title: Successful multimodal treatment for aggressive metastatic and recurrent fibrolamellar hepatocellular carcinoma in a child.
abstract: 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.
authors.affiliation: Departments of *Pediatric Oncology †Pathology ‡Surgery §Nuclear Medicine ∥Pediatric Surgery ¶Radiology, Gazi University Medical Faculty, Beşevler, Ankara, Turkey.
authors.forename: Arzu
authors.initials: A
authors.lastname: Okur
authors.forename: Eylem Pinar
authors.initials: EP
authors.lastname: Eser
authors.forename: Güldal
authors.initials: G
authors.lastname: Yilmaz
authors.forename: Aydin
authors.initials: A
authors.lastname: Dalgiç
authors.forename: Ümit Özgür
authors.initials: ÜÖ
authors.lastname: Akdemir
authors.forename: Aynur
authors.initials: A
authors.lastname: Oğuz
authors.forename: Ceyda
authors.initials: C
authors.lastname: Karadeniz
authors.forename: Gülen
authors.initials: G
authors.lastname: Akyol
authors.forename: Billur
authors.initials: B
authors.lastname: Demiroğullari
authors.forename: Öznur
authors.initials: Ö
authors.lastname: Boyunağa
authors.forename: Faruk Güçlü
authors.initials: FG
authors.lastname: Pinarli
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D006528:Carcinoma, Hepatocellular / Q000556:secondary / Q000628:therapy*; D002648:Child; D002945:Cisplatin / Q000008:administration & dosage; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage; D005260:Female; D005472:Fluorouracil / Q000008:administration & dosage; D006498:Hepatectomy; D006801:Humans; D016898:Interferon-alpha / Q000008:administration & dosage; D008113:Liver Neoplasms / Q000473:pathology / Q000628:therapy*; D008207:Lymphatic Metastasis; D009364:Neoplasm Recurrence, Local / Q000473:pathology / Q000628:therapy*; D011379:Prognosis; D012074:Remission Induction; D013792:Thalidomide / Q000008:administration & dosage
chemical_list: D016898:Interferon-alpha; D013792:Thalidomide; D004317:Doxorubicin; D003520:Cyclophosphamide; D002945:Cisplatin; D005472:Fluorouracil