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  • Successful treatment of chi...
    Zsíros, József; Maibach, Rudolf; Shafford, Elizabeth; Brugieres, Laurence; Brock, Penelope; Czauderna, Piotr; Roebuck, Derek; Childs, Margaret; Zimmermann, Arthur; Laithier, Veronique; Otte, Jean-Bernard; de Camargo, Beatriz; MacKinlay, Gordon; Scopinaro, Marcelo; Aronson, Daniel; Plaschkes, Jack; Perilongo, Giorgio

    Journal of clinical oncology, 05/2010, Letnik: 28, Številka: 15
    Journal Article

    The primary objective was to determine the efficacy of a newly designed preoperative chemotherapy regimen in an attempt to improve the cure rate of children with high-risk hepatoblastoma. High risk was defined as follows: tumor in all liver sections (ie, Pretreatment Extension IV PRETEXT-IV), or vascular invasion (portal vein P+, three hepatic veins V+), or intra-abdominal extrahepatic extension (E+), or metastatic disease, or alpha-fetoprotein less than 100 ng/mL at diagnosis. Patients were treated with alternating cycles of cisplatin and carboplatin plus doxorubicin (preoperatively, n = 7; postoperatively, n = 3) and delayed tumor resection. Of the 151 patients (150 evaluable for response) 118 (78.7%) achieved a partial response to chemotherapy. Complete resection of the liver tumor could be achieved in 115 patients (76.2%) either by partial hepatectomy (55.6%) or by liver transplantation (20.6%). In 106 children (70.2%), complete resection of all tumor lesions (including metastases) was achieved. Among the patients with initial lung metastases, 52.2% achieved complete remission of the lung lesions with chemotherapy alone. In half of the patients with initial PRETEXT-IV tumor as the only high-risk feature, the tumor could be completely resected with partial hepatectomy. Event-free (EFS) and overall survival (OS) estimates at 3 years were 65% (95% CI, 57% to 73%) and 69% (95% CI, 62% to 77%) for the whole group. EFS and OS for all patients with PRETEXT-IV tumor were 68% and 69%, respectively, and they were 56% and 62%, respectively, for patients with metastasis. The applied treatment rendered a great proportion of tumors resectable, and, in comparison with previously published results, led to an improved survival in patients with high-risk hepatoblastoma.