Loosen Sven H, Gaisa Nadine T, Schmeding Maximilian, Heining Christoph, Uhrig Sebastian, Wirtz Theresa H, Kalverkamp Sebastian, Spillner Jan, Tacke Frank, Stenzinger Albrecht, Glimm Hanno, Fröhling Stefan, Trautwein Christian, Roderburg Christoph, Longerich Thomas, Neumann Ulf Peter, Luedde Tom
Clinic for Gastroenterology, Hepatology and Infectious Diseases, University Hospital Düsseldorf, Medical Faculty of Heinrich Heine University Düsseldorf, Düsseldorf, Germany.
Department of Medicine III, University Hospital RWTH Aachen, Aachen, Germany.
Case Rep Gastroenterol. 2020 Dec 10;14(3):658-667. doi: 10.1159/000511034. eCollection 2020 Sep-Dec.
Combined hepatocellular-cholangiocarcinoma (cHCC/CCA) represents a rare type of primary liver cancer with a very limited prognosis. Although just recently genomic studies have contributed to a better understanding of the disease's genetic landscape, therapeutic options, especially for advanced-stage patients, are limited and often experimental, as no standardized treatment protocols have been established to date. Here, we report the case of a 38-year-old male patient who was diagnosed with extensive intrahepatic cHCC/CCA in an otherwise healthy liver without signs of chronic liver disease. An interdisciplinary stepwise therapeutic approach including locoregional liver-targeted therapy, systemic chemotherapy, liver transplantation, surgical pulmonary metastasis resection, and next-generation sequencing-based targeted therapy led to a prolonged overall survival beyond 5 years with an excellent quality of life. This case report comprises several provocative treatment decisions that are extensively discussed in light of the existing literature on this rare but highly aggressive malignancy.
肝内胆管癌合并肝细胞癌（cHCC/CCA）是一种罕见的原发性肝癌，预后非常有限。尽管最近的基因组研究有助于更好地了解该疾病的遗传特征，但治疗选择，尤其是对于晚期患者，仍然有限且往往是试验性的，因为迄今为止尚未建立标准化的治疗方案。在此，我们报告一例38岁男性患者的病例，该患者在无慢性肝病迹象的健康肝脏中被诊断为广泛肝内cHCC/CCA。一种跨学科的逐步治疗方法，包括局部肝脏靶向治疗、全身化疗、肝移植、手术切除肺转移灶以及基于二代测序的靶向治疗，使患者总生存期延长超过5年，且生活质量良好。本病例报告包含了几个具有启发性的治疗决策，并根据关于这种罕见但高度侵袭性恶性肿瘤的现有文献进行了广泛讨论。
