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

Short and long-term outcomes of prophylactic total gastrectomy in 54 consecutive individuals with germline pathogenic mutations in the CDH1 gene.
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DOI:
10.1002/jso.27084
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发表时间:
2022-12
影响因子:
2.5
通讯作者:
Yoon, Sam S.
Yoon, Sam S.
中科院分区:
医学3区
文献类型:
--
作者:
Stillman, Mason D.;Kusche, Nicole;Toledano, Sabrina;Hilfrank, Kimberly J.;Yoon, Changhwan;Gabre, Joel T.;Rustgi, Sheila D.;Hur, Chin;Kastrinos, Fay;Ryeom, Sandra W.;Yoon, Sam S.

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Germline mutation of CDH1 is rare and leads to hereditary diffuse gastric cancer (DGC). Patients (pts) with CDH1 mutation who underwent multidisciplinary counseling followed by open prophylactic total gastrectomy (PTG) by a single surgeon were reviewed. Fifty-four pts with a median age of 41 years (16–70 years) underwent PTG between 2006–2021. Median operative time was 161 minutes, 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. PTG can be performed safely at high-volume referral centers with very good QOL but nutritional sequelae persist.
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