Okur Arzu, Eser Eylem Pinar, Yilmaz Güldal, Dalgiç Aydin, Akdemir Ümit Özgür, Oğuz Aynur, Karadeniz Ceyda, Akyol Gülen, Demiroğullari Billur, Boyunağa Öznur, Pinarli Faruk Güçlü
Departments of *Pediatric Oncology †Pathology ‡Surgery §Nuclear Medicine ∥Pediatric Surgery ¶Radiology, Gazi University Medical Faculty, Beşevler, Ankara, Turkey.
J Pediatr Hematol Oncol. 2014 Jul;36(5):e328-32. doi: 10.1097/MPH.0000000000000137.
Fibrolamellar variant of hepatocellular carcinoma (FLHCC) does not have a favorable prognosis than conventional HCC, and there is no difference regarding the response to chemotherapy and the degree of surgical resectability. FLHCC commonly recurs after complete surgical resection, and there is a high rate of lymph node metastases. Herein, we report a 12-year-old girl with metastatic FLHCC with multiple recurrences aggressively treated with surgery, chemotherapy, and antiangiogenic agents. She is in complete remission after 4 years and 2 months after the diagnosis of metastatic FLHCC. The standard treatment of FLHCC is excision of the primary tumor and its metastases. Chemotherapy for FLHCC is controversial, and it has been suggested that cytoreductive chemotherapy was ineffective and adjuvant chemotherapy did not improve survival. Our patient with multiple recurrences was successfully treated with surgery, first-line chemotherapy with cisplatin and doxorubicin, second-line chemotherapy with 5-fluorouracil/interferon-α combination, and adjuvant antiangiogenic agents like cyclophosphamide and thalidomide. As FLHCC patients have no underlying liver disease, they can tolerate higher doses of chemotherapy compared with conventional HCC patients. We support the use of repeated aggressive surgery with adjuvant chemotherapy and antiangiogenic therapy, which provided complete remission in our patient with metastatic and recurrent FLHCC.
肝细胞癌纤维板层样变异型（FLHCC）的预后并不优于传统肝细胞癌，在化疗反应和手术可切除程度方面也没有差异。FLHCC在完整手术切除后常复发，且淋巴结转移率高。在此，我们报告一名12岁患有转移性FLHCC且多次复发的女孩，积极接受了手术、化疗和抗血管生成药物治疗。在诊断转移性FLHCC后4年零2个月，她处于完全缓解状态。FLHCC的标准治疗是切除原发肿瘤及其转移灶。FLHCC的化疗存在争议，有人认为减瘤化疗无效，辅助化疗也不能提高生存率。我们这名多次复发的患者通过手术、顺铂和阿霉素一线化疗、5-氟尿嘧啶/干扰素-α联合二线化疗以及环磷酰胺和沙利度胺等辅助抗血管生成药物成功得到治疗。由于FLHCC患者没有潜在肝脏疾病，与传统肝细胞癌患者相比，他们能够耐受更高剂量的化疗。我们支持对转移性和复发性FLHCC患者采用重复的积极手术联合辅助化疗和抗血管生成治疗，这使我们的患者实现了完全缓解。
