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. 2022 Dec;126(8):1413-1422.
doi: 10.1002/jso.27084. Epub 2022 Sep 5.

Short and long-term outcomes of prophylactic total gastrectomy in 54 consecutive individuals with germline pathogenic mutations in the CDH1 gene

Mason D Stillman  1 , Nicole Kusche  1 , Sabrina Toledano  2 , Kimberly J Hilfrank  2 , Changhwan Yoon  1 , Joel T Gabre  2   3 , Sheila D Rustgi  2 , Chin Hur  2   3 , Fay Kastrinos  2   3 , Sandra W Ryeom  1   3 , Sam S Yoon  1   3
Affiliations

Short and long-term outcomes of prophylactic total gastrectomy in 54 consecutive individuals with germline pathogenic mutations in the CDH1 gene

Mason D Stillman et al. J Surg Oncol. 2022 Dec.

Abstract

Background: Germline mutation of CDH1 is rare and leads to hereditary diffuse gastric cancer (DGC).

Methods: Patients (pts) with CDH1 mutation who underwent multidisciplinary counseling followed by open prophylactic total gastrectomy (PTG) by a single surgeon were reviewed.

Results: Fifty-four pts with a median age of 41 years (16-70 years) underwent PTG between 2006 and 2021. Median operative time was 161 min, and median hospital stay was 7 days (range 6-12). There were 5 complications (9.2%) within 30 days, and two complications (pulmonary embolism and pancreatitis) required readmission. There were no anastomotic leaks. The pathologic analysis of the first 10 pts included the entire gastric mucosa, revealing a median of 15 foci of DGC (range 5-136). The subsequent 44 pts with more limited analysis had a median of 2 foci (range 0-5), and two pts (3.7%) had no foci identified. Median maximum weight loss was 19%. In long-term follow-up (median 4.6 years) of 20 pts, median global QOL was 2.0 (very good), the majority had persistent difficulty with certain foods or liquids, and all stated they would again elect PTG over surveillance endoscopy.

Conclusions: PTG can be performed safely at high-volume referral centers with very good QOL but nutritional sequelae persist.

Keywords: germline CDH1 mutation; hereditary diffuse gastric cancer; prophylactic gastrectomy.

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Conflict of interest statement

Conflicts of interest: The authors have no conflicts of interest to disclose.

Figures

Figure 1.
Figure 1.
Pedigree of First Patient’s Family. Circles represent female family members, and squares male family members. The diamond represents the sex is unknown. Slashes indicate persons who have died, and the numbers beneath the circles or squares are age at death or age at the time the pedigree was created. The first patient is shown by the arrow. Modified from Chung DC et al. .
Figure 2.
Figure 2.
A. Graph showing patient age and number of foci of cancer. B. Graph showing median weight of patients at various time points. C. Graph showing QOL measure. Bars represent standard deviation.
See this image and copyright information in PMC

References

    1. Sung H, Ferlay J, Siegel RL, et al. Global Cancer Statistics 2020: GLOBOCAN Estimates of Incidence and Mortality Worldwide for 36 Cancers in 185 Countries. CA Cancer J Clin 2021; 71(3): 209–49. - PubMed
    1. Armstrong DK, Bundy B, Wenzel L, et al. Intraperitoneal cisplatin and paclitaxel in ovarian cancer. N Engl J Med 2006; 354(1): 34–43. - PubMed
    1. Hooi JKY, Lai WY, Ng WK, et al. Global Prevalence of Helicobacter pylori Infection: Systematic Review and Meta-Analysis. Gastroenterology 2017; 153(2): 420–9. - PubMed
    1. Lauren P The Two Histological Main Types of Gastric Carcinoma: Diffuse and So-Called Intestinal-Type Carcinoma. An Attempt at a Histo-Clinical Classification. Acta Pathol Microbiol Scand 1965; 64: 31–49. - PubMed
    1. Zanghieri G, Di Gregorio C, Sacchetti C, et al. Familial occurrence of gastric cancer in the 2-year experience of a population-based registry. Cancer 1990; 66(9): 2047–51. - PubMed