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Carcinoid TumorAka: Carcinoid Syndrome, Malignant Carcinoid, Carcinoid, Thorson-Bioerck Syndrome, Argentaffinoma Syndrome, Flush Syndrome, Cassidy-Scholte Syndrome

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  1. Epidemiology
    1. Prevalence: 1-2 per 100,000
    2. Age at presentation: 50-60 years
  2. 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
  3. 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%)
  4. 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
  5. 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
  6. 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)
  7. Differential Diagnosis of Flushing
    1. Systemic Mastocytosis
    2. Idiopathic Anaphylaxis
    3. Alcohol ingestion
    4. Post-Menopause
    5. Hyperthyroidism
    6. Vipoma
    7. Thyroid Medullary carcinoma
  8. Labs
    1. 24 hour Urine 5-hydroxyindoleacetic acid (5-HIAA)
      1. High false positive and false negative rate
    2. Serum Chromogranin A
      1. Test Sensitivity: 80%
      2. Test Specificity: 95%
        1. False positives in Multiple Myeloma
  9. 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)
  10. 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)
  11. 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
  12. Complications
    1. Second Primary Malignancy (in up to 46% of carcinoids)
      1. Gastrointestinal and Genitourinary tumors
      2. Lung and bronchial cancers
  13. Prognosis
    1. Five year survival: 67% (variable based on type)
      1. Modlin (2003) Cancer 97:934
  14. Resources
    1. Carcinoid Cancer Foundation, Inc
      1. http://www.carcinoid.org
  15. 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
    5. Robertson (2006) Am Fam Physcian 74:429

Carcinoid Tumor (C0007095)

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)
Definition (NCI)(KAR-sin-oyd) A type of tumor usually found in the gastrointestinal system (most often in the appendix), and sometimes in the lungs or other sites. Carcinoid tumors are usually benign.
Definition (NCI)Carcinoid tumor, also known as well differentiated endocrine neoplasm, is 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.
ConceptsNeoplastic Process (T191)
MSHD002276
EnglishCarcinoid, Carcinoid Neoplasm, Carcinoid Tumor, Carcinoid tumor - morphology, Carcinoid tumors, Carcinoid tumour, Carcinoid tumour - morphology, Carcinoids, Well-Differentiated Endocrine Neoplasm
Spanishtumor carcinoide - morfologia
Parent ConceptsAdenocarcinoma (C0001418), Neuroendocrine Tumors (C0206754), Carcinoid Tumor (C0007095), Endocrine tumor morphology (C0474803)
SourcesCOSTAR, MEDLINEPLUS, MSH, MTH, NCI, NDFRT, OMIM, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)


Malignant Carcinoid Syndrome (C0024586)

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)
ConceptsDisease or Syndrome (T047)
ICD9259.2, 259.2
MSHD008303
EnglishCarcinoid syndrome, Excessive serotonin secretion, Hormonal tumor, Hormonal tumour, Hormone secretion by carcinoid tumor, Hormone secretion by carcinoid tumors, Hormone secretion by carcinoid tumour, Malignant Carcinoid Syndrome, Malignant Carcinoid Syndromes, Serotonin syndrome
Spanishsecrecion de hormonas por el tumor carcinoide, sindrome carcinoide, sindrome de serotonina, sindrome serotoninergico, tumor hormonal
Parent ConceptsOther and unspecified endocrine disorders (C0154223), Carcinoid Tumor (C0007095), Ectopic hormone secretion syndromes associated with neoplasias (C0851689), Endocrine Gland Neoplasms (C0014132), Ambiguous concept (C1274012), Primary malignant neoplasm of trunk (C1304852), Duplicate concept (C1274013)
SourcesCOSTAR, CSP, DXP, ICD9CM, LCH, MSH, MTH, MTHICD9, NCI, NDFRT, QMR, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)



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