Abstract
An understanding of the clinical features of gastric cancer (GC) in hereditary diffuse gastric cancer (HDGC) families may be helpful in deciding who should receive prophylactic total gastrectomy and when. This study evaluates the sex ratio and age of onset features of these patients. Forty-seven HDGC families were screened from the English (40 families), Chinese (7 families) literature. One family was ascertained in our hospital. A total of 48 HDGC families with 192 GC cases were analyzed. The patients were grouped as Asian and non-Asian cases according the first author corresponding address. The male to female ratio of GC patients in our study was less than general GC population (0.769 vs. 1.826, P < 0.0001). The mean age of male cases was higher than that for females (48.9 vs. 43.1 years; P = 0.012). The mean age for parents of cases was higher than that for the offspring of cases (52.5 vs. 37.4 years; P = 0.0001). There was a significant age correlation between sib-sib GC pairs (P < 0.0001). The male to female ratio of Asian GC patients was higher than that of non-Asian GC cases (1.450 vs. 0.614, P = 0.01). The age for Asian GC patients was higher than that of non-Asian GC patients (52.1 vs. 43.5 years, P = 0.001). The results showed that female GC patients were more frequent and younger than male patients in HDGC families. The parents of cases were older than offspring cases. There was a significant age correlation between sib-sib cases. There was regional disparity in the sex ratio and age features.
Similar content being viewed by others
References
Ekstrom AM, Serafini M, Nyren O, Hansson LE, Ye W, Wolk A (2000) Dietary antioxidant intake and the risk of cardia cancer and noncardia cancer of the intestinal and diffuse types: a population-based case-control study in Sweden. Int J Cancer 87:133–140
Park JG, Yang HK, Kim WH, Caldas C, Yokota J, Guilford PJ (2000) Report on the first meeting of the international collaborative group on hereditary gastric cancer. J Natl Cancer Inst 92:1781–1782
Ghaffari SR, Rafati M, Sabokbar T, Dastan J (2010) A novel truncating mutation in the E-cadherin gene in the first Iranian family with hereditary diffuse gastric cancer. Eur J Surg Oncol 36:559–562
Guilford P, Hopkins J, Harraway J et al (1998) E-cadherin germline mutations in familial gastric cancer. Nature 392:402–405
Brooks-Wilson AR, Kaurah P, Suriano G et al (2004) Germline E-cadherin mutations in hereditary diffuse gastric cancer: assessment of 42 new families and review of genetic screening criteria. J Med Genet 41:508–517
Kaurah P, MacMillan A, Boyd N et al (2007) Founder and recurrent CDH1 mutations in families with hereditary diffuse gastric cancer. Jama 297:2360–2372
Pharoah PD, Guilford P, Caldas C (2001) Incidence of gastric cancer and breast cancer in CDH1 (E-cadherin) mutation carriers from hereditary diffuse gastric cancer families. Gastroenterology 121(6):1348–1353
Norton JA, Ham CM, Van Dam J et al (2007) CDH1 truncating mutations in the E-cadherin gene: an indication for total gastrectomy to treat hereditary diffuse gastric cancer. Ann Surg 245:873–879
Newman EA, Mulholland MW (2006) Prophylactic gastrectomy for hereditary diffuse gastric cancer syndrome. J Am Coll Surg 202:612–617
Hebbard PC, Macmillan A, Huntsman D et al (2009) Prophylactic total gastrectomy (PTG) for hereditary diffuse gastric cancer (HDGC): the Newfoundland experience with 23 patients. Ann Surg Oncol 16:1890–1895
Pandalai PK, Lauwers GY, Chung DC, Patel D, Yoon SS (2011) Prophylactic total gastrectomy for individuals with germline CDH1 mutation. Surgery 149:347–355
Lynch HT, Silva E, Wirtzfeld D, Hebbard P, Lynch J, Huntsman DG (2008) Hereditary diffuse gastric cancer: prophylactic surgical oncology implications. Surg Clin North Am 88(4):759–778, vi–vii
Yoon KA, Ku JL, Yang HK, Kim WH, Park SY, Park JG (1999) Germline mutations of E-cadherin gene in Korean familial gastric cancer patients. J Hum Genet 44:177–180
Guilford PJ, Hopkins JB, Grady WM et al (1999) E-cadherin germline mutations define an inherited cancer syndrome dominated by diffuse gastric cancer. Hum Mutat 14:249–255
Keller G, Vogelsang H, Becker I et al (1999) Diffuse type gastric and lobular breast carcinoma in a familial gastric cancer patient with an E-cadherin germline mutation. Am J Pathol 155:337–342
Avizienyte E, Launonen V, Salovaara R, Kiviluoto T, Aaltonen LA (2001) E-cadherin is not frequently mutated in hereditary gastric cancer. J Med Genet 38:49–52
Huntsman DG, Carneiro F, Lewis FR et al (2001) Early gastric cancer in young, asymptomatic carriers of germ-line E-cadherin mutations. N Engl J Med 344:1904–1909
Humar B, Toro T, Graziano F et al (2002) Novel germline CDH1 mutations in hereditary diffuse gastric cancer families. Hum Mutat 19:518–525
Yabuta T, Shinmura K, Tani M et al (2002) E-cadherin gene variants in gastric cancer families whose probands are diagnosed with diffuse gastric cancer. Int J Cancer 101:434–441
Oliveira C, Bordin MC, Grehan N et al (2002) Screening E-cadherin in gastric cancer families reveals germline mutations only in hereditary diffuse gastric cancer kindred. Hum Mutat 19:510–517
Concolino P, Papa V, Mozzetti S et al (2004) The unsolved enigma of CDH1 down-regulation in hereditary diffuse gastric cancer. J Surg Res 121:50–55
Oliveira C, de Bruin J, Nabais S et al (2004) Intragenic deletion of CDH1 as the inactivating mechanism of the wild-type allele in an HDGC tumour. Oncogene 23:2236–2240
Oliveira C, Moreira H, Seruca R, de Oliveira MC, Carneiro F (2005) Role of pathology in the identification of hereditary diffuse gastric cancer: report of a Portuguese family. Virchows Arch 446:181–184
Roviello F, Corso G, Pedrazzani C et al (2007) Hereditary diffuse gastric cancer and E-cadherin: description of the first germline mutation in an Italian family. Eur J Surg Oncol 33:448–551
More H, Humar B, Weber W et al (2007) Identification of seven novel germline mutations in the human E-cadherin (CDH1) gene. Hum Mutat 28:203
Lynch HT, Kaurah P, Wirtzfeld D et al (2008) Hereditary diffuse gastric cancer: diagnosis, genetic counseling, and prophylactic total gastrectomy. Cancer 112:2655–2663
Mayrbaeurl B, Keller G, Schauer W et al (2010) Germline mutation of the E-cadherin gene in three sibling cases with advanced gastric cancer: clinical consequences for the other family members. Eur J Gastroenterol Hepatol 22:306–310
Qiu QW (1999) Cases report-five siblings with gastric cancer. New Chin Med 30:709
He L, Chen YH (1999) Familial gastric cancer-a report of four brothers with gastric cancer. Chin J Clin Oncol Rehabil 6:59
Zhou XJ, Yang YQ (2003) A report of four gasstric cancer patients in a family. West Chin Med J 18:83
Niu HX, Xu ZF, Shi HY, Wang LL, Zhang M (2009) Analysis of clinical and pathological features of familial gastric cancer. Chin J Curr Ad Gen Surg 12:968–970
Xu SJ, Wang YJ, Qian ZX (2008) Familial gastric cancer-a report of three cases. J Practi Oncol 23:65–66
Yu JX (2008) Familiar gastric cancer: case report and systematic review of the Chinese literature. Zhonghua Yi Xue Za Zhi 88:2789–2791
Parkin DM, Bray F, Ferlay J, Pisani P (2005) Global cancer statistics, 2002. CA Cancer J Clin 55:74–108
Guilford P, Humar B, Blair V (2010) Hereditary diffuse gastric cancer: translation of CDH1 germline mutations into clinical practice. Gastric Cancer 13:1–10
Kim IJ, Park JH, Kang HC et al (2003) A novel germline mutation in the MET extracellular domain in a Korean patient with the diffuse type of familial gastric cancer. J Med Genet 40:e97
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Yu, J., Li, Z. The sex ratio and age of onset features of gastric cancer patients in hereditary diffuse gastric cancer families. Familial Cancer 10, 573–579 (2011). https://doi.org/10.1007/s10689-011-9452-z
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10689-011-9452-z