Metastatic Gastrointestinal Carcinoid Tumors
Although the definitive role of surgery in metastatic disease has not been established, conservative resections of the intestine, mesenteric tumors, and fibrotic areas may improve symptoms and quality of life substantially in patients with metastatic hepatic, mesenteric, and peritoneal carcinoids. If the condition of the patient is such that surgery is not a greater risk than the disease, the primary tumor should be resected to prevent an emergency presentation with obstruction, perforation, or bleeding. Despite common acceptance that resection of at least 90% of the tumor burden is required to achieve palliation, approximately 60% of patients with surgery alone will experience symptom recurrence; the 5-year survival rate is between 35% and 80%, depending on the experience of the surgical center.[2,3] Because treatment with somatostatin analogs can achieve similar rates of symptom relief with fewer adverse effects, in each patient the benefits of surgical treatment of gastrointestinal (GI) carcinoid tumors should be weighed carefully against the potential risks of an open exploration. Tumor debulking, however, may potentiate pharmacologic therapy by decreasing the secretion of bioactive substances.
Management of hepatic metastases may include surgical resection; hepatic artery embolization; cryoablation and radiofrequency ablation; and orthotopic liver transplantation. (Refer to the Treatment of Hepatic Metastases section of the Treatment Option Overview section of this summary for more information.) Cytoreductive surgery for hepatic metastases from GI carcinoids can be performed safely with minimal morbidity and mortality resulting in regression of symptoms and prolonged survival in most patients. In one large review that included 120 carcinoid patients, a biochemical response rate of 96% and a 5-year survival rate of 61% were reported for patients whose hepatic metastases were resected surgically.[Level of evidence: 3iiDii]
In the case of liver metastases, localization and resection of the primary tumor may be considered, even among patients in whom the primary neoplasm is asymptomatic. In a retrospective study involving 84 patients, 60 of whom had their primary neoplasm resected, the resected group had a greater median progression-free survival (PFS) of 56 months, compared with 25 months of PFS for the primary nonresected group (P < .001). Median survival time for the resected group was longer at 159 months when compared with 47 months for the nonresected group (P < .001).[Level of evidence: 3iiDii ]
Although the response of carcinoid tumors to external-beam radiation therapy is very limited, palliative radiation therapy has some efficacy for bone and brain metastases and in the management of spinal cord metastases.
Treatment with single-agent chemotherapy or multiple-agent chemotherapy appears to be of little benefit in the management of GI carcinoids because no regimen has shown objective tumor response rates greater than 15%.
Treatment with radionuclides such as 131I-MIBG and 177Lu-octreotate may be of benefit. (Refer to the Radionuclides section of the Treatment Option Overview section of this summary for more information.)
Somatostatin analogs and interferon-alpha are the primary agents used in the treatment of carcinoid syndrome. (Refer to the Somatostatin Analogs section and Interferons section of the Treatment Option Overview section of this summary for more information.) Management of the symptoms of carcinoid syndrome may also include dietary modification and the use of various antidiarrheal agents, antihistaminics for skin rashes, and theophylline or beta-2 adrenergic receptor agonists for bronchospasm. (Refer to the Symptomatic Therapy section of the Treatment Option Overview section of this summary for more information.)
Information about ongoing clinical trials is available from the NCI Web site.
Current Clinical Trials
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with metastatic gastrointestinal carcinoid tumor. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
- Läuffer JM, Zhang T, Modlin IM: Review article: current status of gastrointestinal carcinoids. Aliment Pharmacol Ther 13 (3): 271-87, 1999. [PUBMED Abstract]
- McEntee GP, Nagorney DM, Kvols LK, et al.: Cytoreductive hepatic surgery for neuroendocrine tumors. Surgery 108 (6): 1091-6, 1990. [PUBMED Abstract]
- Plöckinger U, Rindi G, Arnold R, et al.: Guidelines for the diagnosis and treatment of neuroendocrine gastrointestinal tumours. A consensus statement on behalf of the European Neuroendocrine Tumour Society (ENETS). Neuroendocrinology 80 (6): 394-424, 2004. [PUBMED Abstract]
- Modlin IM, Latich I, Kidd M, et al.: Therapeutic options for gastrointestinal carcinoids. Clin Gastroenterol Hepatol 4 (5): 526-47, 2006. [PUBMED Abstract]
- Hodul P, Malafa M, Choi J, et al.: The role of cytoreductive hepatic surgery as an adjunct to the management of metastatic neuroendocrine carcinomas. Cancer Control 13 (1): 61-71, 2006. [PUBMED Abstract]
- Sarmiento JM, Heywood G, Rubin J, et al.: Surgical treatment of neuroendocrine metastases to the liver: a plea for resection to increase survival. J Am Coll Surg 197 (1): 29-37, 2003. [PUBMED Abstract]
- Givi B, Pommier SJ, Thompson AK, et al.: Operative resection of primary carcinoid neoplasms in patients with liver metastases yields significantly better survival. Surgery 140 (6): 891-7; discussion 897-8, 2006. [PUBMED Abstract]