II. Epidemiology

  1. Prevalence: 1-2 per 100,000
  2. Age at presentation: 50-60 years

III. Pathophysiology

  1. Carcinoid Tumor originates from Gastrointestinal Tract
    1. Embryonic foregut derivatives
      1. Lungs or Bronchi (23%)
      2. Stomach (7%)
    2. Embryonic midgut derivatives
      1. Ileum source (25%)
      2. Appendix (12%)
    3. Embryonic hindgut derivatives
      1. Distal colon (7%)
      2. Rectum (26%)
  2. Malignant neoplasms of enterochromaffin cells
    1. Excessive Serotonin (5-HT)
    2. Excessive Tachykinins (Substance P)
      1. Causes Flushing
    3. Excessive Histamine may occur
      1. May also be responsible for Flushing
  3. Metastases
    1. Most common sources of metastases
      1. Colon (more than two thirds at diagnosis)
      2. Small Intestine (most at diagnosis)
      3. Rectal sources (most if >2 cm tumor)
    2. Most common metastatic sites
      1. Liver
      2. Lung

IV. Classification (WHO, where PI is proliferation index)

  1. Well-Differentiated endocrine tumor (PI <2%)
  2. Well-Differentiated endocrine carcinoma (PI 2-15%)
  3. Poorly differentiated endocrine carcinoma (PI >15%)

V. Presentations of Carcinoid Tumor

  1. Incidental asymptomatic Carcinoid Tumor (most common)
  2. Vague non-specific symptoms
    1. Diagnosis often delayed for up to 1 decade
    2. Abdominal Pain or Bowel Obstruction may occur
  3. Carcinoid Syndrome (occurs in 10% of Carcinoid Tumors)
    1. Late finding for Carcinoid Tumor
    2. Metastases are usually present when occurs
    3. Associated with midgut tumors (appendix, ileum)
    4. See related symptoms below

VI. Symptoms: Carcinoid Syndrome

  1. Intermittent migratory Flushing of head and neck skin
    1. Most common symptom
    2. Rapid color changes between red, white, and violet
  2. Symptoms accompanying Flushing episodes
    1. Explosive Diarrhea
    2. Nausea and Vomiting
    3. Abdominal Pain or cramping
    4. Bronchoconstriction
  3. Symptoms with prolonged Flushing attacks
    1. Lacrimation
    2. Periorbital edema
  4. Provocative
    1. Exertion
    2. Tyramine vasoactive amines (blue cheese, chocolate)
    3. Alcohol

VII. Signs

  1. Cardiovascular Changes
    1. Thickening of endocardium (valves, chambers)
    2. Tricuspid insufficiency
    3. Pulmonary stenosis
    4. Right Heart Failure
    5. Hypotension
  2. Telangiectasia
  3. Intestinal hypermotility (borborygmi)

VIII. Differential Diagnosis of Flushing

  1. Systemic Mastocytosis
  2. Idiopathic Anaphylaxis
  3. Alcohol ingestion
  4. Post-Menopause
  5. Hyperthyroidism
  6. Vipoma
  7. Thyroid Medullary carcinoma

X. Imaging: CT Abdomen or MRI Abdomen

  1. Indicated for abdominal symptoms
  2. Carcinoid Tumor appearance on imaging
    1. Mucosal thickening
    2. Submucosal mass
    3. Bowel lumen narrowed
    4. Spiculated mass (if infiltrated)

XI. Imaging: Neuroendocrine tumor localization

  1. Indium-111 labeled Octreotide Scan (preferred)
    1. Somatostatin receptor scintigraphy
    2. Test Sensitivity: 60%
    3. Test Specificity: 90%
  2. I-Labeled Meta-iodobenzylguanidine (MIPG)
  3. Positron Emission Tomography (PET Scan)

XII. Management: Options vary depending on presentation

  1. Octreotide 75 to 150 ug up to 750 ug q8 hours
    1. Indications: Flushing
    2. Adverse: Hypoglycemia, Steatorrhea, Cholelithiasis
  2. Alpha-Interferon
    1. Possibly efficacious as adjunctive therapy
  3. Antihistamines
    1. May be useful in histamine-related tumors
  4. Surgery
    1. Excision of primary tumor
    2. Tumor debulking

XIII. Complications

  1. Second Primary Malignancy (in up to 46% of Carcinoids)
    1. Gastrointestinal and Genitourinary tumors
    2. Lung and Bronchial cancers

XIV. Prognosis

  1. Five year survival: 67% (variable based on type)
    1. Modlin (2003) Cancer 97:934-59 [PubMed]

XV. Resources

  1. Carcinoid Cancer Foundation, Inc
    1. http://www.carcinoid.org

XVI. References

  1. Abeloff (2000) Clinical Oncology, p. 1379-81
  2. Dumbro (1999) 5 Minute Clinical Consultant, p. 1192
  3. Goldman (2000) Cecil Textbook Medicine,p.1295-7
  4. Kulke (1999) N Engl J Med 340:858-68 [PubMed]
  5. Robertson (2006) Am Fam Physcian 74:429-34 [PubMed]

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Related Studies

Ontology: Carcinoid Tumor (C0007095)

Definition (CHV) A usually small, slowly growing tumor usually found in gastrointestinal tract
Definition (CHV) A usually small, slowly growing tumor usually found in gastrointestinal tract
Definition (CHV) A usually small, slowly growing tumor usually found in gastrointestinal tract
Definition (CHV) A usually small, slowly growing tumor usually found in gastrointestinal tract
Definition (CHV) A usually small, slowly growing tumor usually found in gastrointestinal tract
Definition (CHV) A usually small, slowly growing tumor usually found in gastrointestinal tract
Definition (MEDLINEPLUS)

Carcinoid tumors are rare, slow-growing cancers. They usually start in the lining of the digestive tract or in the lungs. They grow slowly and don't produce symptoms in the early stages. As a result, the average age of people diagnosed with digestive or lung carcinoids is about 60.

In later stages the tumors sometimes produce hormones that can cause carcinoid syndrome. The syndrome causes flushing of the face and upper chest, diarrhea, and trouble breathing.

Surgery is the main treatment for carcinoid tumors. If they haven't spread to other parts of the body, surgery can cure the cancer.

Definition (NCI_NCI-GLOSS) A slow-growing type of tumor usually found in the gastrointestinal system (most often in the appendix), and sometimes in the lungs or other sites. Carcinoid tumors may spread to the liver or other sites in the body, and they may secrete substances such as serotonin or prostaglandins, causing carcinoid syndrome.
Definition (NCI_CDISC) A neuroendocrine neoplasm arising from enterochromaffin cells in the gastrointestinal tract and (less common) the bronchi with undetermined malignancy status.
Definition (NCI) A slow growing neuroendocrine tumor, composed of uniform, round, or polygonal cells having monotonous, centrally located nuclei and small nucleoli, infrequent mitoses, and no necrosis. The tumor may show a variety of patterns, such as solid, trabecular, and acinar. Electron microscopy shows small secretory granules. Immunohistochemical studies reveal NSE, as well as chromogranin immunoreactivity. Malignant histology (cellular pleomorphism, hyperchromatic nuclei, prominent nucleoli, necrosis, and mitoses) can occasionally be seen. Such cases may have an aggressive clinical course. Gastrointestinal tract and lung are common sites of involvement.
Definition (MSH) A usually small, slow-growing neoplasm composed of islands of rounded, oxyphilic, or spindle-shaped cells of medium size, with moderately small vesicular nuclei, and covered by intact mucosa with a yellow cut surface. The tumor can occur anywhere in the gastrointestinal tract (and in the lungs and other sites); approximately 90% arise in the appendix. It is now established that these tumors are of neuroendocrine origin and derive from a primitive stem cell. (From Stedman, 25th ed & Holland et al., Cancer Medicine, 3d ed, p1182)
Concepts Neoplastic Process (T191)
MSH D002276
ICD10 D3A.00
SnomedCT 443492008, 189615009, 189608001, 189607006
English Carcinoid, Tumor, Carcinoid, Tumors, Carcinoid, Carcinoids, Carcinoid tumors NOS, Carcinoid tumours, Carcinoid tumours NOS, [M]Carcinoid tumors, [M]Carcinoid tumors NOS, [M]Carcinoid tumours, [M]Carcinoid tumours NOS, Carcinoid tumour NOS, Carcinoid tumor NOS, [M]Carcinoid tumor NOS, [M]Carcinoid tumour NOS, Carcinoid tumor, Carcinoid tumor (disorder), Carcinoid tumour, Carcinoid Tumor [Disease/Finding], carcinoid tumours, carcinoid tumor, carcinoids, carcinoid tumour, carcinoid tumors, Carcinoid Tumors, [M]Carcinoid tumor NOS (morphologic abnormality), [M]Carcinoid tumors NOS (morphologic abnormality), WELL-DIFFERENTIATED ENDOCRINE NEOPLASM, CARCINOID TUMOR, UNDETERMINED, CARCINOID, CARCINOID NEOPLASM, carcinoid, Carcinoid tumor - morphology, Carcinoid tumour - morphology, Carcinoid tumor - morphology (morphologic abnormality), Carcinoid Tumor, Carcinoid tumors
Italian Tumore carcinoide, Tumori carcinoidi, Tumore carcinoide NAS, Carcinoide
Dutch carcinoïdtumor NAO, carcinoïdtumoren, carcinoïdtumor, Carcinoïd, Carcinoïdtumor
French Tumeur carcinoïde SAI, Tumeurs carcinoïdes, Carcinoïde, Tumeur carcinoïde
German karzinoider Tumor NNB, Karzinoide Tumoren, karzinoider Tumor, Karzinoidtumor, Karzinoid
Portuguese Tumor carcinóide NE, Carcinoide, Tumor Carcinoide, Tumor carcinóide, Tumores carcinóides
Spanish Tumor carcinoide NEOM, tumor carcinoide (trastorno), tumor carcinoide, [M]tumor carcinoide, SAI (anomalía morfológica), [M]tumores carcinoides, SAI, [M]tumores carcinoides, SAI (anomalía morfológica), [M]tumor carcinoide, SAI, tumor carcinoide - morfología (anomalía morfológica), tumor carcinoide - morfología, Tumores carcinoides, Tumor carcinoide, Carcinoide, Tumor Carcinoide
Japanese カルチノイド腫瘍NOS, カルチノイドシュヨウ, カルチノイドシュヨウNOS, 好銀腫, 嗜銀腫, 腸クロム親和細胞腫, 銀親和腫, 嗜銀細胞腫, 杯細胞カルチノイド, カルチノイド, カルチノイド-杯細胞, 類癌腫, 親銀細胞腫, 銀親和性細胞腫, 好銀性細胞腫, 類がん腫, Carcinoid腫瘍, カルチノイド腫瘍
Swedish Karcinoidtumör
Czech karcinoid, Karcinoid, Karcinoid NOS, Karcinoidní nádory, GEP-NET, gastroenteropankreatické neuroendokrinní nádory
Finnish Karsinoidi
Polish Rakowiak
Hungarian Carcinoid tumor, Carcinoid daganat, Carcinoid tumor k.m.n., Carcinoid tumorok
Norwegian Carcinoide svulster, Karsinoide svulster, Karsinoide tumorer

Ontology: Malignant Carcinoid Syndrome (C0024586)

Definition (NCI) A syndrome characterized by signs and symptoms caused by advanced carcinoid tumors. They include skin flushing, diarrhea, wheezing, and tachycardia.
Definition (NCI_NCI-GLOSS) A combination of symptoms caused by the release of serotonin and other substances from carcinoid tumors of the gastrointestinal tract. Symptoms may include flushing of the face, flat angiomas (small collections of dilated blood vessels) of the skin, diarrhea, bronchial spasms, rapid pulse, and sudden drops in blood pressure.
Definition (MSH) A symptom complex associated with CARCINOID TUMOR and characterized by attacks of severe flushing of the skin, diarrheal watery stools, bronchoconstriction, sudden drops in blood pressure, edema, and ascites. The carcinoid tumors are usually located in the gastrointestinal tract and metastasize to the liver. Symptoms are caused by tumor secretion of serotonin, prostaglandins, and other biologically active substances. Cardiac manifestations constitute CARCINOID HEART DISEASE. (Dorland, 27th ed; Stedman, 25th ed)
Concepts Disease or Syndrome (T047)
MSH D008303
ICD9 259.2
ICD10 E34.0
SnomedCT 109950000, 190580007, 154717002, 35868009
English Carcinoid Syndrome, Malignant, Carcinoid Syndromes, Malignant, Malignant Carcinoid Syndrome, Malignant Carcinoid Syndromes, Syndromes, Malignant Carcinoid, CARCINOID SYNDROME, Syndrome, Malignant Carcinoid, carcinoid syndrome (diagnosis), carcinoid syndrome, Malignant Carcinoid Syndrome [Disease/Finding], serotonin syndrome, malignant carcinoid syndrome, carcinoids syndrome, syndrome serotonin, Hormonal tumor (disorder), Malignant carcinoid syndrome, Serotonin syndrome, Carcinoid syndrome, Hormone secretion by carcinoid tumor, Excessive serotonin secretion, Hormone secretion by carcinoid tumour, Carcinoid syndrome (disorder), Hormonal tumor, Hormonal tumour, carcinoid; hormone secretion, tumor, carcinoid; syndrome, hormone secretion; carcinoid tumor, secretion; carcinoid tumor, hormone, syndrome; carcinoid, Carcinoid Syndrome, Hormone secretion by carcinoid tumors
Italian Sindrome carcinoide, Sindrome da carcinoide maligno
Japanese カルチノイド症候群, カルチノイドショウコウグン
Swedish Malignt karcinoidsyndrom
Czech maligní karcinoidní syndrom, Karcinoidní syndrom
Finnish Pahanlaatuinen karsinoidioireyhtymä
Korean 카르시노이드 증후군
Polish Zespół rakowiaka
Hungarian Carcinoid syndroma
Norwegian Malignt karsinoid-syndrom
Portuguese Síndrome do Carcinoide Maligno, Síndrome carcinóide
Spanish tumor hormonal, tumor hormonal (trastorno), secreción de hormonas por el tumor carcinoide, síndrome carcinoide (trastorno), síndrome carcinoide, síndrome de serotonina, síndrome serotoninérgico, Síndrome carcinoide, Síndrome Carcinoide Maligno
Dutch carcinoïd; hormoonsecretie, tumor, carcinoïd; syndroom, hormoonsecretie; carcinoïdtumor, secretie; carcinoïdtumor, hormoon, syndroom; carcinoïd, Carcinoïdsyndroom, carcinoïdsyndroom, Carcinoïdsyndroom, maligne, Maligne carcinoïdsyndroom, Syndroom, maligne carcinoïd-
German Karzinoid-Syndrom, Malignes Karzinoidsyndrom, Cassidy-Scholte-Syndrom, Karzinoidsyndrom, Biörck-Thorson-Syndrom
French Carcinoïdose, Syndrome carcinoïde, Syndrome carcinoïde malin, Syndrome de Björk