Primary pure squamous cell carcinomas (SCC) of the duodenum are very uncommon. To the best of our knowledge, only a few cases of SCC of the duodenum exist in the reported literature. Here, we report a case of SCC of the duodenum in northern China. A 45-year-old Chinese Han male patient presented with abdominal pain and weight loss. CT, endoscopic examinations, X-rays, and immunohistochemical markers were used to confirm this rare diagnosis of SCC. We performed a pancreaticoduodenectomy with a curative intention. However, histological examination revealed SCC of the duodenum. Postoperative chemotherapy was started after surgery. To the best of our knowledge, pancreaticoduodenectomy is the preferred form of treatment for carcinoma of the duodenum. This is supplemented with chemotherapy, which can further prolong survival.

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