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
For qualifying candidates, surgical resection of tumor and chemotherapy (5-FU and doxorubicin) can improve outcomes.
Surgery
Surgery, if feasible, is the only curative therapy. If the tumour has metastasized (most commonly, to the liver), the tumour 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 tumours 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.
In general 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.