What is the management of Carcinoid (Neuroendocrine) Tumor?

From the Guidelines

Management of carcinoid tumors requires a multidisciplinary approach tailored to tumor location, size, and metastatic status, with the primary goal of controlling symptoms, slowing tumor growth, and improving quality of life, as evidenced by the PROMID study which showed that octreotide LAR significantly delayed tumor progression in patients with metastatic midgut carcinoid tumors 1.

Key Considerations

• For localized disease, surgical resection is the primary treatment with the goal of complete tumor removal, as it can be curative and provide 5-year survival rates of 80-100% 1.
• For metastatic disease, somatostatin analogs like octreotide LAR (20-30 mg intramuscularly every 4 weeks) or lanreotide (90-120 mg subcutaneously every 4 weeks) are first-line treatments to control carcinoid syndrome symptoms and slow tumor growth, with the addition of short-acting octreotide (100-500 mcg subcutaneously 2-4 times daily) for breakthrough symptoms.
• Liver-directed therapies such as hepatic artery embolization or radiofrequency ablation may help control liver metastases, and targeted therapies like everolimus (10 mg daily) or peptide receptor radionuclide therapy (PRRT) with lutetium-177 dotatate may be considered for progressive disease despite somatostatin analogs.

Monitoring and Follow-up

• Regular monitoring with chromogranin A levels, 5-HIAA urine tests, and imaging studies is essential to assess treatment response and disease progression.
• Patients should also be monitored for carcinoid heart disease with echocardiography and managed for complications like intestinal obstruction or carcinoid crisis.

Treatment Options

• Surgery plays an important role in reducing tumor mass and can be performed before or concomitantly with medical treatment, with other means of cytoreductive procedures such as radiofrequency ablation, laser therapy, and embolization of liver metastases also being important 1.
• Biological treatment, such as somatostatin analogs and a-interferons, has proved effective in controlling associated clinical syndromes related to hormone production and release, and tumor-targeted radioactive treatment is an option in patients with tumors that present a high grade of uptake of In111-pentaoctreotide (Octreoscan) scintigraphy. The most effective treatment approach is often a combination of these options, tailored to the individual patient's needs and disease characteristics, with the goal of improving morbidity, mortality, and quality of life 1.

From the FDA Drug Label

Octreotide acetate injection is indicated 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. 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 management of carcinoid tumor involves the use of octreotide acetate injection for symptomatic treatment, specifically to suppress or inhibit severe diarrhea and flushing episodes associated with the disease 2.

• The recommended dosage range for carcinoid tumors is 100 mcg to 600 mcg daily in two to four divided doses during the initial 2 weeks of therapy 3.
• It is essential to note that octreotide acetate injection studies were not designed to show an effect on the size, rate of growth, or development of metastases 2.

From the Research

Management of Carcinoid Tumor

• The management of carcinoid tumors can be simple or complex, depending on the size and location of the tumor, as well as the presence of metastases 4.
• Somatostatin analogs, such as octreotide and lanreotide, are cornerstones in the symptomatic management of patients suffering from carcinoid tumors, and antiproliferative activity has also been reported for these agents 5.
• Surgical management of gastrointestinal carcinoid tumors involves local excision for incidental tumors, curative radical resection for symptomatic or incidentally discovered tumors, and palliative radical resection for patients with metastatic carcinoid tumors 4.
• A multidisciplinary approach is required for the management of giant bronchial carcinoid tumors, combining the expertise of surgeons, oncologists, anesthesiologists, and radiologists for effective results 6.
• Long-term treatment with somatostatin analogs, such as octreotide, can be effective in controlling carcinoid tumors, and the optimal dosage can be determined based on tumor marker levels 7.
• Somatostatin analogs are the standard of care for controlling symptoms of patients with functional gastroenteropancreatic neuroendocrine tumors (GEP-NETs), and have also been shown to have antiproliferative activity 8.

Treatment Options

• Octreotide and lanreotide are the most commonly used somatostatin analogs for the management of carcinoid tumors 5, 8.
• Surgical resection, including local excision and radical resection, is a treatment option for gastrointestinal carcinoid tumors 4.
• Liver dearterialization and peptide receptor radionuclide therapy (PRRT) are also treatment options for advanced carcinoid tumors 4, 8.
• A multidisciplinary approach, including surgery, medical therapy, and radiologic interventions, is necessary for the effective management of giant bronchial carcinoid tumors 6.

Symptomatic Management

• Somatostatin analogs, such as octreotide and lanreotide, can control symptoms in more than 70% of patients with carcinoid syndrome 8.
• Medical therapy, primarily octreotide, should be offered to patients with disseminated tumors, and should be administered and available during the perioperative period to patients undergoing resection or liver dearterialization 4.
• Symptomatic management of carcinoid tumors can also involve the use of other medications, such as anti-diarrheal agents and pain medications, to control symptoms such as diarrhea and pain.