The goal of this research was to describe how pathogenic CDH1 variant carriers (pCDH1vc) are treated by the FREGAT (FRench Eso-GAsTric tumor) network. Clinical and pathological results were the primary emphasis, whereas identifying risk factors for postoperative morbidity (POM) was a secondary goal. In pCDH1vc, endoscopic surveillance is an alternative to prophylactic total gastrectomy (PTG) to prevent gastric cancer. Between 2003 and 2021, researchers from multiple institutions conducted a retrospective, observational study. Median IQR or count data were shown (proportion). Univariate analysis was conducted using standard tests. Binary logistic regression was used to identify risk factors for POM and for severe POM (Clavien-Dindo grade 3 or higher). There were 99 patients, 14 of which were considered “index cases,” reported from 11 different locations. The majority of index cases (71.4%) had advanced peritoneal carcinomatosis at diagnosis and had a median survival time of 12.0 (7.6-16.4) months. About 77 (median age=34.6 (23.7-46.2), American Society of Anesthesiologists score 1: 75%) of the remaining 85 patients underwent a PTG, with the majority (51.9%). Age 40 or more and low-volume centers were predictors of POM (P=0.030 and 0.038, respectively), with the POM rate totaling 37.7% and including 20.8% of severe POM. The percentage of cancer found in specimens after PTG was 54.5% (n=42, all pT1a), but preoperative endoscopy found malignancy in only 59.5% of patients (n=25). Index instances of pCDH1vc have a very grim prognosis. PTG patients continue to face a significant and unexpected cancer risk, which must be weighed against the procedure’s potential morbidity and functional consequences.

Source: journals.lww.com/annalsofsurgery/Abstract/2022/11000/Management_of_Pathogenic_CDH1_Variant_Carriers.13.aspx