1].

Patients diagnosed with duodenal carcinoids should undergo a thorough cardiological examination to evaluate the degree of DCS-related heart disease [3].

Serotonin Increased
  • Increased levels of urinary 5-hydroxyindoleacetic acid have a 100% specificity for carcinoid tumors. Of note, alterations of hepatic parameters may be due to metastases in the liver or may be caused by obstructive cholestasis.[symptoma.com]

Treatment

In case of a single, resectable duodenal carcinoid, the complete removal of the tumor is sought. In general, endoscopic mucosal resection is performed if the tumor measures <1 cm in diameter and hasn't invaded the muscular layer [5]. Transduodenal resection, possibly combined with regional lymphadenectomy, is recommended in case of solitary tumors measuring >1 cm in diameter [1] [2] [6]. Depending on their localization and size, multiple tumors may be removed in a similar manner, or Whipple's pancreaticoduodenectomy and regional lymphadenectomy may be performed [8]. Hepatic metastases may be treated by partial liver resection, radiofrequency ablation, cryoablation, and/or embolization.

If the patient isn't eligible for surgery or the resection of the primary tumor and its metastases isn't feasible, cytoreductive surgery plus pharmacological treatment should be considered. Long-term symptom control is feasible with somatostatin analogs, although these drugs don't affect tumor growth [3] [9]. Octreotide and lanreotide are most widely used to alleviate DCS-related symptoms, with immediate-release formulations being used thrice daily at the beginning of therapy. Once tolerability and efficacy are established, the patients are switched to long-acting formulations, which are injected intramuscularly once a month [3]. Other agents, such as interferon alfa and antihistaminics, should be applied as needed. The efficacy of systemic chemotherapy and radiotherapy has not been proven in DCS patients, so these treatments should be reserved for those with progressive disease and multiple metastases [2] [3]. In any case, medical therapy should be supported by maximal, possibly repeated debulking [3].

Regular assessments of the urinary excretion of 5-hydroxyindoleacetic acid and plasma levels of chromogranin A may help to evaluate the patient's response to therapy and to predict an imminent relapse [3] [7].

Prognosis

DCS may be fatal due to severe cardiac damage and right-sided heart failure, or due to a carcinoid crisis. Such a crisis may be triggered by drugs, especially by anesthetics, or by manipulation of the tumor during surgery. As a result, large quantities of biologically active substances are released from the tumor, and life-threatening drops in blood pressure may occur [2].

Duodenal carcinoids may be benign or malignant tumors. The latter tend to grow invasively, to spread to the regional lymph nodes and to form hematogenic metastases in distant organs. The distinction of benign and malignant carcinoids may pose a major challenge but is essential to the prognosis. Additionally, high plasma concentrations of chromogranin A have been related to a worse outcome and may indicate a relapse [7]. In sum, even malignant variants of duodenal carcinoids tend to grow slowly, so the prognosis remains favorable if DCS-related symptoms can be controlled. Due to the rarity of duodenal carcinoids, reliable data regarding the outcome are scarce, but for patients diagnosed with any carcinoid, the five-year survival rate is 67% [3]. About 40% of individuals diagnosed with metastatic small intestine carcinoids remain alive after five years [9]. If the neoplasm can be removed in its entirety, the patient has an excellent prognosis [4].

Etiology

DCS comprises the systemic effects of mediators released by neuroendocrine tumor cells, but little is known about the genesis of carcinoid tumors. Multiple endocrine neoplasia type 1 is a hereditary disorder known to predispose to neuroendocrine tumors and a variety of other neoplasms, but it accounts for a small percentage of DCS cases only. On the other hand, duodenal carcinoids are rare manifestations of multiple endocrine neoplasia type 1 [10] [11].

Epidemiology

Duodenal carcinoids are rare and account for <2% of gastrointestinal carcinoid tumors. Considering an overall incidence of carcinoid tumors of approximately 2 per 100,000 inhabitants and year, duodenal carcinoids probably affect about 2-3 per 10,000,000 people a year [3]. DCS is even rarer because the majority of duodenal carcinoids remains non-functional for prolonged periods of time. There are no larger studies, but available case reports describe both male and female patients, most of them in middle and advanced adulthood [2] [4] [5].

Sex distribution
Age distribution

Pathophysiology

Carcinoids are slow-growing tumors, and those that develop in the duodenum are rarely functional. On rare occasions though, duodenal carcinoid tumors produce and release biologically active substances, such as serotonin, histamine, prostaglandins, kallikrein, adrenocorticotropic hormone, gastrin, calcitonin, and growth hormone, among others [3].

  • Serotonin has been shown to cause cardiac damage and to participate in the induction of bronchoconstriction and diarrhea, so it plays a key role in DCS pathogenesis.
  • Histamine is a well-known trigger of hot flushes.
  • The clinical presentation of an index case depends on the hormone release pattern of the specific tumor. The classical carcinoid syndrome is observed in only about 20% of all cases [9].

Interestingly, the synchronous or metachronous development of an adenocarcinoma of the colon is observed in up to 25% of patients presenting with functional carcinoids. Presumably, this is due to the effects of carcinoid-derived growth factors on susceptible cells [9].

Prevention

The causes of duodenal carcinoids remain elusive, so no recommendations can be given to prevent the development of this type of tumor. Factors contributing to the neuroendocrine activity of tumor cells are neither known. In a patient diagnosed with duodenal carcinoids, however, treatment with somatostatin analogs may alleviate systemic symptoms and prevent the manifestation of DCS. Similarly, the pre-surgical administration of somatostatin analogs like octreotide reduces the risk of a carcinoid crisis during tumor manipulation [3].

Summary

Carcinoids are uncommon, well-differentiated neuroendocrine tumors. About two-thirds of carcinoids develop in the gastrointestinal tract, although the duodenum is rarely affected [3]. Regardless of their location, these tumors may produce hormones and hormone-like mediators that exert systemic effects. The entirety of these effects is referred to as carcinoid syndrome. If it is caused by a duodenal neoplasm, the patient may be diagnosed with DCS. When DCS manifests, the patient has probably been living with the primary tumor for more than five years [9]. It should be noted that functionality does not allow for a reliable conclusion regarding the grade of the tumor, so DSC may be associated with benign or malignant processes [3]. Both the diagnosis of a duodenal carcinoid and an assessment of its malignancy require the histopathological examination of tissue samples.

Patient Information

Carcinoids are tumors deriving from cells that are able to produce and release hormones and hormone-like mediators; they are so-called neuroendocrine tumors. They may develop in distinct organ systems but are most frequently found in the gastrointestinal tract. Accordingly, they may also develop in the duodenum, the most proximal part of the small intestine. Carcinoids are slow-growing neoplasms and although they rarely interfere with the intestinal transit, they may induce epigastric pain. Most patients remain asymptomatic, though, and their tumors are detected incidentally during endoscopic examinations realized for other reasons.

Few patients with duodenal carcinoids develop what is referred to as duodenal carcinoid syndrome (DCS). This term describes a complex of systemic complaints, namely hot flushes, cardiac disease, respiratory difficulties, and diarrhea, which are triggered by the biologically active substances released by the tumor. If these substances reach the circulation, they can affect the function of distant organs and tissues. This can be a greater burden for the patients than the underlying disease, and DCS may significantly reduce their quality of life. Indeed, DCS-associated cardiac disease may have fatal consequences.

In an ideal scenario, where the whole tumor can be removed in a surgical intervention, cure of DCS and duodenal carcinoids is feasible. The surgical removal of the tumor poses a major challenge, though, if metastases have formed in the liver, bones, or other organs. DCS patients with metastatic disease are usually offered pharmacological treatment. They are administered so-called somatostatin analogs, which impair the production of hormones by the tumors. Because duodenal carcinoids and metastatic tumors follow a slowly progressive course, their prognosis is favorable.

References

Article

  1. Waisberg J, Joppert-Netto G, Vasconcellos C, Sartini GH, Miranda LS, Franco MI. Carcinoid tumor of the duodenum: a rare tumor at an unusual site. Case series from a single institution. Arq Gastroenterol. 2013; 50(1):3-9.
  2. Naalla R, Konchada K, Kannappan O, Lingadakai R. Duodenal carcinoid with carcinoid syndrome. BMJ Case Rep. 2014; 2014.
  3. Maroun J, Kocha W, Kvols L, et al. Guidelines for the diagnosis and management of carcinoid tumours. Part 1: the gastrointestinal tract. A statement from a Canadian National Carcinoid Expert Group. Curr Oncol. 2006; 13(2):67-76.
  4. Matsumoto S, Miyatani H, Yoshida Y, Nokubi M. Duodenal carcinoid tumors: 5 cases treated by endoscopic submucosal dissection. Gastrointest Endosc. 2011; 74(5):1152-1156.
  5. Abraham A, Singh J, Siddiqui G, et al. Endoscopic management of a primary duodenal carcinoid tumor. Case Rep Gastroenterol. 2012; 6(1):135-142.
  6. Zyromski NJ, Kendrick ML, Nagorney DM, et al. Duodenal carcinoid tumors: how aggressive should we be? J Gastrointest Surg. 2001; 5(6):588-593.
  7. Gut P, Czarnywojtek A, Fischbach J, et al. Chromogranin A - unspecific neuroendocrine marker. Clinical utility and potential diagnostic pitfalls. Arch Med Sci. 2016; 12(1):1-9.
  8. Hartel M, Wente MN, Sido B, Friess H, Büchler MW. Carcinoid of the ampulla of Vater. J Gastroenterol Hepatol. 2005; 20(5):676-681.
  9. Gustafsson BI, Kidd M, Modlin IM. Neuroendocrine tumors of the diffuse neuroendocrine system. Curr Opin Oncol. 2008; 20(1):1-12.
  10. Sugimoto K, Oosawa S, Furuta T, et al. Multiple endocrine neoplasia type 1 accompanied by duodenal carcinoid tumors and hypergastrinemia: a familial case. Intern Med. 1995; 34(7):649-653.
  11. Yazawa K, Kuroda T, Watanabe H, et al. Multiple carcinoids of the duodenum accompanied by type I familial multiple endocrine neoplasia. Surg Today. 1998; 28(6):636-639.

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Last updated: 2019-07-11 20:22