Brian Zeidan

September 16, 2014

Family Practice Rotation Dr. Nadem Sayegh




Carcinoid Syndrome

Carcinoid syndrome is a group of symptoms that occur due to release of hormones
from neuroendocrine tumors of the gastrointestinal tract called carcinoid tumors.

Etiology/Presentation

Carcinoid tumors arise from enterochromaffin cells (neuroendocrine cells also can
occur in pancreas or bronchi). 70% of the time these tumors can be found in the
midgut at the level of ileum. These tumors release serotonin (via hepatic portal
circulation) which in excess can lead to:
Flushing (94% of patients)
Diarrhea (80%)
Carcinoid Heart Disease (40%)
Pellagra (5%) due to niacin deficiency
Carcinoid crisis severe flushing, hypotension, confusion and breathing
difficulty. This can be triggered by anesthesia used in surgery, can be fatal


Pathophysiology

Along with serotonin, the carcinoid tumors also produce kallikrein, which is
reponsbile for breaking down kininogen to form bradykinin. With an increase in
kallikrein, an excess amount of bradykinin causes vasodilation (flushing). Serotonin
acts on the smooth muscle causing diarrhea, colic and malabsorption.

Diagnosis

Diagnosis is usually made by patient presentation of symptoms. Confirmation of
diagnosis is made my by increased urinary excretion of serotonin metabolite 5-
hydroxyindoleacetic acid (5-HIAA).


Complications

Bowel obstruction, thickening heart valves, hypotension






Brian Zeidan
September 16, 2014
Family Practice Rotation Dr. Nadem Sayegh


Treatment

Surgical resection of the primary carcinoids is often curative. If patient presents
with hepatic metastases, surgical debulking will relieve symptoms and increase
survival. Certain symoptoms, such as flushing, can be relieved by octreotide which
decreases the secretion of serotonin. To prevent tumor growth interferon is given.
Patients should be told to avoid alcohol, large meals, and foods high in tyramine to
avoid triggering symptoms.

Prognosis

Depends on the site, grade, and stage of tumors. Surgically treated patients have an
overall favorable 83% 5-year survival rate.









1. Hande KR. Carcinoid syndrome. In Goldman L, Schafer AI, eds. Cecil Medicine. 24th ed.
Philadelphia, Pa: Saunders Elsevier; 2011: chap 240.

2. National Comprehensive Cancer Network. National Comprehensive Cancer Network Clinical
Practice Guidelines in Oncology: Neuroendocrine tumors. 2012. Version 1.2012
3. Shebani, Khaled O., Wiley W. Souba, Dianne M. Finkelstein, Paul C. Stark, Khaled M. Elgadi,
Kenneth K. Tanabe, and Mark J. Ott. "Prognosis and Survival in Patients With Gastrointestinal
Tract Carcinoid Tumors." Annals of Surgery 229.6 (1999): 815. Web.