Backgrounds: Clear cell adenocarcinoma (CCA) of duodenum has not been reported. Aims: To report the first case of CCA of duodenum. Case: A 76-year-old woman presented epigastralgia, and upper GI endoscopy revealed an ulcerated tumor in the 1 st portion (next to stomach) of duodenum close to pylorus (remote from pylorus by 2 cm). No tumor was seen in duodenal papilla. Biopsy from the lesion showed proliferation and invasion of high-grade carcinoma cells with very clear cytoplasma but without glandular structures. No signet-ring carcinoma cells were seen. Histochemically, mucus stains showed no mucins but suggested glycogens. Immunohistochemically, tumor cells were positive for cytokeratin (CK)7, CK18, CK19, CEA, CA19-9, p53, MUC1, and Ki67 (labeling index = 36%), but negative for CD45, CK34BE12, CK5, CK6, CK20, vimentin, CA125, CDX-2, HepPar1, AFP, PSA, CD10, renal cell carcinoma (RCC) markers, PSA, AMACR, HER2, S100 protein, TTF-1, NSE, NCAM, synaptophysin, chromogranin, MUC2, MUC5AC, MUC6, estrogen receptor and progesterone receptor. Conclusion: The first case of primary CCA of duodenum is reported.
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