Prophylactic Laparoscopic Total Gastrectomy with Jejunal Pouch Reconstruction in Patients Carrying a CDH1 Germline Mutation

L Haverkamp,P.C Van Der Sluis,M.G.E.M Ausems,S Van Der Horst,G J A Offerhaus,P.D Siersema,M G E M Ausems,J.P Ruurda,P C Van Der Sluis,G.J.A Offerhaus,R Van Hillegersberg

doi:10.1007/s11605-015-2963-4

L Haverkamp, P.C Van Der Sluis + Show 9 more

Open Access

https://doi.org/10.1007/s11605-015-2963-4

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Abstract

BackgroundFor patients with an identified germline E-cadherin-1 (CDH1) mutation, prophylactic gastrectomy is the treatment of choice to eliminate the high risk of developing diffuse gastric cancer. Laparoscopic total gastrectomy with jejunal pouch reconstruction is a novel approach that may be especially suitable in these patients. MethodsPatients with a germline CDH1 mutation who underwent prophylactic laparoscopic total gastrectomy with jejunal pouch were included in our prospective database. ResultsA total of 11 patients with a median age of 40 (22–61) years were included. The average operative time was 4:26 ± 0:49 h and the average blood loss was 219 ± 155 ml. Median length of hospital stay was 10 (7–27) days. In two patients, an esophagojejunal anastomotic leakage occurred (grade 4). The leakages were seen in patient numbers 2 and 3, which may be a result of a learning curve. The latter eight patients did not develop anastomotic leakage. Pulmonary complications occurred in one patient with atelectasis and in one patient with pneumonia (grade 2). The 60-day mortality rate was 0 %. Multiple foci of intramucosal diffuse gastric signet ring cell carcinoma were found in the resection specimen of 9/11 (82 %) patients. All 11/11 (100 %) resections were microscopically radical. ConclusionsProphylactic laparoscopic total gastrectomy with jejunal pouch reconstruction in patients with a CDH1 germline mutation is feasible and safe. In 82 % of patients, foci of intramucosal diffuse gastric signet ring cell carcinoma in the resection specimen were found.

Highlights

The estimated number of newly diagnosed gastric cancer patients is 989,600 worldwide, accounting for 738,000 cancerrelated deaths.[1]
We describe our initial experience with prophylactic laparoscopic total gastrectomy with jejunal pouch in a case series of patients carrying a CDH1 germline mutation
Patients (n=11) with identified germline CDH1 mutations who were referred to the University Medical Centre Utrecht for prophylactic total gastrectomy between April 2006 and May 2015 were included in this study

Summary

Introduction

The estimated number of newly diagnosed gastric cancer patients is 989,600 worldwide, accounting for 738,000 cancerrelated deaths.[1]. Hereditary diffuse gastric cancer represents 1–3 % of all gastric cancers.[2] In approximately 30 % of families with hereditary diffuse gastric cancer, germline mutations are found in the Ecadherin-1 (CDH1) tumor suppressor gene.[3,4,5,6] The CDH1 gene codes for the E-cadherin protein, and loss of function of the CDH1 gene leads to diffuse gastric cancer. Patients who carry CDH1 germline mutations have a lifetime risk of >70 % of. For patients with an identified germline E-cadherin-1 (CDH1) mutation, prophylactic gastrectomy is the treatment of choice to eliminate the high risk of developing diffuse gastric cancer. Laparoscopic total gastrectomy with jejunal pouch reconstruction is a novel approach that may be especially suitable in these patients

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