Carcinoid syndrome surgery

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

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

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.

• Type I gastric carcinoids: smaller than 1 cm can be removed with endoscopic mucosal resection. Local surgical excision may be performed for larger tumors.^[1][2]

• Type II tumors can usually be managed with endoscopic resection (tumors <1 cm) followed by close endoscopic surveillance.^[3]
• For larger or multifocal tumours liberal surgical excision or gastric resection with regional lymphadenectomy is performed.^[4]
• Type II carcinoids tumor can usually be managed with endoscopic resection (tumors <1 cm) followed by close endoscopic surveillance.
• For larger or multifocal tumors liberal surgical excision or gastric resection with regional lymphadenectomy is performed.
• For type III gastric carcinoids larger than 2 cm or those with atypical histology or gastric wall invasion are most appropriately dealt with by gastrectomy or radical gastrectomy.

• Primary duodenal carcinoids smaller than 1 cm are treated by endoscopic excision.

• Duodenal carcinoids smaller than 2 cm may be excised locally. 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.^[5]
• Tumors larger than 2 cm can be treated with operative full-thickness excision and regional lymphadenectomy.^[5]
• 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

• Carcinoids of the small intestine have metastatized in the regional lymph nodes or the liver in 50%-60% of cases^[1]
• 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.^[6]
• Surgical treatment for advanced carcinoids involves prophylactic removal of mesenteric metastases early on because later the disease may become impossible to manage surgically.
• Due to fibrosis between regions of the intestine,surgery may result in fistulae, intestinal devascularization, or creation of a short bowel.

• Appendiceal carcinoids smaller than 1 are cured by appendectomy.^[6]
• Appendiceal carcinoids larger than 2 cm require right-sided hemicolectomy and ileocecal lymphadenectomy because of the significant risk of metastasis.^[1]
• For tumors between 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.

• Colonic carcinoid tumors of the colon are treated similarly to adenocarcinoma of the colon.
• Radical resection by hemicolectomy or subtotal colectomy with lymphadenectomy should be done for colonic carcinoids tumors.

• Tumors smaller than 1 cm can be removed by endoscopic excision but excised specimens should be examined histologically to exclude muscularis invasion.^[7]
• 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.

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.^[1][8]

• The primary tumor should be resected to prevent an emergency presentation with obstruction, perforation, or bleeding if the condition of the patient is such that surgery is not a greater risk than the disease
• Management of hepatic metastases may include:

1. Surgical resection
2. Hepatic artery embolization
3. Cryoablation
4. Radiofrequency ablation
5. 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.

Treatment of recurrent gastrointestinal carcinoid tumor depends on many factors:^[1]

• Previous treatment
• Site of recurrence
• Individual patient considerations

• Valve replacement is the most effective treatment option for advanced carcinoid heart disease.“Carcinoid Valve Disease | SpringerLink”.^[9]

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