title: Prophylactic Laparoscopic Total Gastrectomy with Jejunal Pouch Reconstruction in Patients Carrying a CDH1 Germline Mutation.
abstract: BACKGROUND
  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.
  
  
  METHODS
  Patients with a germline CDH1 mutation who underwent prophylactic laparoscopic total gastrectomy with jejunal pouch were included in our prospective database.
  
  
  RESULTS
  A 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.
  
  
  CONCLUSIONS
  Prophylactic 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.
authors.affiliation: Department of Surgery, University Medical Center Utrecht, Heidelberglaan 100, 3584 CX, Utrecht, The Netherlands. leonie.haverkamp@gmail.com.
authors.forename: L
authors.initials: L
authors.lastname: Haverkamp
authors.affiliation: Department of Surgery, University Medical Center Utrecht, Heidelberglaan 100, 3584 CX, Utrecht, The Netherlands.
authors.forename: P C
authors.initials: PC
authors.lastname: van der Sluis
authors.affiliation: Department of Medical Genetics, University Medical Center Utrecht, Heidelberglaan 100, 3584 CX, Utrecht, The Netherlands.
authors.forename: M G E M
authors.initials: MG
authors.lastname: Ausems
authors.affiliation: Department of Surgery, University Medical Center Utrecht, Heidelberglaan 100, 3584 CX, Utrecht, The Netherlands.
authors.forename: S
authors.initials: S
authors.lastname: van der Horst
authors.affiliation: Department of Gastroenterology and Hepatology, University Medical Center Utrecht, Heidelberglaan 100, 3584 CX, Utrecht, The Netherlands.
authors.forename: P D
authors.initials: PD
authors.lastname: Siersema
authors.affiliation: Department of Surgery, University Medical Center Utrecht, Heidelberglaan 100, 3584 CX, Utrecht, The Netherlands.
authors.forename: J P
authors.initials: JP
authors.lastname: Ruurda
authors.affiliation: Department of Pathology, University Medical Center Utrecht, Heidelberglaan 100, 3584 CX, Utrecht, The Netherlands.
authors.forename: G J A
authors.initials: GJ
authors.lastname: Offerhaus
authors.affiliation: Department of Surgery, University Medical Center Utrecht, Heidelberglaan 100, 3584 CX, Utrecht, The Netherlands. r.vanhillegersberg@umcutrecht.nl.
authors.forename: R
authors.initials: R
authors.lastname: van Hillegersberg
mesh_terms: D000328:Adult; D015703:Antigens, CD; D015820:Cadherins / Q000235:genetics*; D018279:Carcinoma, Signet Ring Cell / Q000235:genetics / Q000473:pathology / Q000517:prevention & control*; D039021:Colonic Pouches; D005260:Female; D005743:Gastrectomy; D018095:Germ-Line Mutation / Q000235:genetics*; D006801:Humans; D010535:Laparoscopy; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D013274:Stomach Neoplasms / Q000235:genetics / Q000473:pathology / Q000517:prevention & control*; D055815:Young Adult
chemical_list: D015703:Antigens, CD; C513384:CDH1 protein, human; D015820:Cadherins
keywords: Cancer; E-cadherin; Gastrectomy; Laparoscopy; Prophylactic