Abstract

Introduction: Advanced mesenteric desmoid tumors involving retroperitoneal organs are considered unresectable and fatal disease. Quality of life of patients is significantly impaired with enteric fistulae, ureteric obstruction, long-term chemotherapy and pain with narcotics requirement. Debulking of mesenteric desmoid tumor aims to prolong patient survival, improve quality of life, and preconditioning for visceral transplantation. The aim of this paper is to discuss a safe resection technique involving piecemeal enterectomy with tumor debulking. Methods: Between 2013-2017, 7 patients with advanced mesenteric tumor underwent debulking with a median age of 45 years. The median age of diagnosis was 20 years. Four patients underwent extensive chemotherapy for a median of 2.5 years. The clinical course was complicated with enteric complications in all patients such as fistulae and obstruction, and with ureteric obstruction in 3 patients. At referral, all patients were on narcotic treatment, with abdominal drains in 3 patients, nephrostomy in 2 patients, and median number of surgeries of 3.5. To strategize the surgical resection, we defined the resection plane as 1 cm anterior to the posterior parietal peritoneum through the tumor. Enterectomies were performed at this plane in a piecemeal fashion to avoid injury to the great vessels, followed by resection of residual tumors below the resection plane around aorta, vena cava, ureters, and pelvic organs, followed by ureteric reconstruction. Results: The tumor was involving the aorta, vena cava, iliac vessels, and ureters in 7 patients. Small tumor remnants were left to create enterostomy safely due to duodenal/pancreatic infiltration in 5 patients, and was planned to be resected at the time of transplant. Three patients underwent ureteric reconstruction by urologists. Near complete resection was accomplished with a median of 14 hours. Median length of stay was 16.5 days, with a median chemotherapy-free period of 11 months. The need of narcotics was significantly reduced, and all patients were free of percutaneous drains or nephrostomies. Conclusion: Piecemeal enterectomy and tumor debulking of advanced mesenteric desmoid tumors is a safe procedure with careful attention of the retroperitoneal structures. It needs advanced setup with urology involvement. The results proved marked improvement of quality of life, freedom from pain, chemotherapy, and abdominal drains even in patients who are not qualified for transplant.

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