Prophylactic Total Gastrectomy (PTG) for Hereditary Diffuse Gastric Cancer (HDGC): The Newfoundland Experience with 23 Patients

Hebbard, Pamela; MacMillan, Andrée; Huntsman, David G.; Kaurah, Pardeep; Carneiro, Fátima; Wen, Xin; Kwan, Ambrose; Boone, Darrell; Bursey, Ford; Green, J; Fernandez, Bridget A.; Fontaine, Dorrie K.; Wirtzfeld, Debrah

doi:10.1245/s10434-009-0471-z

Cited by 95 publications

(62 citation statements)

References 27 publications

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“…Twenty-eight articles [12,13,17,19,23,24,26,34,37,44,51,52,[59][60][61][62][63][64][65][66][67][68][69][70][71][72][73][74] reported prophylactic gastrectomy results in HDGC patients (Fig. 2, for full details see Appendix C).…”

Section: Prophylactic Gastrectomy Resultsmentioning

confidence: 99%

“…The largest single-center experience with prophylactic gastrectomy for CDH1 mutation carriers comes from Newfoundland, Canada [12,17]. Hebbard et al [12] reported outcomes from 23 patients who underwent prophylactic total gastrectomy.…”

Section: Discussionmentioning

confidence: 99%

“…Hebbard et al [12] reported outcomes from 23 patients who underwent prophylactic total gastrectomy. They reported no mortality, a 17% major complication rate, 48% overall complication rate, and a median length of hospital stay of 11 days (range: 7-107 days).…”

Section: Discussionmentioning

confidence: 99%

“…Regardless of mutation type, the majority (75%) result in a truncated loss-of-function protein [11]. CDH1 mutations are found among all ethnicities, with large clusters being identified in New Zealand and Canada [11][12][13]. A paradox is noted for Asian countries, where a high incidence of sporadic GC is present, but a low incidence of CDH1 mutations has been found.…”

Section: Introductionmentioning

confidence: 99%

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A systematic review of the indications for genetic testing and prophylactic gastrectomy among patients with hereditary diffuse gastric cancer

et al. 2011

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Background Hereditary diffuse gastric cancer (HDGC) is a familial cancer syndrome specifically associated with germline mutations to the E-cadherin (CDH1) gene. HDGC is characterized by autosomal dominance and high penetrance and a high cumulative risk for advanced gastric cancer. Our purpose in this study was to identify and synthesize findings from all articles on: (1) current recommendations for CDH1 screening and prophylactic gastrectomy; (2) CDH1 testing results in HDGC patients; and (3) prophylactic gastrectomy results in HDGC patients. Methods Systematic electronic literature searches were conducted using Medline, Embase, and the Cochrane Central Register of Controlled Trials from 1985 to 2009. Results Seventy articles were included in this review. Among patients with a positive family history of gastric cancer, 1085 were screened from 454 families, and 38.4% tested positive. Mutation-positive families also had a considerable family history of breast and colon cancer. Of the 322 patients screened for CDH1 mutations by current HDGC screening criteria, 29.2% tested positive. Among the 76.8% of patients who underwent prophylactic gastrectomy following positive CDH1 test results, 87.0% had positive final histopathology results and 64.6% had signet ring cells identified. Some of the patients with negative final histopathology results had opted to undergo prophylactic gastrectomy prior to CDH1 testing, and were ultimately found to be negative for CDH1 mutations. Conclusion CDH1 mutation testing in families with a history of gastric cancer and prophylactic gastrectomy in mutation-positive patients are recommended for the management of HDGC.

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Section: Prophylactic Gastrectomy Resultsmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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A systematic review of the indications for genetic testing and prophylactic gastrectomy among patients with hereditary diffuse gastric cancer

et al. 2011

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“…Currently, no screening tests are available for early diagnosis in this patient population. Direct visualization with endoscopy tends to detect the cancer late in the disease process, and multiple random endoscopic biopsies often produce false-negative results 9,10 . Therefore, for asymptomatic carriers of CDH1 mutations, prophylactic gastrectomy is recommended 7 .…”

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confidence: 99%

Report from the 17th Annual Western Canadian Gastrointestinal Cancer Consensus Conference; Edmonton, Alberta; 11–12 September 2015

et al. 2016

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The 17th annual Western Canadian Gastrointestinal Cancer Consensus Conference (wcgccc) was held in Edmonton, Alberta, 11-12 September 2015. The wcgccc is an interactive multidisciplinary conference attended by health care professionals from across Western Canada (British Columbia, Alberta, Saskatchewan, and Manitoba) who are involved in the care of patients with gastrointestinal cancer. Surgical, medical, and radiation oncologists; pathologists; radiologists; and allied health care professionals participated in presentation and discussion sessions for the purposes of developing the recommendations presented here. This consensus statement addresses current issues in the management of gastric cancer.

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Hereditary Diffuse Gastric Cancer Syndrome

et al. 2015

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This is the largest reported series of CDH1 mutation carriers, providing more precise estimates of age-associated risks of gastric and breast cancer that will improve counseling of unaffected carriers. In HDGC families lacking CDH1 mutations, testing of CTNNA1 and other tumor suppressor genes should be considered. Clinically defined HDGC families can harbor mutations in genes (ie, BRCA2) with different clinical ramifications from CDH1. Therefore, we propose that HDGC syndrome may be best defined by mutations in CDH1 and closely related genes, rather than through clinical criteria that capture families with heterogeneous susceptibility profiles.

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Prophylactic Total Gastrectomy (PTG) for Hereditary Diffuse Gastric Cancer (HDGC): The Newfoundland Experience with 23 Patients

Cited by 95 publications

References 27 publications

A systematic review of the indications for genetic testing and prophylactic gastrectomy among patients with hereditary diffuse gastric cancer

A systematic review of the indications for genetic testing and prophylactic gastrectomy among patients with hereditary diffuse gastric cancer

Report from the 17th Annual Western Canadian Gastrointestinal Cancer Consensus Conference; Edmonton, Alberta; 11–12 September 2015

Hereditary Diffuse Gastric Cancer Syndrome

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