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Carcinoid Tumors: Cancers in Slow Motion
Ali Ahmad, MD | February 16, 2017
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Adapted images courtesy of Kitchens WH, Elias N, Blaszkowsky LS, Cosimi AB, Hertl M. World J Surg Oncol. 2011;9:11. [Open access.] PMID: 21281518, PMCID: PMC3038967.
Carcinoid Tumors: Cancers in Slow Motion
Ali Ahmad, MD | February 16, 2017 | Contributor Information
Carcinoid tumors ("carcinoids") are the most common neuroendocrine tumors in the United States,[1] with an estimated 12,000 new cases each year,[1-3] representing about 0.5% of all newly diagnosed malignancies.[4] These tumors are derived from primitive stem cells in the gut wall,[5] generally grow slowly ("cancer in slow motion"[3]), and typically affect adults in their 60s.[6,7] There is a slight female preponderance, and the incidence rate is higher in black individuals.[2,3,6,7]
The incidence of carcinoids appears to be increasing, for unknown reasons,[1,3] but the greater use of imaging modalities in other conditions may play a role in the incidental identification of asymptomatic carcinoids.[1,6]
The preoperative abdominal computed tomography (CT) scans in a 60-year-old man with intestinal varices and severe recurrent gastrointestinal (GI) bleeding show obstruction of the superior mesenteric vein by a carcinoid tumor. A large mesenteric soft-tissue mass (arrows) encases the mesenteric vasculature, with evident bowel wall and mesenteric edema, along with trace ascites fluid.
Image courtesy of Cancer Research UK (CRUK) via Wikimedia Commons.
Carcinoid Tumors: Cancers in Slow Motion
Ali Ahmad, MD | February 16, 2017 | Contributor Information
Anatomy
Carcinoid tumors can involve nearly any organ system; however, the GI (approximately two thirds) and pulmonary (about one third) systems are the predominant sites.[1-3,5,8] The thyroid, gallbladder, pancreas, kidney, and gonads (testes, ovaries) may also be affected.[3-5,8]
The following are the most common sites of carcinoid tumors[3]:
Small intestine (39%)
Rectum (15%)
Bronchopulmonary system (10%)
Appendix (7%)
Colon (5%-7%)
Stomach (2%-4%)
Pancreas (2%-3%)
Liver (1%)
Carcinoids are generally classified on the basis of the segment of primitive gut that gave rise to the tissue in which the tumor is located, as follows[4,5]:
Foregut carcinoid tumors (about 20%): Sporadic primary tumors (lung, thymus, stomach, proximal duodenum, pancreas) and tumors secondary to gastric achlorhydria
Midgut carcinoid tumors (about 50%): Second portion of the duodenum; jejunum, ileum, right colon, and appendix
Gross specimen images of multiple small bowel (midgut) carcinoid tumors (top) and a close-up view of a single small bowel carcinoid tumor (bottom inset) courtesy of Ed Uthman, MD.
Carcinoid Tumors: Cancers in Slow Motion
Ali Ahmad, MD | February 16, 2017 | Contributor Information
Etiopathogenesis and Risk Factors
The etiology of carcinoid tumors is unclear, but genetic abnormalities are suspected, including changes in chromosomes such as loss of heterogeneity and numerical imbalances.[5] Certain hereditary syndromes (eg, multiple endocrine neoplasia type 1 [MEN1], neurofibromatosis type 1 [NF1], tuberous sclerosis, von Hippel-Lindau syndrome), and hypergastrinemia are known risk factors for these lesions.[2,5,6,8]
In the intestinal tract, the gradual formation of carcinoids leads to the development of small, firm nodules that bulge into the intestinal lumen; they have a yellow, tan, or gray-brown appearance that can be observed through the intact mucosa.[5] The yellow coloration is a result of cholesterol and lipid accumulation within the tumor. Occasionally, the lesions become ulcerated and may be a source of GI bleeding.[9] Metastases to regional lymph nodes as well as to the liver, lungs, ovaries, peritoneum, and spleen can occur.[10]
Foregut and midgut carcinoids account for approximately 60%-80% of all carcinoid tumors in adults (especially those of the appendix and distal ileum), and they are also occasionally seen in children.[4,5] In the pediatric population, appendiceal carcinoids are the most common; they are often an incidental finding in appendectomy specimens, with more than 70% occurring at the tip of the appendix.[5] Bulky tumors, which are relatively rare in this age group, may require formal resection, such as a right hemicolectomy.[5]
Intraoperative image of a neuroendocrine carcinoma of the mesentery that resulted in ectopic Cushing syndrome courtesy of Fasshauer M, Lincke T, Witzigmann H, et al. BMC Cancer. 2006;6:108. [Open access.] PMID: 16643652, PMCID: PMC1464147.
Carcinoid Tumors: Cancers in Slow Motion
Ali Ahmad, MD | February 16, 2017 | Contributor Information
Carcinoid tumors can also arise from the Meckel diverticulum, cystic duplications, and the mesentery (shown).[5] Each of these entities has distinctive clinical, histochemical, and secretory features. For example, foregut carcinoids have a low serotonin (5-hydroxytryptamine [5-HT]) content, but they secrete 5-hydroxytryptophan (5-HTP), histamine, and several polypeptide hormones. These tumors can metastasize to bone and may be associated with atypical carcinoid syndrome, acromegaly, Cushing disease, other endocrine disorders, telangiectasia, or hypertrophy of the skin in the face and upper neck.[5]
Midgut carcinoids are argentaffin positive and can produce high levels of 5-HT, kinins, prostaglandins, substance P (SP), and other vasoactive peptides.[5] Rarely, these tumors may produce corticotropic hormone (previously adrenocorticotropic hormone [ACTH]). Bone metastasis is uncommon.[5]
Hindgut carcinoid tumors are argentaffin negative and rarely secrete 5-HT, 5-HTP, or any other vasoactive peptides.[5] Consequently, these lesions do not produce related symptomatology. Bone metastasis may occur with hindgut carcinoids.
Image courtesy of Wikimedia Commons/Mikael Häggström.
Carcinoid Tumors: Cancers in Slow Motion
Ali Ahmad, MD | February 16, 2017 | Contributor Information
Presentation
The clinical presentation of carcinoid tumors varies greatly and is dependent on the location and size of the lesions. Although most carcinoids are asymptomatic, up to 20% of patients may develop carcinoid syndrome, which includes the following signs/symptoms[1,4,5,8]:
Cutaneous flushing: Typically of the head and neck
Abdominal pain
Diarrhea: Watery, frothy, or bulky stools
Bronchoconstriction: Wheezing or asthma-like manifestations
Carcinoid heart disease (>33% of patients with carcinoid syndrome): Tricuspid and pulmonic regurgitation, pulmonary stenosis, mitral and aortic insufficiency, cardiac dysrhythmias
Carcinoid syndrome results from the release of 5-HT, histamine, or tachykinins into the systemic circulation.[4,8] Individuals with small-intestine lesions, appendiceal carcinoids, or metastatic disease are particularly affected.[8]
Image courtesy of Wikimedia Commons/James Heilman, MD.
Carcinoid Tumors: Cancers in Slow Motion
Ali Ahmad, MD | February 16, 2017 | Contributor Information
This image shows appendiceal carcinoid tumors.
Images courtesy of Flickr/Yale Rosen.
Carcinoid Tumors: Cancers in Slow Motion
Ali Ahmad, MD | February 16, 2017 | Contributor Information
These images from different patients each demonstrate a large, polypoid, tan carcinoid tumor within the lumen of a major bronchus, which caused bronchial obstruction and obstructive pneumonia.
Image courtesy of Wikimedia Commons/Dr Joachim Guntau.
Carcinoid Tumors: Cancers in Slow Motion
Ali Ahmad, MD | February 16, 2017 | Contributor Information
This endoscopic image shows a carcinoid tumor of the rectum.
Adapted image courtesy of the AJCC.
Carcinoid Tumors: Cancers in Slow Motion
Ali Ahmad, MD | February 16, 2017 | Contributor Information
Classification, Stage, and Grade
The tumor/node/metastasis (TNM) staging classification of the American Joint Committee on Cancer (AJCC) for carcinoid tumors is the same as that for other neuroendocrine tumors.[4,8]
Carcinoids are broadly categorized as follows[3]:
Localized: The tumor has not spread outside the wall of the primary organ
Regional: The tumor has spread from the primary organs to regional tissues (eg, lymph nodes, fat, muscle)
Distant/metastatic: The tumor has spread to other organ sites and tissues
The World Health Organization (WHO) and the European Neuroendocrine Tumor Society (ENETS) both incorporate mitotic count and Ki-67 proliferation index (ie, the percentage of tumor cells expressing Ki-67, a marker of cancer cell growth and division) for the histologic classification of gastroenteropancreatic neuroendocrine tumors (GEP-NETs).[8] For neuroendocrine tumors of the lungs and thymus, the WHO includes mitotic count and assessment of necrosis.[13]
Neuroendocrine tumors are histologically assigned one of three broad grades on the basis of tumor differentiation, as follows[8]:
G1: Well differentiated, low grade
G2: Well differentiated, intermediate grade
G3: Poorly differentiated, high grade
Images courtesy of Senda E, Fujimoto K, Ohnishi K, et al. World J Surg Oncol. 2009;7:9. [Open access.] PMID: 19159493, PMCID: PMC2636813.
Carcinoid Tumors: Cancers in Slow Motion
Ali Ahmad, MD | February 16, 2017 | Contributor Information
In addition to using the site of origin in the primitive gut to classify carcinoids, well-differentiated carcinoid tumors are further subclassified as typical and atypical.[3,4,8] Typical carcinoids in the GI tract (excluding the pancreas) are well-differentiated neuroendocrine tumors; atypical carcinoids are malignant lesions at the same GI sites.[4]
Grading of GEP-NETs with the WHO and ENETS criteria is generally as follows[8]:
G1: Well differentiated, low grade; have fewer than 2 mitoses per 10 high-power field (HPF) and/or less than 3% Ki-67 index
G2: Well differentiated, intermediate grade; have 2-10 mitoses/10 HPF and/or 3%-20% Ki-67 index
G3: Poorly differentiated, high grade; demonstrate over 20 mitoses/10 HPF and/or more than 20% Ki-67 index
Grading of pulmonary (and thymus) carcinoids is as follows[8]:
Typical (G1): Well differentiated, low grade; have fewer than 2 mitoses/10 HPF and no necrosis
Atypical (G2): Well differentiated, intermediate grade; have 2-10 mitoses/10 HPF and/or foci of necrosis
G3: Poorly differentiated, high grade; more than 10 mitoses/10 HPF
The images were obtained from specimens in a patient with a carcinoid tumor at the ampulla of Vater (image a,arrow) in the common bile duct (CBD) and lymph node metastases. The histologic images reveal (b) monotonous tumor cells with small round nuclei (hematoxylin and eosin [H&E] stain; ×400 magnification), (c) carcinoid tumor cells within a peripancreatic lymph node (×200), (d) tumor cells positive for synaptophysin, a neuroendocrine marker (×40), (e) endolymphatic tumor emboli (D2-40 antibody stain; ×400), and (f) positive staining for MIB-1 antibody in approximately 3.2% of the tumor cell nuclei (×400).
Images courtesy of Wang Y, He Q, Shi W, Wang J, Ji H. World J Surg Oncol. 2014;12:209. [Open access.] PMID: 25022617, PMCID: PMC4110236.
Carcinoid Tumors: Cancers in Slow Motion
Ali Ahmad, MD | February 16, 2017 | Contributor Information
Workup
Laboratory studies
Patients with suspected carcinoid tumors should be assessed and managed by multidisciplinary teams at centers with expertise on neuroendocrine tumors.[8,11] Histologic confirmation is required for the diagnosis; as discussed earlier, the mitotic count and Ki-67 proliferation index are used in the WHO and ENETS classification systems.
In addition to obtaining a detailed history and physical examination and routine blood tests, the initial baseline evaluation should include 5-HT and fasting gastrin levels (for gastric carcinoids), as well as biochemical investigations of chromogranin A and 24-hour urinary 5-hydroxyindoleacetic acid (5-HIAA).[2-4,8,11] These markers may be associated with disease severity, effectiveness of therapy, and/or recurrent disease.[4-6]
Note: Chromogranin A levels are sensitive but nonspecific for carcinoids, and levels may be elevated in patients taking proton pump inhibitors.[4,8,11] 5-HIAA test results are sensitive and specific; however, in the 48 hours before undergoing 5-HIAA testing, patients should avoid foods rich in 5-HT (eg, bananas, avocados, grapefruit, tomatoes, eggplant, hickory nuts, walnuts).[4,8,11]
Genetic studies are indicated to identify inherited genetic syndromes (eg, MEN1, NF1) and/or, potentially, any specific genetic features of the carcinoids.[8,11]
The images reveal a mixture of pulmonary carcinoid tumors, extensive neuroendocrine proliferation, and multiple pulmonary sclerosing hemangiomas (PSH). Image A is from a 5.5-mm typical carcinoid tumor composed of uniform short-spindled or oval cells (H&E stain and polymer method; ×20 magnification). Image B reveals a carcinoid tumorlet (nodular proliferation of neuroendocrine cells <0.5 cm) (right solid arrow) admixed with PSH (left dotted arrow) in a single nodule (×40). Image C shows foci of pulmonary neuroendocrine cell proliferation highlighted by synaptophysin staining beneath airway epithelium (arrows) (×40). Image D demonstrates positive chromogranin staining in a tumorlet. Note the contrast in the surrounding negative PSH areas (×20).
Adapted images courtesy of Fasshauer M, Lincke T, Witzigmann H, et al. BMC Cancer. 2006;6:108. [Open access.] PMID: 16643652, PMCID: PMC1464147.
Carcinoid Tumors: Cancers in Slow Motion
Ali Ahmad, MD | February 16, 2017 | Contributor Information
Imaging studies
Abdominal, pelvic, and/or chest multiphasic CT scanning or magnetic resonance imaging (MRI) are recommended to localize primary and/or metastatic tumors.[2,4,6-8,11] As appropriate, a combination of the following imaging modalities may also be used to evaluate suspected carcinoids:
Echocardiography and/or angiography if carcinoid heart disease is suspected
The CT scans and MRIs are from the same patient discussed in slide 4 who had a neuroendocrine carcinoma of the mesentery that resulted in ectopic Cushing syndrome. The 4/2005 CT scan (top left image) reveals a mass that had been interpreted as normal intestine on previous scans (top middle and right images). The bottom images are fat-saturated and T1-weighted MRIs before/after administration of gadolinium showing a mass in the mesentery outside the intestine.
Adapted image courtesy of Fasshauer M, Lincke T, Witzigmann H, et al. BMC Cancer. 2006;6:108. [Open access.] PMID: 16643652, PMCID: PMC1464147.
Carcinoid Tumors: Cancers in Slow Motion
Ali Ahmad, MD | February 16, 2017 | Contributor Information
These 111In-pentetreotide octreotide scintigraphy images are from the same patient discussed in slides 4 and 12. Radiotracer accumulation is seen in the left thyroid in the 10/2003 scan (arrow). In the 4/2005 scan, the mesenterial neuroendocrine tumor became clearly visible (arrow).
Images courtesy of Senda E, Fujimoto K, Ohnishi K, et al. World J Surg Oncol. 2009;7:9. [Open access.] PMID: 19159493, PMCID: PMC2636813.
Carcinoid Tumors: Cancers in Slow Motion
Ali Ahmad, MD | February 16, 2017 | Contributor Information
These contrast-enhanced CT scans are from the same patient with a carcinoid tumor at the ampulla of Vater and lymph node metastases discussed on slide 10.
Image (a): The common bile duct is markedly dilated (arrow), and two enlarged lymph nodes can be seen in the peripancreatic region. The arrowhead indicates one of the two enlarged lymph nodes near the upper border of the pancreas.
Image (b): Another enlarged lymph node is seen near the lower border of the pancreas (arrowhead).
Adapted images courtesy of National Cancer Institute (NCI) (left) and CRUK via Wikimedia Commons (right).
Carcinoid Tumors: Cancers in Slow Motion
Ali Ahmad, MD | February 16, 2017 | Contributor Information
Endoscopic procedures (eg, bronchoscopy [shown], esophagogastroscopy, gastroscopy, colonoscopy) may also be used to biopsy tissue specimens. On histologic examination, carcinoid tumors have five generally accepted distinctive patterns[5,12]:
Insular (common): Solid, nodular nests; associated with a favorable prognosis
Trabecular (common): Ribbons, bands, or loops with anastomosing features; associated with a favorable prognosis
Glandular (low incidence): Tubules with acinar or rosettelike patterns; associated with a poor prognosis
Undifferentiated: Poorly differentiated or atypical patterns; associated with a poor prognosis
Mixed patterns
Tubules can contain mucinous secretions, and individual tumor cells can contain mucin-positive material.[5] Capillaries are often prominent. The tumor cells are uniformly round or polygonal with a central nucleus and punctate chromatin as well as small nucleoli and infrequent mitosis. Eosinophilic granules may be present. Immunohistochemically, these tumors have a strong positive reaction to keratin and neuroendocrine markers, such as chromogranin and synaptophysin.
Images courtesy of Senda E, Fujimoto K, Ohnishi K, et al. World J Surg Oncol. 2009;7:9. [Open access.] PMID: 19159493, PMCID: PMC2636813.
Carcinoid Tumors: Cancers in Slow Motion
Ali Ahmad, MD | February 16, 2017 | Contributor Information
These images are from the same patient with a carcinoid tumor at the ampulla of Vater and lymph node metastases discussed on slides 10 and 14.
The left image is an endoscopic view showing a slightly enlarged ampullary region, suggesting the existence of a submucosal tumor because the epithelium has a normal appearance.
The right image was obtained during endoscopic retrograde cholangiopancreatography (ERCP). Severe stenosis of the distal portion of the common bile duct with marked proximal dilatation is seen. The main pancreatic duct is not dilated.
Images courtesy of Huang J, Lu ZS, Yang YS, et al. World J Surg Oncol. 2014;12:23. [Open access.] PMID: 24472342, PMC3912495.
Carcinoid Tumors: Cancers in Slow Motion
Ali Ahmad, MD | February 16, 2017 | Contributor Information
These images are from a patient who underwent endoscopic mucosal resection with circumferential incision of a rectal carcinoid tumor.
Image A is a conventional endoscopic view showing a carcinoid tumor in the rectum.
Image B is an endoscopic ultrasonographic (EUS) image that reveals the lesion does not invade the muscularis propria layer.
Image C demonstrates marking dots placed around the lesion, image D shows the circumferential incision around the dots, and image E demonstrates snaring following the circumferential incision.
Image F depicts the area after endoscopic mucosal resection with circumferential incision, and image G reveals the resected specimen.
Image H is the histologic view of the rectal carcinoid tumor (H&E stain; ×40 magnification).
Images courtesy of Wang Y, He Q, Shi W, Wang J, Ji H. World J Surg Oncol. 2014;12:209. [Open access.] PMID: 25022617, PMCID: PMC4110236.
Carcinoid Tumors: Cancers in Slow Motion
Ali Ahmad, MD | February 16, 2017 | Contributor Information
General Therapeutic Approaches
Treatment approaches to GI carcinoids vary according to their anatomic location, stage/grade, and extent.
Many consensus guidelines, including those from the following organizations, are available regarding the diagnosis and treatment of GI neuroendocrine tumors; they detail site-specific approaches to managing GI carcinoids:
These images are from the same patient discussed in slide 11 who had multiple PSH and extensive neuroendocrine lesions, including pulmonary neuroendocrine cell hyperplasia, multiple carcinoid tumorlets, and typical carcinoid tumors within one pulmonary lobe.
Image A is a CT scan demonstrating a nodular and irregular density (arrow) in the medial segment of the right middle pulmonary lobe.
Image B shows the resected lung specimen; multiple nodules of varying sizes can be seen both beneath the subpleural and within the pulmonary parenchyma. The overall lesion measured 9.0 cm in its greatest dimension and was composed of nodules ranging from 0.3 cm to 2.2 cm.
Image courtesy of Flickr/Yale Rosen.
Carcinoid Tumors: Cancers in Slow Motion
Ali Ahmad, MD | February 16, 2017 | Contributor Information
Surgery
If feasible, the treatment of choice for carcinoid tumors is surgical excision,[2-5,8,11] which has the potential to be curative for localized tumors.[4] Nonetheless, resection does not guarantee prevention of metastasis at a later date.
Surgical technique may vary according to the type or location of the tumor, as in the following examples:
Appendiceal tumors: Most often, simple appendectomy is sufficient for treatment of small carcinoids (<2 cm) that are away from the base of the appendix.[5,8]
Intestinal lesions: En bloc resection of the tumor with adjacent lymph nodes should be attempted.[5,8]
Bronchopulmonary carcinoids: Aggressive surgical resection, rather than bronchoscopic removal, is recommended.[5,8,13]
Rectal tumors: Endoscopic resection in adults may be sufficient for tumors up to 2 cm in size, those limited to the mucosa, and those resected with adequate margin at presentation.[8]
The image shows a gross specimen of a resected bronchial carcinoid (arrow). The large polypoid tumor completely obstructed the lumen of a lobar bronchus, resulting in severe, diffuse bronchiectasis distal to the obstruction.
Images courtesy of Huang J, Lu ZS, Yang YS, et al. World J Surg Oncol. 2014;12:23. [Open access.] PMID: 24472342, PMC3912495.
Carcinoid Tumors: Cancers in Slow Motion
Ali Ahmad, MD | February 16, 2017 | Contributor Information
These images were obtained during an incomplete endoscopic mucosal resection of a rectal carcinoid tumor.
Image A is a conventional endoscopic view showing a carcinoid tumor in the rectum.
Image B is an EUS view of the lesion in the muscularis propria layer.
Image C shows snaring after injection of submucosal injection solution.
Image D shows the remnant carcinoid tumor on the post–endoscopic mucosal resection ulcer (arrows).
Images courtesy of Aslam MI, Salha IB, Muller S, Jameson JS. Cases J. 2009;2:8317. [Open access.] PMID: 19918418, PMCID: PMC2769428.
Carcinoid Tumors: Cancers in Slow Motion
Ali Ahmad, MD | February 16, 2017 | Contributor Information
These images are from a patient with synchronous ileal carcinoid and primary colonic neoplasms.
The CT scan shows a polypoidal lesion at the ileocecal junction (arrow).
The intraoperative image reveals a yellowish white carcinoid lesion at the ileocecal junction. The terminal ileum was opened for demonstration of the lesion.
Image courtesy of the NCI/Bill Branson.
Carcinoid Tumors: Cancers in Slow Motion
Ali Ahmad, MD | February 16, 2017 | Contributor Information
Systemic management options
For symptomatic and/or locoregional control of carcinoid tumors, the long-acting somatostatin analogue octreotide (long-acting release [LAR] 20-30 mg intramuscularly [IM] every 4 weeks) or lanreotide (120 mg deep subcutaneous [SC] injection every 4 weeks), in conjunction with supportive measures, may be administered.[2,4,8,11]
Chemotherapy is usually limited to unresectable and metastatic tumors, and it includes use of the following cytotoxic agents alone or in combination[8]:
Everolimus
5-Fluorouracil
Capecitabine
Dacarbazine
Oxaliplatin
Streptozocin
Temozolomide
Interferon alfa-2b
Radiotherapy is generally not used to treat carcinoid tumors, but it may be an option for palliation.[2,3]
Molecular structure image of the VEGF-A protein courtesy of Wikimedia Commons/Emw.
Carcinoid Tumors: Cancers in Slow Motion
Ali Ahmad, MD | February 16, 2017 | Contributor Information
Molecular-targeted therapies
Emerging therapies include those that target vascular endothelial growth factor (VEGF), platelet-derived growth factor receptor, mammalian target of rapamycin (mTOR), and stem-cell factor (c-kit).[4,11] Investigational agents for these purposes include the following[4]:
The VEGF monoclonal antibody bevacizumab
VEGF tyrosine kinase inhibitors
Sunitinib
Vatalanib
Sorafenib
The mTOR inhibitor everolimus (RAD001)
Image of a pulmonary carcinoid tumor courtesy of Flickr/Yale Rosen.
Carcinoid Tumors: Cancers in Slow Motion
Ali Ahmad, MD | February 16, 2017 | Contributor Information
Prognosis and Survival Rates
As is the case for most neoplasms, the prognosis for patients with carcinoid tumors varies according to the stage at diagnosis, histologic classification, and primary site of the lesion.
Elevated expression of the proliferation antigen Ki-67 and the tumor suppressor protein p53 has been associated with a poorer prognosis; however, some investigators suggest that the Ki-67 index may help in establishing the prognosis of gastric lesions only.[4] Other adverse clinical prognostic indicators include the presence of the following:
Carcinoid syndrome
Carcinoid heart disease
Elevated concentrations of tumor markers 5-HIAA and chromogranin A
The estimated 5-year survival rate for patients with a nonmetastatic GI carcinoid tumors ranges from 65% to 90%, depending on the tumor site.[3] If regional metastasis has occurred, the 5-year survival ranges from 46% to 78%. With distant metastasis, survival decreases to 14%-54%.
The estimated 5-year survival for patients with a typical lung carcinoid tumor is 85%-90%. Those with an atypical lung carcinoid tumor have a 5-year survival rate of 50%-70%.[3]
Once the leading cause of US cancer deaths, the incidence of gastric cancer has declined in the past few decades. However, most patients present with advanced disease and have poor outcomes. Do you know the risk factors and diagnostic/management options for this potentially fatal malignancy?Slideshows, October 2015
Carcinoid tumors are derived from primitive stem cells in the gut wall but can be seen in other organs, including the lungs, mediastinum, thymus, liver, pancreas, bronchus, ovaries, prostate, and kidneys. In children, most tumors occur in the appendix and are benign and asymptomatic.Diseases/Conditions, October 2016
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Contributor Information
Author
Ali Ahmad, MD
Complex General Surgical Oncology Fellow
Roger Williams Medical Center
Providence, Rhode Island
Disclosure: Ali Ahmad, MD, has disclosed no relevant financial relationships.
Contributor
Vera Ruvinskaya, MS
Disclosure: Vera Ruvinskaya, MS, has disclosed no relevant financial relationships.
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Olivia Wong, DO
Section Editor
Medscape Drugs & Diseases
New York, New York
Disclosure: Olivia Wong, DO, has disclosed no relevant financial relationships.
John Heinegg, RN
Section Editor
Medscape Drugs & Diseases
New York, New York
Disclosure: John Heinegg, RN, has disclosed no relevant financial relationships.
Ramage JK, Ahmed A, Ardill J, et al, for the UK and Ireland Neuroendocrine Tumour Society. Guidelines for the management of gastroenteropancreatic neuroendocrine (including carcinoid) tumours (NETs). Gut. 2012 Jan;61(1):6-32. PMID: 22052063.
Debois JM. Carcinoids. Selected Topics in Clinical Oncology: An in-depth Study of 18 Cancers Usually Neglected in Classical Textbooks. Dordrecht, Netherlands: Springer Science; 2012: chapter 5.
National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology (NCCN guidelines). Small cell lung cancer. Version 2.2017 — September 15, 2016. Available at: http://www.nccn.org/professionals/physician_gls/pdf/sclc.pdf. Accessed: February 6, 2017.
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