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. 1995 May;32(5):330–335. doi: 10.1136/jmg.32.5.330

Familial pancreatic adenocarcinoma: association with diabetes and early molecular diagnosis.

J P Evans 1, W Burke 1, R Chen 1, R L Bennett 1, R A Schmidt 1, E P Dellinger 1, M Kimmey 1, D Crispin 1, T A Brentnall 1, D R Byrd 1
PMCID: PMC1050425  PMID: 7616537

Abstract

We report a large pedigree in which pancreatic cancer is inherited in an autosomal dominant fashion. Diabetes and exocrine insufficiency was observed in all family members who eventually developed pancreatic cancer. The presence of diabetes, often years before the diagnosis of cancer, allowed identification of those people who had inherited the predisposing allele and who were thus at high risk for the development of malignancy. This family shows that genetic factors can have a striking effect on the development of pancreatic malignancy and diabetes mellitus. Moreover, preclinical diagnosis of pancreatic cancer in family members provided a unique opportunity to study early molecular changes that accompany the development of human pancreatic cancer. Finally, the molecular approach applied here to the early diagnosis of pancreatic cancer may prove valuable in this family for identification of subjects at risk.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Adami H. O., McLaughlin J., Ekbom A., Berne C., Silverman D., Hacker D., Persson I. Cancer risk in patients with diabetes mellitus. Cancer Causes Control. 1991 Sep;2(5):307–314. doi: 10.1007/BF00051670. [DOI] [PubMed] [Google Scholar]
  2. Almoguera C., Shibata D., Forrester K., Martin J., Arnheim N., Perucho M. Most human carcinomas of the exocrine pancreas contain mutant c-K-ras genes. Cell. 1988 May 20;53(4):549–554. doi: 10.1016/0092-8674(88)90571-5. [DOI] [PubMed] [Google Scholar]
  3. Appel M. F. Hereditary pancreatitis. Review and presentation of an additional kindred. Arch Surg. 1974 Jan;108(1):63–65. doi: 10.1001/archsurg.1974.01350250053014. [DOI] [PubMed] [Google Scholar]
  4. Boring C. C., Squires T. S., Tong T. Cancer statistics, 1993. CA Cancer J Clin. 1993 Jan-Feb;43(1):7–26. doi: 10.3322/canjclin.43.1.7. [DOI] [PubMed] [Google Scholar]
  5. Cuzick J., Babiker A. G. Pancreatic cancer, alcohol, diabetes mellitus and gall-bladder disease. Int J Cancer. 1989 Mar 15;43(3):415–421. doi: 10.1002/ijc.2910430312. [DOI] [PubMed] [Google Scholar]
  6. Danes B. S., Lynch H. T. A familial aggregation of pancreatic cancer. An in vitro study. JAMA. 1982 May 28;247(20):2798–2802. [PubMed] [Google Scholar]
  7. Dat N. M., Sontag S. J. Pancreatic carcinoma in brothers. Ann Intern Med. 1982 Aug;97(2):282–282. doi: 10.7326/0003-4819-97-2-282_1. [DOI] [PubMed] [Google Scholar]
  8. Ehrenthal D., Haeger L., Griffin T., Compton C. Familial pancreatic adenocarcinoma in three generations. A case report and a review of the literature. Cancer. 1987 May 1;59(9):1661–1664. doi: 10.1002/1097-0142(19870501)59:9<1661::aid-cncr2820590923>3.0.co;2-h. [DOI] [PubMed] [Google Scholar]
  9. Fisher W. E., McCullough P. J., Rämö O. J., Bell R. H., Jr Further studies of enhanced growth of pancreatic carcinoma in diabetes. J Surg Res. 1990 May;48(5):403–407. doi: 10.1016/0022-4804(90)90003-k. [DOI] [PubMed] [Google Scholar]
  10. Friedman J. M., Fialkow P. J. Familial carcinoma of the pancreas. Clin Genet. 1976 May;9(5):463–469. doi: 10.1111/j.1399-0004.1976.tb01598.x. [DOI] [PubMed] [Google Scholar]
  11. Goelz S. E., Hamilton S. R., Vogelstein B. Purification of DNA from formaldehyde fixed and paraffin embedded human tissue. Biochem Biophys Res Commun. 1985 Jul 16;130(1):118–126. doi: 10.1016/0006-291x(85)90390-0. [DOI] [PubMed] [Google Scholar]
  12. Lowenfels A. B., Maisonneuve P., Cavallini G., Ammann R. W., Lankisch P. G., Andersen J. R., Dimagno E. P., Andrén-Sandberg A., Domellöf L. Pancreatitis and the risk of pancreatic cancer. International Pancreatitis Study Group. N Engl J Med. 1993 May 20;328(20):1433–1437. doi: 10.1056/NEJM199305203282001. [DOI] [PubMed] [Google Scholar]
  13. Lynch H. T., Fitzsimmons M. L., Smyrk T. C., Lanspa S. J., Watson P., McClellan J., Lynch J. F. Familial pancreatic cancer: clinicopathologic study of 18 nuclear families. Am J Gastroenterol. 1990 Jan;85(1):54–60. [PubMed] [Google Scholar]
  14. MacDermott R. P., Kramer P. Adenocarcinoma of the pancreas in four siblings. Gastroenterology. 1973 Jul;65(1):137–139. [PubMed] [Google Scholar]
  15. Ponder B. A. Inherited predisposition to cancer. Trends Genet. 1990 Jul;6(7):213–218. doi: 10.1016/0168-9525(90)90181-5. [DOI] [PubMed] [Google Scholar]
  16. Reimer R. R., Fraumeni J. F., Jr, Ozols R. F., Bender R. Pancreatic cancer in father and son. Lancet. 1977 Apr 23;1(8017):911–911. doi: 10.1016/s0140-6736(77)91244-2. [DOI] [PubMed] [Google Scholar]
  17. Tada M., Omata M., Kawai S., Saisho H., Ohto M., Saiki R. K., Sninsky J. J. Detection of ras gene mutations in pancreatic juice and peripheral blood of patients with pancreatic adenocarcinoma. Cancer Res. 1993 Jun 1;53(11):2472–2474. [PubMed] [Google Scholar]

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