Bhaijee Feriyl, Locketz Michael L, Krige Jake E J
Surgical Gastroenterology Unit, Groote Schuur Hospital, Cape Town.
S Afr J Surg. 2009 Nov;47(4):108-11.
Fibrolamellar carcinoma (FLC) is an uncommon malignant tumour of hepatocyte origin that differs from hepatocellular carcinoma (HCC) in aetiology, demographics, condition of the affected liver, and tumour markers. Controversy exists whether FLC demonstrates a more favourable prognosis than typical HCC. A review of existing literature reveals a dearth of FLC data from the African continent.
We utilised the prospective liver resection database at Groote Schuur Hospital to identify all patients who underwent surgery for FLC between 1990 and 2008.
Seven patients (median age 21 years, range 19 - 42, 5 men, 2 women) underwent surgery for FLC. No patient had underlying liver disease or an elevated alpha fetoprotein (AFP) at either initial presentation or recurrence. Six patients had a solitary tumour at diagnosis (mean largest diameter = 12cm), and underwent left hepatectomy (N=2), right hepatectomy (N=1), extended right hepatectomy (N=1), right hepatectomy (N=1), extended right hepatectomy (N=1), and segmentectomies (N=2). Three patients underwent a portal lymphadenectomy for regional lymphatic tumour involvement. One patient with advanced extrahepatic portal nodal metastasis was unresectable. No peri-operative deaths occurred. Recurrence occurred post resection in all 6 patients. Median overall survival was 60 months, and overall 5-year survival was 4 out of 7 (57%). Post-resection survival (N=6) was 61 months, with a 5-year survival rate of 4 out of 6 (67%). The patient with unresectable disease survived 38 months after tumour embolisation with Lipiodol.
Our series suggests that despite (i) a high resection rate of solitary lesions with clear tumour resection margins, and (ii) absence of underlying liver disease, FLC has a high recurrence rate with an ultimately poor clinical outcome. These findings concur with recent international experience of FLC. experience of FLC.
纤维板层癌（FLC）是一种罕见的肝细胞源性恶性肿瘤，在病因、人口统计学、患肝状况及肿瘤标志物方面与肝细胞癌（HCC）有所不同。对于FLC的预后是否比典型HCC更有利存在争议。对现有文献的回顾显示非洲大陆缺乏FLC数据。
我们利用格罗特舒尔医院的前瞻性肝切除数据库，确定1990年至2008年间所有接受FLC手术的患者。
7例患者（中位年龄21岁，范围19 - 42岁，5例男性，2例女性）接受了FLC手术。在初次就诊或复发时，无患者有潜在肝脏疾病或甲胎蛋白（AFP）升高。6例患者诊断时为孤立性肿瘤（平均最大直径 = 12cm），并接受了左肝切除术（N = 2）、右肝切除术（N = 1）、扩大右肝切除术（N = 1）、右肝切除术（N = 1）、扩大右肝切除术（N = 1）和肝段切除术（N = 2）。3例患者因区域淋巴结肿瘤受累接受了门静脉淋巴结清扫术。1例有晚期肝外门静脉淋巴结转移的患者无法切除。无围手术期死亡发生。所有6例患者术后均复发。中位总生存期为60个月，7例患者中5年总生存率为4例（57%）。切除术后生存期（N = 6）为61个月，5年生存率为6例中的4例（67%）。无法切除疾病的患者在使用碘油进行肿瘤栓塞后存活了38个月。
我们的系列研究表明，尽管（i）孤立性病变的切除率高且肿瘤切除边缘清晰，以及（ii）无潜在肝脏疾病，但FLC复发率高，最终临床结局较差。这些发现与最近关于FLC的国际经验一致。FLC的经验。
