Raimundo Fernandes, SBaldaia, CPinto Marques, HTortosa, FRamalho, F2017-07-202017-07-202017-05Rev Esp Enferm Dig. 2017 May;109(5):382-385http://hdl.handle.net/10400.17/2730Clear-cell cholangiocarcinoma is a very uncommon variant of cholangiocarcinoma with a largely unknown natural history and prognosis. We report a case of a 51-year-old previously healthy woman presenting with a large liver nodule found on routine imaging. Needle biopsy of the lesion suggested a non-hepatocellular carcinoma. After extensive workup for other primary neoplasms, the patient underwent a partial hepatectomy. Histopathology was compatible with a moderately differentiated clear-cell cholangiocarcinoma. There was no evidence of liver disease in the remaining tissue. The patient underwent chemotherapy and remains in clinical remission after two years.engCHLC CHBPTAdenocarcinoma, Clear Cell/diagnosisAdenocarcinoma, Clear Cell/pathologyBile Duct Neoplasms/diagnosisBile Duct Neoplasms/pathologyBile Ducts, Intrahepatic/pathologyCholangiocarcinoma/diagnosisCholangiocarcinoma/pathologyIntrahepatic Clear Cell Cholangiocarcinoma - An Uncommon Histologic Subtype: Case Report and Literature Reviewjournal article10.17235/reed.2017.4239/2016