Malignant Carcinoid Syndrome Medication

Updated: Aug 14, 2019
  • Author: Luigi Santacroce, MD; Chief Editor: N Joseph Espat, MD, MS, FACS  more...
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Medication Summary

Currently, octreotide is considered the drug of choice worldwide for treating both carcinoids and related malignant syndromes. [55] Somatostatin is rarely administered because of its poor half-life. In patients with diffuse metastases, antiproliferative drugs may be useful for symptom palliation. [56]  

Telotristat ethyl should be considered in addition to somatostatin analog (SSA) therapy for diarrhea not controlled by SSA therapy. It is a tryptophan hydroxylase inhibitor that decreases biosynthesis of serotonin. [41]


Antisecretory/GI agents

Class Summary

These drugs are used to reduce blood levels of GH and IGF-I in patients with an inadequate response to surgery, radiation, and bromocriptine.

Octreotide (Sandostatin)

Acts primarily on somatostatin receptor subtypes II and V. Inhibits GH secretion and has many other endocrine and nonendocrine effects, including inhibition of glucagon, VIP, and GI peptides.

Telotristat ethyl (Xermelo)

Telotristat, the active metabolite of telotristat ethyl, inhibits tryptophan hydroxylase, which mediates the rate limiting step in serotonin biosynthesis. Serotonin plays a role in mediating secretion, motility, inflammation, and sensation of the GI tract, and is overproduced in patients with carcinoid syndrome. It is indicated for carcinoid syndrome diarrhea in combination with somatostatin analog (SSA) therapy in adults inadequately controlled by SSA therapy.


Antineoplastic agents

Class Summary

These agents inhibit cell growth and proliferation.

Doxorubicin (Adriamycin)

Anthracycline antibiotic that can intercalate with DNA, affecting many DNA functions, including synthesis. Administered IV and distributes widely into bodily tissues, including the heart, kidneys, lungs, liver, and spleen. Does not cross blood-brain barrier and is excreted primarily in bile. May be helpful in symptom palliation for patients with progressive disease.

Streptozocin, streptozotocin (Zanosar)

Cell-cycle phase-nonspecific antineoplastic agent that alkylates DNA, causing interstrand cross-linking. Also inhibits DNA synthesis by blocking incorporation of DNA precursor and inhibiting cell proliferation. May be helpful in symptom palliation for patients with progressive disease. Dosage is related to body surface area. May cause a complete remission of disease. Administration must be suspended only when desired response or toxicity occurs. Streptozocin may determine severe nephrotoxic effects.

Fluorouracil, 5-FU (Adrucil)

Fluorinated pyrimidine antimetabolite that inhibits thymidylate synthase (TS) and also interferes with RNA synthesis and function. Has some effect on DNA. Useful in symptom palliation for patients with progressive disease.

Cisplatin (Platinol)

Inhibits DNA synthesis and thus cell proliferation by causing DNA crosslinks and denaturation of double helix. May help with symptom palliation for patients with progressive disease.

Etoposide (Toposar, VePesid)

Inhibits topoisomerase II and causes DNA strand breakage, causing cell proliferation to arrest in the late S or early G2 portion of the cell cycle. May help with symptom palliation for patients with progressive disease.



Class Summary

Interferons are naturally produced proteins with antiviral, antitumor, and immunomodulatory actions. Alpha, beta, and gamma interferons may be given topically, systemically, and intralesionally.

Interferon alfa 2a (Roferon-A, Intron-A)

Protein product manufactured by recombinant DNA technology. Mechanism of antitumor activity is not clearly understood; however, direct antiproliferative effects against malignant cells and modulation of host immune response may play important roles. Therapeutic trials in selected patients.


H1 antihistamines

Class Summary

These agents act by competitive inhibition of histamine at the H1 receptor. This mediates the wheal and flare reactions, bronchial constriction, mucous secretion, smooth-muscle contraction, edema, hypotension, CNS depression, and cardiac arrhythmias.

Cyproheptadine (Periactin)

Competitively inhibits H1 receptor, which mediates bronchial constriction, smooth-muscle contraction, edema, hypotension, CNS depression, and cardiac arrhythmias. Prevents histamine release in blood vessels and is more effective in preventing histamine response than in reversing it. May be useful in patients with syndromes sustained by histamine-producing tumors.


H2-receptor antagonists

Class Summary

The combination of H1 and H2 antagonists may be useful in chronic idiopathic urticaria not responding to H1 antagonists alone. They may also be useful for itching and flushing in anaphylaxis, pruritus, urticaria, and contact dermatitis (IV).

Ranitidine (Zantac)

Competitive and reversible H2-receptor blockers. Highly selective antagonists that do not affect the H1 receptors and may be administered contemporary to H1-receptor antagonists. May be useful for treatment of severe itching, flushing, and urticaria.


Alpha2-adrenergic agonists

Class Summary

These agents improve the hemodynamic status by increasing myocardial contractility and heart rate, resulting in increased cardiac output. They are useful in reducing some symptoms of malignant carcinoid syndrome (eg, diarrhea, hypertension, tachycardia).

Clonidine (Catapres)

Stimulate alpha2 adrenoreceptors in brain stem, activating an inhibitory neuron, which, in turn, results in reduced sympathetic outflow. These effects result in a decrease in vasomotor tone and heart rate.