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Total Gastrectomy for CDH-1 Mutation Carriers: An Institutional Experience.

Sandra R DiBrito¹, Alex B Blair¹, Vishnu Prasath¹

¹Department of Surgery, Johns Hopkins Medical Institutions, Baltimore, Maryland.

The Journal of Surgical Research

|November 6, 2019

Summary

This summary is machine-generated.

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Prophylactic gastrectomy in patients with CDH1 mutations prevents gastric cancer. This study shows low morbidity and good function after total gastrectomy with jejunal pouch reconstruction in this high-risk population.

Area of Science:

Oncology
Gastroenterology
Genetics

Background:

Gastric cancer is a significant global health issue.
Germline CDH1 gene mutations confer a high lifetime risk for diffuse gastric cancer.
Prophylactic gastrectomy is recommended for individuals with CDH1 mutations, but outcomes data are limited.

Purpose of the Study:

To describe the outcomes of total gastrectomy with intestinal pouch reconstruction in patients with CDH1 mutations.
To evaluate postoperative morbidity and weight loss in this cohort.

Main Methods:

A single-institution series of ten patients with confirmed CDH1 mutations who underwent total gastrectomy between 2010 and 2018.
Patients underwent either cancer-directed or prophylactic gastrectomy.
Outcomes assessed included presenting symptoms, preoperative screening, clinicopathologic features, weight loss, and postoperative complications.

Keywords:

CDH1 Familial gastric cancer Hereditary diffuse gastric cancer Prophylactic gastrectomy

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Main Results:

Eight of ten patients underwent prophylactic gastrectomy; three had occult gastric cancer detected pathologically.
Median weight loss was 10 kg at 6 months and 11 kg at 1 year post-surgery.
Postoperative morbidity was low, with one case each of anastomotic leak, hematoma, and pneumonia. All patients remained disease-free.

Conclusions:

Total gastrectomy with jejunal pouch reconstruction is an effective cancer-preventing operation for individuals with CDH1 mutations.
The procedure demonstrated encouragingly low postoperative morbidity and weight loss, with good functional outcomes.