From the Guidelines
Carcinoid syndrome is primarily treated with somatostatin analogs, such as octreotide or lanreotide, which alleviate symptoms in 70-80% of patients. The symptoms of carcinoid syndrome include facial flushing, diarrhea, wheezing, abdominal cramping, and heart valve damage due to excess serotonin and other hormones released by carcinoid tumors.
Treatment Options
- Somatostatin analogs: Octreotide (typical starting dose 100-150 mcg subcutaneously three times daily, or long-acting Sandostatin LAR 20-30 mg intramuscularly every 4 weeks) or lanreotide (120 mg deep subcutaneous injection every 4 weeks) are the primary treatment options for carcinoid syndrome, as they block hormone release and alleviate symptoms in 70-80% of patients 1.
- Breakthrough symptoms: Short-acting octreotide can be used as needed to manage breakthrough symptoms.
- Additional medications: Telotristat ethyl (250 mg three times daily with food) can be used to reduce diarrhea by inhibiting serotonin production, and antihistamines or leukotriene inhibitors can be used to manage flushing.
- Surgical removal: Surgical removal of the primary tumor and metastases is recommended when possible.
- Hepatic-directed therapies: Hepatic-directed therapies like embolization or ablation may help when liver metastases are present.
Management
- Avoid triggers: Patients should avoid triggers like alcohol, spicy foods, and strenuous exercise which can precipitate symptoms.
- Monitoring: Long-term management requires monitoring for complications such as carcinoid heart disease, which affects the right heart valves and may eventually require valve replacement. According to the most recent and highest quality study, the therapeutic approach in NETs with carcinoid syndrome involves somatostatin analogs, debulking surgery, radiofrequency ablation, and locoregional therapies 1.
From the FDA Drug Label
Carcinoid Tumors: For the symptomatic treatment of patients with metastatic carcinoid tumors where it suppresses or inhibits the severe diarrhea and flushing episodes associated with the disease. (1.2)
The symptoms of carcinoid syndrome include severe diarrhea and flushing episodes. Treatment options for carcinoid syndrome include octreotide acetate injection, which is used for the symptomatic treatment of patients with metastatic carcinoid tumors to suppress or inhibit these symptoms 2.
From the Research
Symptoms of Carcinoid Syndrome
- Flushing, diarrhea, hypotension, tachycardia, bronchoconstriction, venous telangiectasia, dyspnea, and fibrotic complications (mesenteric and retroperitoneal fibrosis, and carcinoid heart disease) are the main signs and symptoms associated with carcinoid syndrome 3
- Atypical signs and symptoms can include wheezing, abdominal pain, valvular heart disease, telangiectasias, pellagra, and the complications of mesenteric fibrosis, including ureteral obstruction, bowel obstruction, and bowel ischemia 4
- The classic "carcinoid triad" associated with the syndrome includes flushing, diarrhea, and cardiac involvement 5
Treatment Options for Carcinoid Syndrome
- Somatostatin analogues, such as lanreotide and octreotide, effectively control flushing and diarrhoea in patients with the carcinoid syndrome 6, 5, 7
- Lanreotide, 30 mg intramuscularly every 14 days, is an effective and convenient treatment in patients with the carcinoid syndrome 6
- Surgery and medical management with somatostatin analogs are treatment options for carcinoid syndrome 4
- Antiproliferative medical strategies to control the growth of metastatic carcinoid tumors include long-acting somatostatin analogs, interferon alpha, and the combination of the two 7
- Chemotherapy, including etoposide and cisplatin, has been shown to be effective only for purely differentiated neuroendocrine carcinomas and not for slowly growing carcinoids 7