The association between cholangiooaroinoma and hereditary nonpolyposis colorectal carcinoma

  title={The association between cholangiooaroinoma and hereditary nonpolyposis colorectal carcinoma},
  author={Jukka-Pekka Mecklin and Heikki J{\"a}rvinen and Martti Virolainen},
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… 

Treatment and follow-up strategies in hereditary nonpolyposis colorectal carcinoma

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.

Hereditary gastrointestinal polyposis and nonpolyposis syndromes.

  • A. Rustgi
  • Medicine
    The New England journal of medicine
  • 1994
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.

Clinical Aspects and Management of Hereditary Non-Polyposis Colorectal Cancer (HNPCC)

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.

A case of Muir-Torre syndrome associated with mucinous hepatic cholangiocarcinoma and a novel germline mutation of the MSH2 gene

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.

Synchronous Carcinoma of the Ampulla of Vater and Colon Cancer

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.

Hereditary Pancreatic and Hepatobiliary Cancers

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.

Early detection of metachronous bile duct cancer in Lynch syndrome: report of a case

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.

Symposium 17: Pancreas‐biliary tract neoplams

This review will compare the pathologic features of primary tumours of the bile ducts, ampulla, and pancreas, focusing on three major categories of lesions: pre-invasive neoplasia, invasive adenocarcinomas and endocrine tumours.

Evolution of hereditary bowel cancer.

  • J. Jass
  • Medicine
    Mutation research
  • 1993



Tumor spectrum in cancer family syndrome (hereditary nonpolyposis colorectal cancer)

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).

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 was

Hereditary nonpolyposis colorectal cancer--Lynch syndromes I and II.

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.

Biliary involvement in familial adenomatosis coli

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.

Differential diagnosis of hereditary nonpolyposis colorectal cancer (Lynch syndrome I and Lynch syndrome II)

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.

Tumor variation in three extended Lynch syndrome II kindreds.

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.

Gastroduodenal polyps in familial adenomatous and juvenile polyposis.

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.

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 du