The association between cholangiooaroinoma and hereditary nonpolyposis colorectal carcinoma

@article{Mecklin1992TheAB,
  title={The association between cholangiooaroinoma and hereditary nonpolyposis colorectal carcinoma},
  author={J. Mecklin and H. J{\"a}rvinen and M. Virolainen},
  journal={Cancer},
  year={1992},
  volume={69}
}
Eighteen patients with a biliopancreatic carcinoma in 15 different cancer family syndrome (CFS) families were studied. Only families with three or more first‐degree relatives with colorectal carcinoma were included, and other characteristics of CFS were required in at least two cases. In 14 patients, the histologic specimen was available for reevaluation. In 11 (79%), the tumor was confirmed as a carcinoma of the biliary tract or papilla of Vater. In three (21%), carcinoma of the pancreas was… Expand
Treatment and follow-up strategies in hereditary nonpolyposis colorectal carcinoma
TLDR
It was concluded that subtotal colectomy is superior to hemicolectomy or segmental resection in HNPCC patients with colorectal carcinoma and a regular annual endoscopic follow-up of the residual rectum is still necessary, and surveillance for extracolonic cancers must be considered. Expand
Hereditary gastrointestinal polyposis and nonpolyposis syndromes.
  • A. Rustgi
  • Medicine
  • The New England journal of medicine
  • 1994
TLDR
A large number of studies have implicated environmental factors in the pathogenesis of colon cancer, and support for these findings comes from the wide variations in the incidence of Colon cancer among different populations. Expand
Clinical Aspects and Management of Hereditary Non-Polyposis Colorectal Cancer (HNPCC)
TLDR
In this review, the spectrum of the disease is described and analysed with particular attention to the frequency and characteristics of extracolonic cancers in Hereditary non-polyposis colorectal cancer. Expand
Management of extracolonic tumours in patients with Lynch syndrome.
TLDR
No data show efficacy of chemopreventive drugs in reducing the risk of extracolonic cancers for patients with Lynch syndrome, and surveillance is generally recommended for urinary tract and gastric cancer, especially in families with more than one member with these types of cancer. Expand
A case of Muir-Torre syndrome associated with mucinous hepatic cholangiocarcinoma and a novel germline mutation of the MSH2 gene
TLDR
A case of Muir-Torre syndrome associated with intrahepatic cholangiocarcinoma, a location not previously described, and associated with a novel missense mutation of the MSH2 gene (c.2026T > C), predicted to disrupt the function of the gene. Expand
Genetics, natural history, tumor spectrum, and pathology of hereditary nonpolyposis colorectal cancer: an updated review.
TLDR
Current knowledge is summarized, with emphasis on the manner in which this knowledge can be employed effectively for diagnosis and management of HNPCC. Expand
Synchronous Carcinoma of the Ampulla of Vater and Colon Cancer
TLDR
The association of sporadic Ampullary and colonic adenocarcinomas and the mutually increased risk of developing either a synchronous or a metachronous tumor following each other should be considered in patients with primary ampullary or colorectal cancer during the preoperative evaluation and postoperative follow-up of these patients. Expand
Hereditary Pancreatic and Hepatobiliary Cancers
TLDR
Hereditary etiologies of pancreatic and hepatobiliary cancers are increasingly recognized, and diseases whose association with hereditary carcinogenesis is under investigation include hereditary hemochromatosis, autoimmune hepatitis, porphyria, and α1-antitrypsin deficiency. Expand
Hilar Cholangiocarcinoma (Klatskin tumor)
TLDR
The clinical, imaging studies and the lab tests suggest a Klatskin tumor, which was managed by percutaneous biliary drainage to relieve the jaundice of a 71-year-old man with an obstructive lesion of the hepatic hilum. Expand
Early detection of metachronous bile duct cancer in Lynch syndrome: report of a case
TLDR
The bile duct cancer was diagnosed at an early stage in the present patient due to the observation of the gene mutation and the preceding liver tumor, illustrating the importance of continuous surveillance for extracolonic tumors, including bileduct cancer, in patients with Lynch syndrome. Expand
...
1
2
3
4
5
...

References

SHOWING 1-10 OF 11 REFERENCES
Tumor spectrum in cancer family syndrome (hereditary nonpolyposis colorectal cancer)
TLDR
It is concluded that the occurrence of one or more types of extracolonic tumors in members of CFS families does not provide a firm basis for subdividing the CFS (or hereditary nonpolyposis colorectal carcinoma syndrome). Expand
Gardner's syndrome associated with periampullary carcinoma, duodenal and gastric adenomatosis
A 48-year-old man with Gardner's syndrome, who had abdominoperineal resection for rectal carcinoma in 1962, was found to have an ulcerating growth of the duodenum, and pancreaticoduodenectomy wasExpand
Hereditary nonpolyposis colorectal cancer--Lynch syndromes I and II.
TLDR
Increased knowledge about colorectal cancer etiology and carcinogenesis can be attained through the study of families prone to the Lynch syndromes, and biomarkers of acceptable sensitivity and specificity for the genotype are needed. Expand
Frequency of hereditary colorectal carcinoma.
TLDR
The cancer family syndrome type of hereditary nonpolyposis colorectal carcinoma emerged as the most common verifiable risk factor for colon cancer involving 3.8%-5.5% of all colorective carcinoma patients in this study. Expand
Biliary involvement in familial adenomatosis coli
TLDR
Two patients with polypoid bile duct tumors associated with familial adenomatosis coli are presented and support the view that familial gastrointestinal adenOMatosis is a systemic disease which is not restricted to the colon and rectum. Expand
Differential diagnosis of hereditary nonpolyposis colorectal cancer (Lynch syndrome I and Lynch syndrome II)
TLDR
The authors report detailed cancer (all sites) family histories on two prototype families with Lynch syndrome I (Family R) and Lynch syndrome II (Family N), which have been under investigation for more than two decades. Expand
Tumor variation in three extended Lynch syndrome II kindreds.
TLDR
Full scrutiny of cancer of all anatomic sites in the absence of biomarkers of high sensitivity and specificity to the cancer-prone genotype will be necessary to comprehend more clearly whether these (or other) cancer sites are integral to this disorder, whether common environmental exposures are involved and, finally, whether chance can explain these cancer associations. Expand
Gastroduodenal polyps in familial adenomatous and juvenile polyposis.
TLDR
It is concluded that gastroduodenal polyps belong inherently both to adenomatous and to juvenile varieties of gastrointestinal polyposis, and that these two conditions have characteristic histological patterns and topography of polyps in the upper gastrointestinal tract. Expand
UPPER GASTROINTESTINAL CANCER IN FAMILIAL ADENOMATOUS POLYPOSIS
TLDR
Findings confirm an increased risk of upper gastrointestinal cancer in the polyposis patient, particularly distal to the pylorus, and support an adenoma-carcinoma sequence. Expand
Pancreatic carcinoma and hereditary nonpolyposis colorectal cancer: a family study.
On decrit une famille avec une transmission verticale du cancer a travers 5 generations qui presentaient les caracteristiques du cancer colorectal hereditaire non polyposique associe a un cancer duExpand
...
1
2
...