FAST FACT AND CONCEPT #137: Carcinoid Syndrome - Symptom Management

Authors: Stacey Shaffer and Robert Arnold

The Carcinoid Syndrome (CS) is a symptom complex resulting from hormone secretion in patients with carcinoid tumors. Carcinoid tumors typically grow slowly, thus patients may live for many years following diagnosis. CS becomes manifest only when sufficient concentration of hormones reach the systemic circulation, most commonly in the presence of liver metastases. This Fast Fact will focus on managing the symptoms of Carcinoid Syndrome.

The CS includes the complex of: flushing, diarrhea, abdominal cramping, cyanosis, bronchoconstriction and symptoms of right heart failure. Specific symptoms depend on the hormones the tumor secretes. Serotonin-secreting tumors cause diarrhea and cramping; bradykinin and histamine secretion lead to flushing and cyanosis. Carcinoid tumors may also produce somatostatin, norepinephrine, dopamine, gastrin, vasoactive intestinal peptide, etc. Drugs that block the hormonal secretion can help to control the symptoms of carcinoid syndrome.

Somatostatin analogs

These drugs are the treatment of choice for CS. Three formulations are available: Octreotide (continuous infusion or 50-500 m g tid, IV/SQ), Lanreotide (30 mg every other week, IM), and Sandostatin LAR (20 mg monthly, IM). 50-70% of patients experience a significant reduction of diarrhea and flushing episodes within seven days. Efficacy and side effect profiles for the three preparations are similar. Side effects include pain at the injection site, abdominal bloating, fatigue, transient fever, elevated serum glucose, and asymptomatic biliary lithiasis.

Interferon

Interferon alpha is effective in controlling both diarrhea and flushing, although it is inferior to the somatostatin analogs. The dose is 3-9 mU SQ three to seven times per week. Interferon alpha therapy is often limited by its side effects: fever, anemia, thrombocytopenia, neutropenia, fatigue, depression, and flu-like symptoms.

Other Drugs/Symptom Specific Treatments

• Diarrhea--Cyproheptadine is an alternative treatment of carcinoid-associated diarrhea; 60% of patients report improvement within one week. The dosage is 4 mg TID given orally as a tablet; it can be titrated up to 0.5 mg/kg per day. Side effects include sedation, dry mouth, dizziness, mild blurring of vision, nausea, and vomiting. Loperamide and opioids are non-specific anti-diarrheal agents that can be used for mild symptoms.
• Wheezing—bronchodilators
• Heart failure—diuretics; tricuspid valve replacement

Non-Drug Treatments

Patients should be counseled to identify and eliminate stressors that reproducibly cause symptoms—this may include specific stressful situations, foods or alcohol.

Other

Other treatments for refractory symptoms include systemic chemotherapy, hepatic artery embolization or hepatic chemoembolization or debulking surgery of hepatic metastases.

References

1. Kulke, MH, Mayer, RJ. Medical progress: carcinoid tumors. N Engl J Med 1999; 340:858.
2. di Bartololmeo, M, Bajetta, E, Buzzoni, R, et al. Clinical efficacy of octreotide in the treatment of metastatic neuroendocrine tumors. A study by the Italian Trials in Medical Oncology Group. Cancer 1996; 77:402.
3. Ruszniewski, P, Ducreux, M, Chayvialle, J, et al. Treatment of carcinoid syndrome with the longacting somatostatin analogue lanreotide: a prospective study in 39 patients. Gut 1996; 39:279.
4. Garland, J, Buscombe, JR, Bouvier, C, et al. Sandostatin LAR (long-acting octreotide acetate) for malignant carcinoid syndrome: a 3-year experience. Aliment Pharmacol Ther 2003; 17:437.
5. Moertel, CG, Kvols, LK, Rubin, J. A study of cyproheptadine in the treatment of metastatic carcinoid tumor and the malignant carcinoid syndrome. Cancer 1991; 67:33.
6. Oberg, K, Funa, K, Alm, G. Effects of leukocyte interferon on clinical symptoms and hormone levels in patients with mid-gut carcinoid tumors and carcinoid syndrome. N Engl J Med 1983; 309:129.
7. Sitaraman, SV, Goldfinger, SE. Treatment of carcinoid tumors and the carcinoid syndrome. UpToDate 2004.

Copyright/Referencing Information : Users are free to download and distribute Fast Facts for educational purposes only. Citation for referencing: Shaffer S and Arnold R . Carcinoid syndrome. Fast Facts and Concepts #137: May 2005. End-of-Life / Palliative Education Resource Center www.eperc.mcw.edu.

Fast Facts were edited by David Weissman MD, Palliative Care Center, Medical College of Wisconsin until January 2007.  For comments/questions write to the current editor, Drew Rosielle MD: drosiell@mcw.edu. The complete set of Fast Facts is available at EPERC: www.eperc.mcw.edu

Disclaimer: Fast Facts provide educational information, this information is not medical advice. Health care providers should exercise their own independent clinical judgment. Some Fast Fact information cites the use of a product in dosage, for an indication, or in a manner other than that recommended in the product labeling. Accordingly, the official prescribing information should be consulted before any such product is use.

Creation Date: 5/2005

Purpose: Self-Study Guide, Teaching

Audience(s)

ACGME Competencies: Medical Knowledge , Patient Care

Keyword(s): Non pain symptoms & syndromes; cancer

Specific Disease and Organ System Category(s): Cancer