Prophylactic laparoscopic gastrectomy for hereditary diffuse gastric cancer: a case series in a single family

JSLS. Jul-Sep 2010;14(3):348-52. doi: 10.4293/108680810X12924466007449.

Abstract

Background: Hereditary diffuse gastric carcinomas (HDGCs) are particularly troubling because of autosomal dominant heritance, high penetrance, early age of onset, and a lack of effective treatment once symptomatic. HDGC is further complicated by difficulty of effective screening. Gastrectomy provides definitive treatment for CDH1 mutation-positive patients. Attempting to minimize the morbidity and mortality of this procedure via a laparoscopic approach is appropriate.

Methods: Six consanguineous patients, 21 to 51 years of age, were identified as carriers of the CDH1 gene mutation. All of the patients' gastric mucosa was normal by endoscopic appearance and biopsy. After appropriate multispecialty counseling, all patients elected to undergo a laparoscopic total gastrectomy. Demographics, genealogy, operative approach, outcomes, and pathology were reviewed.

Results: All gastrectomies were completed using a laparoscopic approach. Gross examination of resected stomachs was unremarkable. Histological examination demonstrated multiple foci of invasive signet ring adenocarcinoma in all patients. There were no anastomotic leaks, one small bowel obstruction requiring reoperation, and one esophageal stricture requiring dilation.

Conclusions: This series demonstrates the utility and safety of the laparoscopic approach for prophylactic total gastrectomy for carriers of the CDH1 gene mutation. It serves to highlight that patients with CDH1 mutations may be more likely to undergo gastrectomy if they are offered the lower risk laparoscopic approach.

Publication types

  • Comparative Study

MeSH terms

  • Adult
  • Family*
  • Female
  • Follow-Up Studies
  • Gastrectomy / methods*
  • Genetic Predisposition to Disease*
  • Humans
  • Male
  • Middle Aged
  • Pedigree
  • Stomach Neoplasms / genetics
  • Stomach Neoplasms / prevention & control*
  • Treatment Outcome
  • Young Adult