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Prophylactic Total Gastrectomy and Roux-en-Y Reconstruction with a Stapled Side-to-Side Esophagojejunostomy for Germline CDH1 Mutation: A Single Surgeon’s Experience
Kevin K. Chang*, Sam Yoon
Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, NY
Introduction: Germline mutation of the CDH1 gene, which encodes for E-cadherin, confers an estimated lifetime risk of greater than 80% for developing Lauren diffuse-type gastric adenocarcinoma. Prophylactic total gastrectomy is the recommended procedure for patients who can tolerate the operation. Prior studies of prophylactic total gastrectomy have reported anastomotic leak rates of up to 26% and significant morbidity including anastomotic stricture.
Methods: Retrospective review was performed of a prospective database of patients with germline CDH1 mutation. All patients underwent prophylactic total gastrectomy with Roux-en-Y reconstruction. The esophagus was transected with a linear stapler [Endo GIATM Ultra Universal Stapler with 45mm Articulating Medium/Thick (Purple) Reload; Covidien, Mansfield, MA], and an esophagostomy was created in the midportion of the staple line. A side-to-side esophagojejunostomy was created between the antimesenteric wall of the Roux limb and the posterior wall of the esophagus using the same Endo GIA stapler. The common enterotomy was closed with hand-sewn, interrupted 3-0 silk sutures (modified Orringer technique).
Results: Thirty-one patients with germline CDH1 mutation confirmed by genetic testing underwent prophylactic total gastrectomy between April 2006 and July 2015 by a single surgeon. Median age was 41 years (range 16-58), and 22 patients (77%) were female. Median operative time was 193 minutes, and median estimated operative blood loss was 175 mL. No patient experienced a post-operative anastomotic leak as documented by esophagram (performed in 30 of 31 patients) or clinical symptoms. One patient (3%) developed an anastomotic stricture requiring endoscopic dilation. Median length of stay was 8 days (range 7-12 days). The 30-day morbidity rate was 10% (one superficial surgical site infection, one pulmonary embolism, one episode of pancreatitis), and the 30-day mortality rate was 0%. At least one focus of intramucosal and/or early invasive carcinoma was present in 29 resected specimens (94%). Median nadir weight loss was 16% (range 1-43%) from baseline weight. No patients developed recurrent disease.
Conclusions: Prophylactic total gastrectomy is the definitive procedure for individuals with germline CDH1 mutation. In these usually healthy and relatively young patients, an experienced surgeon can perform the operation using a stapled side-to-side esophagojejunostomy technique with anastomotic leak and stricture rates of less than 5%.
Table: Demographic and Clinicopathologic Characteristics in Patients With Germline CDH1 Mutations Who Underwent Prophylactic Total Gastrectomy
| | Prophylactic Total Gastrectomy (n = 31) | | | n or median (% or range) | | Age (y) | 41 (16-58) | | Gender: female | 22 (71.0) | | Body mass index | 25.6 (18.0-41.8) | | Gastrointestinal symptoms at presentation | 10 (32.3) | | Family history: gastric cancer | 31 (100.0) | | Family history: CDH1 mutation | 28 (90.3) | | Family history: breast cancer | 13 (41.9) | | Operative time (min) | 193 (124-308) | | Estimated intraoperative blood loss (mL) | 175 (50-1000) | | Length of Roux limb (cm) | 55 (50-70) | | Jejunal pouch | 1 (3.2) | | Length of stay (d) | 8 (7-12) | | Anastomotic leak | 0 (0.0) | | Anastomotic stricture | 1 (3.2) | | 30-day morbidity | 3 (9.7) | | 30-day mortality | 0 (0.0) | | Weight loss (nadir % from baseline) | 16.0 (0.6-43.2) | | Presence of microscopic foci of carcinoma | 29 (93.5) | | Lymph nodes resected (n) | 12 (1-25) |
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