Table 3. Carcinoid Syndrome–Specific Therapies.

Agent class	MOA	Treatment	Indication	ROA	Schedule
SSAs	Binds to somatostatin receptors to mimic somatostatin inhibition of various endocrine, neuroendocrine, exocrine, and paracrine functions	Octreotide acetate	Symptomatic treatment of severe diarrhea and cutaneous flushing episodes associated with metastatic carcinoid tumors	Subcutaneously or intravenously	• First 2 weeks of treatment: 100–600 μg/d in 2–4 divided doses (mean daily dosage: 300 μg)
		Octreotide LAR	Severe diarrhea and cutaneous flushing episodes associated with metastatic carcinoid tumors in patients who responded and tolerated octreotide acetate	Intramuscular	• 30 mg q4wka
					• Consider dose reducing to 20 mg q4wk for patients who are frail or do not tolerate therapy secondary to side effects
		Lanreotide	Adult patients with unresectable, well- or moderately differentiated, locally advanced or metastatic GEP-NETs to improve PFS; adults with carcinoid syndrome to reduce frequency of short-acting somatostatin analog rescue therapy	Deep subcutaneous	• 120 mg q4wk
					• Consider dose reducing to 90 mg q4wk for patients who are frail or do not tolerate full dose secondary to side effects
Tryptophan hydroxylase inhibitor	Inhibits tryptophan hydroxylase, which catalyzes the rate-limiting step in serotonin production	Telotristat ethyl	Carcinoid syndrome diarrhea in combination with SSAs in adults inadequately controlled by SSAs	Oral	• 250 mg po 3 times per day with food

Note. MOA = mechanism of action; ROA = route of administration; SSA = somatostatin analog; GEP-NETs = gastroenteropancreatic neuroendocrine tumors; PFS = progression-free survival; po = by mouth; q4wk = every 4 weeks. Information from Ipsen Pharma Biotech (2018); Novartis Pharmaceuticals Corp. (2008, 2016); Lexicon Pharmaceuticals, Inc. (2017).

^a30 mg q4wk is the dose most commonly used.