Primary SCC of the liver is extremely rare. According to a few sporadic reports, surgical resections seem to be selected as an initial treatment, if it resectable; however, the prognosis is universally unfavorable. Methods: We presented one case of pure SCC of the liver. A total of 41 cases of primary SCC of the liver previously reported in the English literatures were reviewed. Results: A 57-year-old man with history of right flank pain presented with 1 week of high grade fever. On physical examination, his abdomen was tender, with the most severe pain in the right upper quadrant. Contrast-enhanced computed tomography revealed that there was 14x10cm irregular mass with central necrosis at S4+5 of the liver and swollen nodes, which were compatible with his symptoms. The patient underwent S4+5 segmentectomy with extended resection of gall bladder, transverse colon, and diaphragm. Histological examination revealed pure squamous cell carcinoma of the intrahepatic biliary tract. On the POD 44 prior to discharge, relapse was found around the common bile duct and liver resection edge. Although chemotherapy was proposed, his severe fatigue refused it. He was died of multiple metastasis including bones on the 52 nd day after discharge. We made Kaplan-Meier survival curves of the patients divided by with and without surgery from the review of previous reports. One year survival rate with surgery is better than without surgery (63.4% vs 15.4%, p=0.0024). Conclusion: Radical resection should be firstly recommended; however, case collections and data analysis may be needed to establish better treatments.
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