What is the common clinical presentation of carcinoid tumors?

Updated: Feb 12, 2019
  • Author: Cameron K Tebbi, MD; Chief Editor: Max J Coppes, MD, PhD, MBA  more...
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Answer

Clinical presentations and symptoms

  • The most common clinical presentation for a small intestinal carcinoid is periodic abdominal pain, which can be caused by fibrosis of the mesentery, kinking of the bowel, or intestinal obstruction. A constellation of symptoms called malignant carcinoid syndrome is often associated with this tumor.

  • Production of vasoactive intestinal peptide (VIP) may produce symptoms similar to those of neuroblastoma, which is far more prevalent than carcinoid in children.

  • Ectopic adrenocorticotropic hormone (ACTH) and Cushing syndrome observed with foregut carcinoid tumors must be differentiated from other tumors that produce these symptoms. Likewise, rare acromegaly caused by the carcinoid tumors must be differentiated from pituitary tumors.

  • Carcinoid crisis can occur spontaneously or as a response to stress, such as anesthesia or chemotherapy. Symptoms may include intense flushing, diarrhea, abdominal pain, tachycardia, hypertension or hypotension, altered mental status, and coma. This condition can be life threatening, but treatment with somatostatin analog SMS-201-995 has improved the outcome of patients with carcinoid crisis.

  • An early and frequent (94%) symptom of carcinoid tumors, especially those of midgut with metastases, is cutaneous flushing, which typically affects the head and neck. Striking color changes range from pallor or erythema to cyanosis. Episodes are often associated with an unpleasant warm feeling, itching, palpitation, upper-body erythema and edema, salivation, diaphoresis, lacrimation, and diarrhea. Exercise, stress, or certain foods (eg, cheese) may trigger an attack, although the flushes can also be spontaneous and unrelated to any stimulation. Initial attacks are short, lasting only a few minutes. With time, the duration increases to hours. Flushes are reported to be longest in association with bronchial carcinoids. Some patients develop a constant red or cyanotic discoloration.

  • Diarrhea and malabsorption occur in as many as 84% of patients. Stools are watery, frothy, bulky, or in the form of steatorrhea. Diarrhea may or may not be associated with abdominal pain, flushing, and cramps. It may be profuse and often colicky.

  • Wheezing or asthmalike syndrome is caused by bronchial constriction and may occur in as many as 25% of patients. Some tremors are relatively indolent and result in chronic symptoms such as cough, shortness of breath and dyspnea. [16] Bronchopulmonary carcinoid tumor presenting with Cushing syndrome has been reported. [52] Behavior of bronchial carcinoid tumor can range from benign to aggressive with metastasis. [53] A child with a carcinoid tumor who had late relapse in mediastinum and metastasis to cerebellum 16 years after the initial diagnosis with atypical carcinoid tumor has been reported. [53]

  • Other symptoms of carcinoid tumors may include valvular heart lesions. Cardiac manifestations are observed in as many as 60% of patients. Fibrosis of the endocardium, which often involves the right side of the heart, is observed. The fibrous deposit usually involves the ventricular aspect of the tricuspid valve and associated chordae. Fibrosis of the pulmonic valve is relatively uncommon and results in regurgitation or stenosis. Cardiac lesions may lead to heart failure. The mitral valve is infrequently involved.


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