eMedicine Specialties > Oncology > Carcinomas of the Gastrointestinal Tract

Malignant Carcinoid Syndrome: Differential Diagnoses & Workup

Author: Luigi Santacroce, MD, Assistant Professor, Medical School, State University at Bari, Italy
Coauthor(s): Laura Diomede, University of Bari School of Medicine, Italy; Lodovico Balducci, MD, Professor of Oncology and Medicine, University of South Florida College of Medicine; Division Chief, Senior Adult Oncology Program, H Lee Moffitt Cancer Center and Research Institute
Contributor Information and Disclosures

Updated: Nov 19, 2009

Differential Diagnoses

Anaphylaxis
Tumor Lysis Syndrome
Angioedema
Urticaria
Intestinal Motility Disorders
Irritable Bowel Syndrome
Ogilvie Syndrome

Other Problems to Be Considered

Sprue, nontropical
Bowel obstruction
Pellagra

Workup

Laboratory Studies

  • 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-HIAA in a 24-hour urine collection (normal value [NV] = 0-8.9 mg/d; plasma serotonin NV is 0.04–0.2 mg/mL).
    • Consumption of foods that contain serotonin can complicate the biochemical diagnosis of malignant carcinoid syndrome; in fact, the following foods contain an amount of serotonin that can produce abnormally elevated excretion of urinary 5-HIAA after ingestion:
      • Spinach
      • Eggplant
      • Cheese (eg, parmesan, Roquefort)
      • Wine (chianti, burgundy)
      • Caffeine
      • Tomatoes
      • Kiwi fruit
      • Bananas
      • Walnuts
      • Pineapples
      • Red plums
      • Avocados
    • The following drugs may have the same effects:
      • Isoniazid
      • Phenothiazine
      • Phenacetin
      • Monoamine oxidase inhibitors
      • Acetaminophen
      • Fluorouracil
      • Iodine solutions
    • If dietary (or pharmaceutical) 5-hydroxyindoles are excluded, a urinary excretion of 5-HIAA of 25 mg/d is diagnostic of carcinoid. If the range value is 9-25 mg/d, the differential diagnosis includes carcinoid syndrome, nontropical sprue, or acute intestinal obstruction.
    • The measurement of other bioactive amines (eg, serotonin, catecholamines, histamine, histamine metabolites25 ) in the platelets, plasma, and urine of patients with carcinoid tumors is of interest but has less diagnostic value than an assay of the major metabolite of serotonin in the urine.
    • Some authors have used high-performance liquid chromatography and gas chromatography mass spectrometry to characterize carcinoids. According to these authors, the platelet serotonin level seems to have a higher sensitivity for detection of carcinoid tumors and is more consistently elevated than urinary 5-HIAA, especially with tumors characterized by a low rate of serotonin production. However, in patients with a high rate of serotonin secretion, the platelet serotonin level reaches a maximum, whereas urinary 5-HIAA does not, indicating that the platelet compartment is saturable. Differing from urinary 5-HIAA, platelet serotonin is not influenced by the consumption of a serotonin-rich diet; therefore, the measurement of platelet serotonin should be preferred for making the primary diagnosis.
    • Platelet serotonin levels are also monitored during different treatments to evaluate the effect of therapy.


  • According to a 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. These authors recommended the high cutoff range in order to exclude patients in whom the chromogranin A level was elevated as a result of other non-neoplastic diseases.26
  • In hindgut carcinoid tumors, chromogranin A, alpha human chorionic gonadotropin (α-HCG), β-HCG, and PP must be included in the follow-up study.
  • For lung carcinoids, gastrin, ACTH, growth hormone (GH), α-HCG, and β-HCG are helpful, and levels of urinary 5-HIAA, chromogranin A, and tachykinins are useful in midgut carcinoid tumors
  • Apart from measuring daily urinary 5-HIAA secretion, determining the presence of other bioactive amines enables more sensitive detection and also may indicate specific 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, DIC occurring in some patients with malignant carcinoid syndrome). Platelet aggregation normally occurs within 3-5 minutes.
  • Total protein levels in blood are often low because of malabsorption; tryptophan levels may be low for the same reason, but also because of conversion to serotonin by the tumor.
  • 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.27
  • Routine allergy test results are not usually positive in cases that simulate an anaphylactic attack.

Imaging Studies

  • For the diagnosis of carcinoids, several diagnostic methods have been evaluated, including barium examinations, iodine-131 metaiodobenzylguanidine (MIBG) scanning28 and octreotide scanning, CT scan, angiography, and venous blood sampling with radioimmunoassay of tumor products.29
  • Scintigraphy
    • 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.
    • The 3-day half-life of this radionuclide allows for a scan after 24, 48, and 72 hours.
    • This diagnostic tool also has obviated many of the problems of differential diagnosis with other neuroendocrine tumors that are frequent, using iodine-131 MIBG or iodine-123 tyrosine 3 octreotide scanning.
    • False-negative results are possible in 2% of cases (the mean percentage of carcinoids without receptors).
    • A positive test result usually predicts a good patient response to treatment with octreotide.
    • When administering a radioactive somatostatin analogue (In-111 DTPA-D-Phe1 octreotide), some authors have attempted to provide internal radiation therapy, hoping to kill the tumor cells, but adverse effects limit the clinical application of this therapy.
  • Radiography
    • Barium examination is rarely diagnostic but may show a benign-appearing submucosal lesion or a large bulky ulcerating mass with bowel deformity.
    • A smooth polyp observed in the terminal ileum should always be considered a probable carcinoid tumor.
    • The importance of angiography for carcinoid diagnosis has been decreased by the availability of more recent imaging methods.
  • CT scanning
    • CT scanning may be used to find the primary tumor or to check for any disease spread.
    • Primary carcinoids of the bowel are usually not observed on CT scanning; otherwise, this study allows the assessment of the extent of tumor spread to the mesentery and bowel wall and metastases to the lymph nodes and liver.
    • CT scanning typically shows a homogeneous, ill-defined mesenteric mass with calcifications.
    • A stellate or curvilinear fibrosis radiating from the mass, representing thickened neurovascular bundles and distorting surrounding bowel loops, is usually observed.
  • MRI30
    • Further studies with this diagnostic procedure are required before MRI can be considered a first-choice method for diagnosing and staging carcinoids and related syndromes. At present, this study is very expensive and offers minimal diagnostic advantages.
  • Positron emission tomography (PET) scanning
    • Experience with PET scanning to detect carcinoids is very limited.
    • Either tryptophan or its metabolite 5-hydroxytryptophan (5-HTP) has been used as a tracer substance.
    • PET scanning seems to be capable of identifying carcinoid metastases to various sites, and it may be valuable in the follow-up care of treated patients.
    • According to initial reports, only C11-5-HTP is taken up by serotonin-producing tumors.
  • Ultrasonography
    • Ultrasonographic examination of the abdomen is usually not the first diagnostic method; instead, it is used to further confirm the diagnosis and establish the site and extent of the disease.

Other Tests

  • Several provocative tests have been developed for carcinoid syndrome.
    • The most recent test uses pentagastrin.
    • The traditional test 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.

Procedures

  • A percutaneous or laparotomy biopsy may be performed, when possible, after the primary tumor and its eventual metastases are detected. Fine-needle biopsy of hepatic lesions can precipitate carcinoid crisis in a patient with carcinoid liver metastasis, however, so personnel performing these procedures should be prepared for this possibility.31
  • Diagnostic and operative endoscopy of the lower and upper GI tract may be helpful for diagnosis.

Histologic Findings

In 1963, Williams and Sandler began to classify the carcinoid tumors anatomically and clinically according to embryologic origin from the foregut, midgut, or hindgut. Grossly, these tumors appear as submucosal or intramural masses (see Media files 1-2), and they are usually single but may be multiple. After fixation, the tumor mass appears yellow or brownish, small, and firm. The intestinal mucosa over the tumor is often intact. Submucosal infiltration, often extending beyond the muscularis propria, is the rule.

A section (on the right) of an intestinal carcino...

A section (on the right) of an intestinal carcinoid mass arising from the mucosa (150 X). Image courtesy of Professor Pantaleo Bufo, University of Foggia, Italy.

[ CLOSE WINDOW ]
A section (on the right) of an intestinal carcino...

A section (on the right) of an intestinal carcinoid mass arising from the mucosa (150 X). Image courtesy of Professor Pantaleo Bufo, University of Foggia, Italy.


A section of a carcinoid mass (350 X). Image cour...

A section of a carcinoid mass (350 X). Image courtesy of Professor Pantaleo Bufo, University of Foggia, Italy.

[ CLOSE WINDOW ]
A section of a carcinoid mass (350 X). Image cour...

A section of a carcinoid mass (350 X). Image courtesy of Professor Pantaleo Bufo, University of Foggia, Italy.


 

Histologically, the tumor consists of uniform small cells arranged as islands separated by a fibrous stroma. Cells show a scant pink cytoplasm that is finely granulated and stippled with small round nuclei and small nucleoli. Several patterns can be observed in carcinoid tumors (ie, trabecular and tubular arrangements may be present and include intraluminal mucin). All carcinoids react positively with antichromogranin A antibodies and usually Masson staining, which indicates serotonin production and is positive in midgut primary tumors. Two main histologic features are described by observing with 10X high-power fields, as follows:

  • Typical, showing fewer than 2 mitoses per 2 mm2 of viable tumor and lacking necrosis
  • Atypical, showing 2-10 mitoses per 2 mm2 with or without foci of necrosis

Staging

No internationally accepted staging system exists for carcinoid tumors.

More on Malignant Carcinoid Syndrome

Overview: Malignant Carcinoid Syndrome
Differential Diagnoses & Workup: Malignant Carcinoid Syndrome
Treatment & Medication: Malignant Carcinoid Syndrome
Follow-up: Malignant Carcinoid Syndrome
Multimedia: Malignant Carcinoid Syndrome
References
[ CLOSE WINDOW ]

References

  1. Bendelow J, Apps E, Jones LE, et al. Carcinoid syndrome. Eur J Surg Oncol. Mar 2008;34(3):289-96. [Medline].

  2. Feldman JM. Carcinoid tumors and syndrome. Semin Oncol. Sep 1987;14(3):237-46. [Medline].

  3. Feldman JM. Carcinoid tumors and the carcinoid syndrome. Curr Probl Surg. Dec 1989;26(12):835-85. [Medline].

  4. Lubarsch O. Ueber den primaren Krebs des Ileum, nebst Bemerhunge uber das gleichzeitge Vorkommen von Krebs und Tuberculose. Virchows Arch. 1888;111:280-317.

  5. Oberndorfer S. Uber die "kleinen Dunndarm Carcinome". Verh Dtsch Ges Pathol. 1907;11:113—6.

  6. Erspamer V, Asero B. Identification of enteramine, the specific hormone of the enterochromaffin cell system, as 5-Hydroxytryptamine. Nature. 1952;169:800-1.

  7. Gosset A, Masson P. Tumeurs endocinne de l'appendice. Presse Med. 1914;22:237-40.

  8. Masson, P. Carcinoids (argentaffin-cell tumors) and nerve hyperplasia of appendicular mucosa. Am J Patholol. 1928;4:181-212.

  9. Ahlman H. Midgut carcinoid tumors. Surg Annu. 1986;18:65-93. [Medline].

  10. Papadogias D, Makras P, Kossivakis K, et al. Carcinoid syndrome and carcinoid crisis secondary to a metastatic carcinoid tumour of the lung: a therapeutic challenge. Eur J Gastroenterol Hepatol. Dec 2007;19(12):1154-9. [Medline].

  11. Bonaros N, Muller S, Bonatti J, et al. Primary ovarian carcinoid heart disease curatively treated with a two-stage procedure. Thorac Cardiovasc Surg. Oct 2007;55(7):467-9. [Medline].

  12. Chaowalit N, Connolly HM, Schaff HV, et al. Carcinoid heart disease associated with primary ovarian carcinoid tumor. Am J Cardiol. May 15 2004;93(10):1314-5. [Medline].

  13. Bhattacharyya S, Davar J, Dreyfus G, et al. Carcinoid heart disease. Circulation. Dec 11 2007;116(24):2860-5. [Medline].

  14. Cunningham JL, Janson ET, Agarwal S, Grimelius L, Stridsberg M. Tachykinins in endocrine tumors and the carcinoid syndrome. Eur J Endocrinol. Sep 2008;159(3):275-82. [Medline].

  15. Russo S, Boon JC, Kema IP, Willemse PH, den Boer JA, Korf J, et al. Patients with carcinoid syndrome exhibit symptoms of aggressive impulse dysregulation. Psychosom Med. May-Jun 2004;66(3):422-5. [Medline]. [Full Text].

  16. Quaedvlieg PF, Visser O, Lamers CB, Janssen-Heijen ML, Taal BG. Epidemiology and survival in patients with carcinoid disease in The Netherlands. An epidemiological study with 2391 patients. Ann Oncol. Sep 2001;12(9):1295-300. [Medline]. [Full Text].

  17. Berge T, Linell F. Carcinoid tumours. Frequency in a defined population during a 12-year period. Acta Pathol Microbiol Scand A. Jul 1976;84(4):322-30. [Medline].

  18. Wilson HM. Chronic subacute bowel obstruction caused by carcinoid tumour misdiagnosed as irritable bowel syndrome: a case report. Cases J. Jan 22 2009;2(1):78. [Medline].

  19. Metcalfe DD. Differential diagnosis of the patient with unexplained flushing/anaphylaxis. Allergy Asthma Proc. 2000;21(1):21-4. [Medline].

  20. Kleyn CE, Lai-Cheong JE, Bell HK. Cutaneous manifestations of internal malignancy: diagnosis and management. Am J Clin Dermatol. 2006;7(2):71-84. [Medline].

  21. Monsegu J, Algayres JP, Ciribilli JM, et al. [Tricuspid insufficiency disclosing carcinoid syndrome]. Rev Med Interne. 1994;15(9):593-6. [Medline].

  22. Shah GM, Shah RG, Veillette H, Kirkland JB, Pasieka JL, Warner RR. Biochemical assessment of niacin deficiency among carcinoid cancer patients. Am J Gastroenterol. Oct 2005;100(10):2307-14. [Medline].

  23. Banzali FM Jr, Tiwari AK, Frantz R, et al. Valvular heart disease caused by carcinoid syndrome: emphasis on the use of intraoperative transesophageal echocardiography. J Cardiothorac Vasc Anesth. Dec 2007;21(6):855-7. [Medline].

  24. Kulke MH, Freed E, Chiang DY, Philips J, Zahrieh D, Glickman JN, et al. High-resolution analysis of genetic alterations in small bowel carcinoid tumors reveals areas of recurrent amplification and loss. Genes Chromosomes Cancer. Jul 2008;47(7):591-603. [Medline].

  25. Granerus G, Lindell SE, Waldenstrom J, et al. Histamine metabolism in carcinoidosis. Lancet. Jun 4 1966;1(7449):1267-8. [Medline].

  26. Campana D, Nori F, Piscitelli L, Morselli-Labate AM, Pezzilli R, Corinaldesi R, et al. Chromogranin A: is it a useful marker of neuroendocrine tumors?. J Clin Oncol. May 20 2007;25(15):1967-73. [Medline].

  27. Bhattacharyya S, Toumpanakis C, Caplin ME, Davar J. Usefulness of N-terminal pro-brain natriuretic peptide as a biomarker of the presence of carcinoid heart disease. Am J Cardiol. Oct 1 2008;102(7):938-42. [Medline].

  28. Sinzinger H, Renner F, Granegger S. Unsuccessful iodine-131 MIBG imaging of carcinoid tumors and apudomas. J Nucl Med. Jul 1986;27(7):1221-2. [Medline].

  29. Garg S, Bourantas CV, Nair RK, Alamgir F. Carcinoid syndrome diagnosed by echocardiography. Int J Cardiol. Feb 11 2009;[Medline].

  30. Bastarrika G, Cao MG, Cano D, et al. Magnetic resonance imaging diagnosis of carcinoid heart disease. J Comput Assist Tomogr. Nov-Dec 2005;29(6):756-9. [Medline].

  31. Sinha V, Dyer P, Shuvro RC, Geh JI, Karandikar S. Case of carcinoid crisis following a fine-needle biopsy of hepatic metastasis. Eur J Gastroenterol Hepatol. Jan 2009;21(1):101-3. [Medline].

  32. Joensuu H, Kumpulainen E, Grohn P. Treatment of metastatic carcinoid tumour with recombinant interferon alfa. Eur J Cancer. 1992;28A(10):1650-3. [Medline].

  33. Hodul P, Malafa M, Choi J, et al. The role of cytoreductive hepatic surgery as an adjunct to the management of metastatic neuroendocrine carcinomas. Cancer Control. Jan 2006;13(1):61-71. [Medline].

  34. Eller R, Frazee R, Roberts J. Gastrointestinal carcinoid tumors. Am Surg. Jul 1991;57(7):434-7. [Medline].

  35. Farndon JR. The carcinoid syndrome: methods of treatment and recent experience with hepatic artery ligation and infusion. Clin Oncol. Dec 1977;3(4):365-75. [Medline].

  36. Codd JE, Drozda J, Merjavy J. Palliation of carcinoid heart disease. Arch Surg. Sep 1987;122(9):1076-7. [Medline].

  37. Bhattacharyya S, Davar J, Dreyfus G, Caplin ME. Carcinoid heart disease. Circulation. Dec 11 2007;116(24):2860-5. [Medline].

  38. Chambers AJ, Pasieka JL, Dixon E, Rorstad O. The palliative benefit of aggressive surgical intervention for both hepatic and mesenteric metastases from neuroendocrine tumors. Surgery. Oct 2008;144(4):645-51; discussion 651-3. [Medline].

  39. Bernheim AM, Connolly HM, Rubin J, Møller JE, Scott CG, Nagorney DM, et al. Role of hepatic resection for patients with carcinoid heart disease. Mayo Clin Proc. Feb 2008;83(2):143-50. [Medline].

  40. Gregor M. Therapeutic principles in the management of metastasising carcinoid tumors: drugs for symptomatic treatment. Digestion. 1994;55 Suppl 3:60-3. [Medline].

  41. Kleyn CE, Lai-Cheong JE, Bell HK. Cutaneous manifestations of internal malignancy: diagnosis and management. Am J Clin Dermatol. 2006;7(2):71-84. [Medline].

  42. Ramage JK, Davies AH, Ardill J, et al. Guidelines for the management of gastroenteropancreatic neuroendocrine (includingcarcinoid) tumours. Gut. Jun 2005;54 Suppl 4:iv:1-16. [Medline].

  43. Patients, Caregivers, and Friends. The Carcinoid Foundation. Available at www.carcinoid.org/index.shtml. Accessed September 27, 2009.

  44. Jabbour-Khoury S, Dabbous A, al-Jazzar M, et al. Anesthetic management of a patient having a carcinoid syndrome. Middle East J Anesthesiol. Oct 2003;17(3):435-47. [Medline].

  45. Anderson AS, Krauss D, Lang R. Cardiovascular complications of malignant carcinoid disease. Am Heart J. Oct 1997;134(4):693-702. [Medline].

  46. Anthony LB, Martin W, Delbeke D, et al. Somatostatin receptor imaging: predictive and prognostic considerations. Digestion. 1996;57 Suppl 1:50-3. [Medline].

  47. Bhattacharyya S, Toumpanakis C, Caplin ME, et al. Analysis of 150 patients with carcinoid syndrome seen in a single year at one institution in the first decade of the twenty-first century. Am J Cardiol. Feb 1 2008;101(3):378-81. [Medline].

  48. Bourgault C, Bergeron S, Bogaty P, et al. A most unusual acute coronary syndrome. Can J Cardiol. Apr 2006;22(5):429-32. [Medline].

  49. de Diego C, Marcos Alberca P, Cabrera JA, et al. Pathognomonic echocardiographic features of carcinoid syndrome. Clin Cardiol. Mar 2006;29(3):134. [Medline].

  50. [Guideline] de Herder WW, O'Toole D, Rindi G. ENETS Consensus Guidelinesfor the Diagnosis and Treatment of Neuroendocrine Gastrointestinal TumorsPart 2 – Midgut and Hindgut Tumors. Neuroendocrinology. 2008;87.

  51. Di Lucca J, Mahé E, Saiag P. [Severe acrocyanosis in carcinoid syndrome]. Ann Dermatol Venereol. Nov 2007;134(11):895-6. [Medline].

  52. Farling PA, Durairaju AK. Remifentanil and anaesthesia for carcinoid syndrome. Br J Anaesth. Jun 2004;92(6):893-5. [Medline].

  53. Feldman JM. Histaminuria from histamine-rich foods. Arch Intern Med. Nov 1983;143(11):2099-102. [Medline].

  54. Frilling A, Rogiers X, Knofel WT, et al. Liver transplantation for metastatic carcinoid tumors. Digestion. 1994;55 Suppl 3:104-6. [Medline].

  55. González O, Alberca MT, Santonja C. Echocardiographic diagnosis of carcinoid disease: exceptional evolution after ovarian tumor resection without cardiac surgery. Eur J Echocardiogr. Dec 2007;8(6):497-500. [Medline].

  56. Hodgson HJ. Controlling the carcinoid syndrome. BMJ. Nov 12 1988;297(6658):1213-4. [Medline].

  57. Jaber WA, Klarich KW. Images in cardiovascular medicine. Carcinoid heart disease. Circulation. Feb 21 2006;113(7):e160-1. [Medline].

  58. Jagernauth S, Patel A, Baig K, et al. Fungal endocarditis of the eustachian valve in carcinoid heart disease: a case report. J Heart Valve Dis. Nov 2007;16(6):631-3. [Medline].

  59. Lee KJ, Connolly HM, Pellikka PA. Carcinoid pulmonary valvulopathy evaluated by real-time 3-dimensional transthoracic echocardiography. J Am Soc Echocardiogr. Apr 2008;21(4):407.e1-2. [Medline].

  60. Majeed F, Porter TR, Tarantolo S, et al. Carcinoid crisis and reversible right ventricular dysfunction after embolization in untreated carcinoid syndrome. Eur J Echocardiogr. Oct 2007;8(5):386-9. [Medline].

  61. Mansencal N, Mitry E, Forissier JF, et al. Assessment of patent foramen ovale in carcinoid heart disease. Am Heart J. May 2006;151(5):1129.e1-6. [Medline].

  62. Maton PN. The carcinoid syndrome. JAMA. Sep 16 1988;260(11):1602-5. [Medline].

  63. Merino J, Zuluaga A, Gutierrez-Tejero F, et al. Pure testicular carcinoid associated with intratubular germ cell neoplasia. J Clin Pathol. Dec 2005;58(12):1331-3. [Medline].

  64. Mignon M. Natural history of neuroendocrine enteropancreatic tumors. Digestion. 2000;62 Suppl 1:51-8. [Medline].

  65. [Best Evidence] Moller JE, Pellikka PA, Bernheim AM, et al. Prognosis of carcinoid heart disease: analysis of 200 cases over two decades. Circulation. Nov 22 2005;112(21):3320-7. [Medline].

  66. Mollet NR, Dymarkowski S, Bogaert J. MRI and CT revealing carcinoid heart disease. Eur Radiol. Dec 2003;13 Suppl 4:L14-8. [Medline].

  67. Park SB, Kim JK, Cho KS. Imaging findings of a primary bilateral testicular carcinoid tumor associated with carcinoid syndrome. J Ultrasound Med. Mar 2006;25(3):413-6. [Medline].

  68. Robertson RG, Geiger WJ, Davis NB. Carcinoid tumors. Am Fam Physician. 2006;74(3):429-34. [Full Text].

  69. Rorstad O. Prognostic indicators for carcinoid neuroendocrine tumors of the gastrointestinal tract. J Surg Oncol. Mar 1 2005;89(3):151-60. [Medline].

  70. Rosenberg JM, Welch JP. Ileal tumor causing carcinoid syndrome without hepatic metastases. Arch Surg. Apr 1984;119(4):485. [Medline].

  71. Shapiro RS, Shafir M, Sung M, et al. Cryotherapy of metastatic carcinoid tumors. Abdom Imaging. May-Jun 1998;23(3):314-7. [Medline].

  72. Somak R, Shramana M, Vijay S, et al. Primary carcinoid tumor of the ovary: a case report. Arch Gynecol Obstet. Jan 2008;277(1):79-82. [Medline].

  73. Stella M, Decian F, Mithieux F, et al. Ileal carcinoid with liver metastasis presenting after ten years with abdominal mass and right heart failure: report of a case. Tumori. Jan-Feb 2006;92(1):83-5. [Medline].

  74. van der Horst-Schrivers AN, Wymenga AN, Links TP, et al. Complications of midgut carcinoid tumors and carcinoid syndrome. Neuroendocrinology. 2004;80 Suppl 1:28-32. [Medline].

  75. Yeo CJ, Couse NF, Zinner MJ. Serotonin and substance P stimulate intestinal secretion in the isolated perfused ileum. Surgery. Jan 1989;105(1):86-92. [Medline].

  76. Zeitlin IJ. Kinin release in the carcinoid syndrome. Lancet. Nov 1971;2(7732):1040. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

malignant carcinoid syndrome, malignant carcinoid tumor, carcinoid tumors, cancer metastases, cancer metastasis, serotonin excess, neuroendocrine tumors, intestinal tumors, gastroenteropancreatic tumors, facial flushing, diarrhea, asthma

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Luigi Santacroce, MD, Assistant Professor, Medical School, State University at Bari, Italy
Disclosure: Nothing to disclose.

Coauthor(s)

Laura Diomede, University of Bari School of Medicine, Italy
Disclosure: Nothing to disclose.

Lodovico Balducci, MD, Professor of Oncology and Medicine, University of South Florida College of Medicine; Division Chief, Senior Adult Oncology Program, H Lee Moffitt Cancer Center and Research Institute
Disclosure: Nothing to disclose.

Medical Editor

Sanjiv S Agarwala, MD, Chief of Oncology and Hematology, St Luke's Cancer Center, St Luke's Hospital and Health Network; Associate Professor of Cancer Biology, University of Pennsylvania
Sanjiv S Agarwala, MD is a member of the following medical societies: American Association for Cancer Research, American Society for Head and Neck Surgery, Eastern Cooperative Oncology Group, and European Society for Medical Oncology
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

CME Editor

Rajalaxmi McKenna, MD, FACP, Southwest Medical Consultants, SC, Department of Medicine, Good Samaritan Hospital, Advocate Health Systems
Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis
Disclosure: Nothing to disclose.

Chief Editor

Jules E Harris, MD, Clinical Professor of Medicine, Division of Hematology/Medical Oncology, Department of Internal Medicine, University of Arizona College of Medicine at Tucson; Consulting Staff, Arizona Cancer Center
Jules E Harris, MD is a member of the following medical societies: American Association for Cancer Research, American Association for the Advancement of Science, American Association of Immunologists, American Society of Hematology, and Central Society for Clinical Research
Disclosure: GlobeImmune Salary Consulting; Amplimed Consulting fee Consulting; FibroGen Consulting fee Consulting

RELATED EMEDICINE ARTICLES
 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.