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A PROSPECTIVE STRATEGY FOR MANAGEMENT OF ILEAL NETS WITH LIVER METASTASES (CARCINOID SYNDROME)
Andrea T. Fisher*, Jeffrey A. Norton
Surgery, Stanford University, Stanford, CA

Introduction
Ileal neuroendocrine tumors (NETs) are associated with small bowel obstruction, life-threatening liver metastases, and carcinoid syndrome. 16 years ago we instituted a prospective management strategy for treatment of these patients in which we resected all sites of tumor and treated with long-acting somatostatin analogues postoperatively.

Methods
17 patients had ileal NETs, lymph node and liver metastases, positive SRS scan, non-MEN-1, and no prior treatment. Each was given perioperative octreotide. Each was treated with complete tumor resection (R0) via extended right hemicolectomy/ileal resection, mesenteric lymph node dissection, liver wedge resection/lobectomy/microwave ablation, and cholecystectomy. Each was treated with postoperative octreotide LAR 30 mg IM Q 3-4 weeks to suppress tumor recurrence. Kaplan-Meier plots were used to estimate survival.

Results
Median follow-up was 52 months (range: 15-197 months). For each patient, elevated 24-hour urinary 5-HIAA levels and symptoms indicated preoperative carcinoid syndrome. With the use of perioperative octreotide no patient had any signs of carcinoid crisis. Median number of liver metastases was 4 (range: 1-15). Progression-free 5- and 10-year survival was 62% and 43%. 5- and 10-year overall survival was 83% and 57%. 6 (35%) patients had weight loss, and 1 (6%) patient required home total parenteral nutrition (TPN).

Conclusions
Aggressive surgery to resect primary, lymph node and liver metastatic ileal NETs followed by long-term administration of somatostain analogues achieves excellent progression-free and overall survival. It should be considered as the primary strategy for management of these patients.


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