Small bowel carcinoma revealing HNPCC syndrome.
Small bowel adenocarcinoma is a rare condition with poor prognosis. Like colorectal cancer, small bowel carcinoma may be a part of genetic syndromes with carcinogenetic pathways different from sporadic forms. We report a case of 41-year-old man with small bowel carcinoma revealing hereditary non polyposis colorectal cancer (HNPCC) syndrome. This report supports the systematic study of the MSI status in every patient with a small bowel carcinoma below 60-year-old of age in order to screen for HNPCC syndrome even in the absence of a family history of related cancers.
Babba
T
T
Service de Gastroentérologie, Hôpital Avicenne, 125, rue de Stalingrad, 93009 Bobigny cedex, France. thouraya.babba@avc.aphp.fr
Schischmanoff
O
O
Lagorce
C
C
Wind
P
P
Des Guetz
G
G
Aparicio
T
T
Benamouzig
R
R
D048868:Adaptor Proteins, Signal Transducing / Q000235:genetics; D000230:Adenocarcinoma / Q000473:pathology* / Q000628:therapy; D000328:Adult; D003123:Colorectal Neoplasms, Hereditary Nonpolyposis / Q000175:diagnosis*; D004379:Duodenal Neoplasms / Q000473:pathology* / Q000628:therapy; D005091:Exons; D006801:Humans; D008297:Male; D000070957:MutL Protein Homolog 1; D009154:Mutation; D009687:Nuclear Proteins / Q000235:genetics
D048868:Adaptor Proteins, Signal Transducing; C501486:MLH1 protein, human; D009687:Nuclear Proteins; D000070957:MutL Protein Homolog 1