Association of a Duodenal Follicular Lymphoma and Hereditary Nonpolyposis Colorectal Cancer View Full Text


Ontology type: schema:ScholarlyArticle      Open Access: True


Article Info

DATE

2000-05-01

AUTHORS

Christophe Rosty, Josette Brière, Christophe Cellier, Eric Delabesse, Françoise Carnot, Jean-Philippe Barbier, Pierre Laurent-Puig

ABSTRACT

Hereditary nonpolyposis colorectal cancer (HNPCC) is an inherited predisposition to colorectal and endometrial cancers caused by germline mutation of mismatch repair genes, with hMLH1 and hMSH2 underlying the majority of the cases. Although lymphoid tumors are the most common tumors in mouse models for HNPCC, lymphomas are almost never encountered in patients who have HNPCC, except in rare families with germline homozygous deletion of hMLH1. We report the case of a 53-year-old man who had a history of colon cancers related to constitutional hMLH1 mutation and who was diagnosed as having a duodenal follicular lymphoma. This diagnosis was supported by IgH-BCL2 rearrangement and BCL2 immunoreactivity in tumor cells. The association of both of these possibly related rare diseases has never been reported. To clarify this relationship, we searched for hMLH1 expression and mismatch repair deficiency in the duodenal lymphoma. hMLH1 immunostaining was positive in lymphoid tumor cells, and no microsatellite instability was detected. In agreement with mouse models for HNPCC, these results suggest the involvement of alternative mechanisms to complete mismatch repair deficiency for lymphomagenesis in HNPCC syndrome. More... »

PAGES

586-590

Identifiers

URI

http://scigraph.springernature.com/pub.10.1038/modpathol.3880100

DOI

http://dx.doi.org/10.1038/modpathol.3880100

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1041049349

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/10824932


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