Carcinoid syndrome medical therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Parminder Dhingra, M.D. [2]

Overview

For symptomatic relief of carcinoid sydromes medical therapy many include: octreotide, methysergide maleate, and cyproheptadine.

Medical Therapy

Standard treatments for patients with gastrointestinal (GI) carcinoid tumors include the following:

• Surgery
• Somatostatin analogs
• Interferons
• Treatment of hepatic metastases
• Radionuclides
• Management of carcinoid-related fibrosis

Symptomatic therapy Symptomatic relief may be provided by any of the following medical therapies:

• Octreotide (somatostatin analogue- neutralizes serotonin and decreases urinary 5-HIAA)
• Methysergide maleate (antiserotonin agent but not used because of serious side effect of retroperitoneal fibrosis)
• Cyproheptadine (antihistamine)

Somatostatin Analogs

The development of long-acting and depot formulations of somatostatin analogs has been important in the amelioration of symptoms of carcinoid syndrome. The result has been a substantial improvement in quality of life with relatively mild adverse effects. Experimentally, somatostatin has been shown to have a cytostatic effect on tumor cells. This effect involves hyperphosphorylation of the retinoblastoma gene product and G1 cell cycle arrest, in addition to somatostatin receptor (SSTR) subtype 3 [sst(3)]-mediated (and to a lesser extent, SSTR subtype [sst(2)]-mediated) apoptosis. Somatostatin also appears to have some antiangiogenic properties. However, only a small number of patients treated with somatostatin analog therapy experience partial tumor regression.

Octreotide, a short-acting somatostatin analog and the first biotherapeutic agent used in the management of carcinoid tumors, exhibits beneficial effects that are limited to symptom relief, with about 70% of patients experiencing resolution of diarrhea or flushing.

In the treatment of carcinoids, lanreotide, a long-acting somatostatin analog administered every 10 to 14 days, has an efficacy similar to that of octreotide and an agreeable formulation for patient use.[13] The effects of lanreotide on symptom relief are comparable to those of octreotide, with 75% to 80% of patients reporting decreased diarrhea and flushing; however, there appears to be little improvement in tumor responses over shorter-acting octreotide.

Depot formulations include long-acting repeatable (LAR) octreotide and a slow-release depot preparation of lanreotide.

Chemotherapy

Chemotherapy is of little benefit and is generally not indicated. Octreotide (a somatostatin analogue) may decrease the secretory activity of the carcinoid.

References

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