Carcinoid syndrome surgery

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Parminder Dhingra, M.D. [2]

Overview

Surgery is the mainstay of treatment for carcinoid tumor. The feasibility of surgery depends on the stage of carcinoid tumor at diagnosis.

Surgery

Surgery, if feasible, is the only curative therapy. If the tumor has metastasized (most commonly, to the liver), the tumor may be ineligible for surgery (unresectable), though there are many promising treatment modalities, such as radiolabeled octreotide, that are very effective in arresting the growth of the tumors and prolonging survival in patients with liver metastases.

Gastric Carcinoids

• Type I gastric carcinoids: smaller than 1 cm are indolent with minimal risk for invasion and can be removed with endoscopic mucosal resection. Local surgical excision may be performed for rare larger or invasive tumors, but exceptional cases with large multifocal lesions may require gastric resection.
• Type II gastric carcinoids: because of their generally benign course similar to type I tumors, type II tumors can usually be managed with endoscopic resection (particularly for tumors <1 cm) followed by close endoscopic surveillance. Liberal surgical excision or gastric resection with regional lymphadenectomy is performed for larger and multifocal tumors or for those with deep wall invasion or angioinvasion. In patients with multiple tumors, somatostatin analog treatment may be used to reduce tumor growth, particularly if hypergastrinemia has not been reversed by surgery.
• Type III gastric carcinoids: behave more aggressively than type I and type II tumors, are treated with gastric resection and regional lymphadenectomy. Tumors larger than 2 cm or those with atypical histology or gastric wall invasion are most appropriately dealt with by gastrectomy or radical gastrectomy. Most of these tumors are metastatic at the time of presentation. The 5-year survival may approach 50%, but, in patients with distant metastases, it is only 10%.

Duodenal Carcinoids

• In general, endoscopic excision of primary duodenal carcinoids appears to be most appropriate for tumors smaller than 1 cm.
• Duodenal carcinoids smaller than 2 cm may be excised locally; for tumors between 1 cm and 2 cm, complete resection is ensured by operative full-thickness excision. Follow-up endoscopy is indicated.
• Tumors larger than 1 cm may be difficult to remove completely endoscopically and should be evaluated with endoscopic ultrasonography before endoscopic resection is attempted because of their potential to invade beyond the submucosa.
• Tumors larger than 2 cm can be treated with operative full-thickness excision and regional lymphadenectomy. Lymphadenectomy is performed even in the face of negative preoperative imaging because of the high rate of lymph node metastasis for these tumors.
• A regional lymphadenectomy includes the lymph nodes in the following locations:

• Posterior to the duodenum and pancreatic head and anterior to the inferior vena cava
• Posterolateral to the bile duct and portal vein
• Anterior to the common hepatic artery

Jejunal and Ileal Carcinoids

• At the time of diagnosis, 58% to 64% of patients with carcinoids of the small intestine have metastatic disease in the regional lymph nodes or the liver.
• Early surgical treatment should include removal of the mesentery by wedge resection and resection of lymph node metastases surrounding the mesenteric artery and vein to preserve intestinal vascular supply and to limit the intestinal resection.
• With grossly radical tumor resections, patients may remain symptom free for extended periods of time; however, because of the tenacity of carcinoid tumors, patients should undergo lifelong surveillance.
• Surgical treatment for advanced carcinoids involves prophylactic removal of mesenteric metastases early on because later the disease may become impossible to manage surgically.
• Repeat surgery may be necessary if mesenteric metastases are left during primary surgery or have progressed after primary surgery.
• These operations are difficult because of fibrosis between regions of the intestine, and surgery may result in fistulation, intestinal devascularization, or creation of a short bowel.
• In patients with inoperable metastatic disease, 5-year survival is approximately 50% for those with inoperable liver metastases and approximately 40% for those with inoperable liver and mesenteric metastases.

Appendiceal Carcinoids

• Approximately 90% of appendiceal carcinoids measure smaller than 1 cm and are not located in the appendiceal base, these tumors can be consistently cured by appendectomy.
• Appendiceal carcinoids larger than 2 cm require right-sided hemicolectomy and ileocecal lymphadenectomy because of the significant risk of metastasis.
• For tumors measuring 1 to 2 cm, treatment is controversial, but hemicolectomy may be appropriate if there is invasion in the mesoappendix, if there is residual tumor in the resection margins, or in the presence of lymph node metastases. For same-size lesions confined to the appendiceal wall, appendectomy alone may carry a low risk for metastases.
• Indications for hemicolectomy may include operative specimens that show high proliferative activity (high Ki67 index), high mitotic index, or signs of angioinvasion, but evidence is limited and histologic parameters for risk evaluation in appendiceal carcinoids measuring 1 cm to 2 cm requires definition.

Colonic Carcinoids

• Colonic carcinoid tumors are aggressive with a high proliferation rate, and they often present with more liver metastases than regional lymph node metastases.
• Colonic carcinoid tumors of the colon are treated similarly to adenocarcinoma of the colon.
• Attempts to achieve radical resection by hemicolectomy or subtotal colectomy with lymphadenectomy should be made, but frequently only debulking is possible.
• The overall 5-year survival rate is approximately 40% and is slightly worse than the survival rate for colon adenocarcinoma.

Rectal Carcinoids

• Rectal carcinoids should be evaluated by endoscopic ultrasound (EUS) or rectal magnetic resonance imaging (MRI).
• Tumors smaller than 1 cm can be safely removed by endoscopic excision. Excised specimens should be examined histologically to exclude muscularis invasion.
• Patients with tumors that are greater than 2 cm or that have invasion of the muscularis as seen by endoscopic ultrasound or MRI, surgical resection with abdominoperineal resection (APR) or low anterior resection (LAR) is recommended because of the high rate of nodal metastases and risk of distant metastatic disease.
• There is considerable debate about whether local excision or rectal resection (i.e., APR or LAR) is needed for tumors that are 1 cm to 2 cm.
• Although it may be possible to recognize tumors with particular atypia and high mitotic index before embarking on the more radical surgery, the presence of muscularis invasion or regional metastases generally supports rectal resection.

Metastatic Gastrointestinal Carcinoid Tumors

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.
• Management of hepatic metastases may include surgical resection; hepatic artery embolization, cryoablation and radiofrequency ablation, and orthotopic liver transplantation.
• 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.
• Palliative radiation therapy has some efficacy for bone and brain metastases and in the management of spinal cord metastases.

Recurrent gastrointestinal Carcinoid Tumors

Treatment of recurrent gastrointestinal carcinoid tumor depends on many factors:

• Previous treatment
• Site of recurrence
• Individual patient considerations

References

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