Chun Yun Shin, Zimmitti Giuseppe
Department of Surgical Oncology, Fox Chase Cancer Center, Philadelphia, PA 19111, USA.
Recent Results Cancer Res. 2013;190:101-10. doi: 10.1007/978-3-642-16037-0_7.
The fibrolamellar variant of hepatocellular carcinoma is a rare primary liver cancer occurring in adolescents and young adults without chronic liver disease or known risk factors. Histologically, it is defined by lamellar bands of fibrosis surrounding well-differentiated tumor cells. Radiologic imaging typically demonstrates a large, solitary mass with calcifications and a central scar. Lymph node metastases in the porta hepatis are frequently diagnosed upon presentation. More patients with fibrolamellar carcinoma are candidates for surgical resection than those with conventional hepatocellular carcinoma, owing to their young age and absence of cirrhosis. The most important prognostic factor is surgical resection, which results in 5-year overall survival rates ranging between 50 and 76 %. Despite complete surgical resection, relapse rates are high, and novel therapies are needed to prevent and treat recurrent disease.
纤维板层型肝细胞癌是一种罕见的原发性肝癌，发生于青少年和青年，无慢性肝病或已知危险因素。在组织学上，它由围绕分化良好的肿瘤细胞的板层状纤维化带所定义。放射影像学通常显示一个大的、孤立的肿块，伴有钙化和中央瘢痕。肝门部淋巴结转移在就诊时经常被诊断出来。由于纤维板层癌患者年龄较轻且无肝硬化，与传统肝细胞癌患者相比，更多的纤维板层癌患者适合手术切除。最重要的预后因素是手术切除，其5年总生存率在50%至76%之间。尽管进行了完整的手术切除，但复发率很高，需要新的疗法来预防和治疗复发性疾病。
