Association of a duodenal follicular lymphoma and hereditary nonpolyposis colorectal cancer

被引:14
作者
Rosty, C
Brière, J
Cellier, C
Delabesse, E
Carnot, F
Barbier, JP
Laurent-Puig, P
机构
[1] Hop Laennec, Anat Pathol Lab, Serv Anat Patol, F-75340 Paris 07, France
[2] Hop Necker Enfants Malad, Hematol Lab, Paris, France
[3] INSERM, Unite U490, Paris, France
关键词
follicular lymphoma; hereditary nonpolyposis colorectal cancer; immunohistochemistry; microsatellite instability;
D O I
10.1038/modpathol.3880100
中图分类号
R36 [病理学];
学科分类号
100104 ;
摘要
Hereditary nonpolyposis colorectal cancer (HNPCC) is an inherited predisposition to colorectal and endometriaI 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 far HNPCC, lymphomas are almost never encountered in patients who have HNPCC, except in rare families with germline homozygous deletion of ;HI. We report the case of a 53-year-old man who had a history of colon cancers related to constitutional mMLH1 mutation and who was diagnosed as having a duodenal follicular lymphoma. This diagnosis was supported by IgH-BCL2 rearrangement and BCL2 immunoreactivity in turner 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.
引用
收藏
页码:586 / 590
页数:5
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