Association of a duodenal follicular lymphoma and hereditary nonpolyposis colorectal cancer

Rosty, Christophe, Briere, Josette, Cellier, Christophe, Delabesse, Eric, Carnot, Françoise, Barbier, Jean-Philippe and Laurent-Puig, Pierre (2000) Association of a duodenal follicular lymphoma and hereditary nonpolyposis colorectal cancer. Modern Pathology, 13 5: 586-590. doi:10.1038/modpathol.3880100

Author Rosty, Christophe
Briere, Josette
Cellier, Christophe
Delabesse, Eric
Carnot, Françoise
Barbier, Jean-Philippe
Laurent-Puig, Pierre
Title Association of a duodenal follicular lymphoma and hereditary nonpolyposis colorectal cancer
Journal name Modern Pathology   Check publisher's open access policy
ISSN 0893-3952
Publication date 2000-05
Sub-type Article (original research)
DOI 10.1038/modpathol.3880100
Volume 13
Issue 5
Start page 586
End page 590
Total pages 5
Place of publication London, United Kingdom
Publisher Nature Publishing
Language eng
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 IgHBCL2 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.
Keyword Follicular lymphoma
Hereditary nonpolyposis colorectal cancer
Microsatellite instability
Q-Index Code C1
Q-Index Status Provisional Code
Institutional Status Non-UQ

Document type: Journal Article
Sub-type: Article (original research)
Collection: School of Medicine Publications
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Created: Wed, 09 Mar 2011, 14:22:20 EST