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.