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Follicular lymphoma ( FL ) shows co‐expression of B‐cell lymphoma 2 ( BCL 2) and  CD 10, whereas downexpression of  CD 10 is occasionally experienced in gastrointestinal ( GI )  FL  with unknown significance. Gastrointestinal  FL  is a rare variant of  FL , and its similarity with mucosa‐associated lymphoid tissue lymphoma was reported. We investigated the clinicopathological and genetic features of  CD 10 downexpressed ( CD 10 down )  GI ‐ FL . The diagnosis of  CD 10 down   FL  was carried out with a combination of pathological and molecular analyses. The incidence of  CD 10 down   GI ‐ FL  was shown in 35/172 (20.3%) cases, which was more frequent than nodal  FL  (3.5%,  P  < 0.001). The difference was additionally significant between  GI ‐ FL  and nodal  FL  when the analysis was confined to primary  GI ‐ FL  (55.2%  vs  3.5%,  P <  0.001). Compared to  CD 10 +   GI ‐ FL ,  CD 10 down   GI ‐ FL  significantly involved the stomach or large intestine ( P  = 0.015), and additionally showed the downexpression of  BCL 6 ( P  < 0.001). The follicular dendritic cell meshwork often showed a duodenal pattern in the  CD 10 down  group ( P  = 0.12). Furthermore, a lymphoepithelial lesion was observed in 5/12 (40%) gastric  FL  cases, which indicated caution in the differentiation of mucosa‐associated lymphoid tissue lymphoma. Molecular analyses were undertaken in seven cases of  CD 10 down   GI ‐ FL , and an identical clone was found between  CD 10 down  follicles and  CD 10 +  BCL 2 +  neoplastic follicles. In the diagnosis of cases with  CD 10 down   BCL 2 +  follicles, careful examination with molecular studies should be carried out.

CD10 down expression in follicular lymphoma correlates with gastrointestinal lesion involving the stomach and large intestine