Clinicopathologic features and survival in fibrolamellar carcinoma: comparison with conventional hepatocellular carcinoma with and without cirrhosis
Clinicopathologic features and survival in fibrolamellar carcinoma: comparison with conventional hepatocellular carcinoma with and without cirrhosis
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DOI：
10.1038/modpathol.3800449
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发表时间：
2005-11-01
期刊：
影响因子：
7.5
通讯作者：
Ferrell, LD
中科院分区：
文献类型：
--
作者：
Kakar, S;Burgart, LJ;Ferrell, LD
纤维板层癌发生于年轻人的非增生性肝脏，被认为比传统的肝细胞癌侵袭性低。本研究比较了纤维板层癌和肝细胞癌发生在非增生性和增生性肝脏的生存率和临床病理特征。本文报告20例纤维板层癌的临床病理特征，包括年龄、性别、肿瘤大小、分期及术后生存情况。生存率与切除的肝细胞癌无肝硬化（n = 32）和（n = 30）进行了比较。通过免疫组织化学测定Ki-67的抑制活性。在随访期间，12例（60%）纤维板层癌患者死亡，5年生存率为45%。纤维板层癌的死亡率在出现转移性疾病时更高（6/7，86% vs 5/13，39%，P = 0.06）。年龄、性别和肿瘤大小与生存率无关。纤维板层癌和无肝硬化的肝细胞癌的5年生存率（45 vs 56%，P = 0.4）和总生存率（40 vs 56.3%，P = 0.3）相似。肝细胞癌合并肝硬化的5年生存率和总生存率分别为27%和23.3%，与纤维板层癌相比无显著性差异（P = 0.2）。在无转移的病例中，纤维板层癌（62%）和无肝硬化的肝细胞癌（57%）的5年生存率明显优于肝细胞癌伴肝硬化（27%）（P = 0.03）。所有三组的平均Ki-67指数相似（P = 0.1）。总之，纤维板层癌是一种侵袭性肿瘤，5年生存率为45%，总死亡率为60%。近一半的患者发生淋巴结转移或远处转移。纤维板层癌的预后与常规肝细胞癌相似。在非转移性病例中，纤维板层癌和无肝硬化的肝细胞癌的预后好于肝细胞癌伴肝硬化。纤维板层癌较好的预后似乎是由于没有肝硬化，而不是其独特的临床病理特征。
Fibrolamellar carcinoma arises in noncirrhotic livers of young individuals and has been considered to be less aggressive than conventional hepatocellular carcinoma. This study compares survival and clinicopathologic features of fibrolamellar carcinoma with hepatocellular carcinoma arising in noncirrhotic and cirrhotic livers. Clinical and pathologic features including age, gender, tumor size, stage and survival were recorded in 20 resected cases of fibrolamellar carcinoma. Survival was compared with resected hepatocellular carcinoma without ( n = 32) and with cirrhosis ( n = 30). Proliferative activity was determined by immunohistochemistry for Ki-67. In all, 12 (60%) patients with fibrolamellar carcinoma died during follow-up; the 5-year survival was 45%. Mortality in fibrolamellar carcinoma was higher with metastatic disease at presentation (6/7, 86% vs 5/13, 39%, P = 0.06). Age, gender and tumor size did not correlate with survival. The 5-year (45 vs 56%, P = 0.4) as well as overall survival (40 vs 56.3%, P = 0.3) was similar in fibrolamellar carcinoma and hepatocellular carcinoma without cirrhosis. The 5-year and overall survival in hepatocellular carcinoma with cirrhosis was 27 and 23.3%, respectively, which was not significantly different compared to fibrolamellar carcinoma ( P = 0.2). Among the cases without metastases at presentation, 5-year survival in fibrolamellar carcinoma (62%) and hepatocellular carcinoma without cirrhosis (57%) was significantly better ( P = 0.03) than hepatocellular carcinoma with cirrhosis (27%). The mean Ki-67 index was similar in all three groups ( P = 0.1). In conclusion, fibrolamellar carcinoma is an aggressive neoplasm with 45% 5-year survival and overall mortality of 60%. Nearly half the patients develop lymph node or distant metastasis. The prognosis of fibrolamellar carcinoma is similar to conventional hepatocellular carcinoma. Among nonmetastatic cases, the prognosis is better in fibrolamellar carcinoma and hepatocellular carcinoma without cirrhosis compared to hepatocellular carcinoma with cirrhosis. The better outcome in fibrolamellar carcinoma appears to be due to the absence of cirrhosis rather than its distinct clinicopathologic features.