doiSerbia

Clinical characteristics and survival of children with hepatoblastoma: Single centre experience

Krstovski Nada (Univerzitetska dečja klinika, Beograd)
Janić Dragana (Univerzitetska dečja klinika, Beograd)
Dokmanović Lidija (Univerzitetska dečja klinika, Beograd)
Smoljanić Željko (Univerzitetska dečja klinika, Beograd)
Vukanić Dragan (Univerzitetska dečja klinika, Beograd)

INTRODUCTION Hepatoblastoma is the most frequent malignant liver tumour of childhood and it accounts for 1% of all paediatric cancers. The outcome is significantly improved by introducing intensive chemotherapy regimens followed by complete surgical tumour resection. The long-term survival is 75-95% at present. OBJECTIVE To summarize clinical characteristics and treatment results in children with hepatoblastoma. METHOD The patients diagnosed with hepatoblastoma and treated at the Department of Haematology of the University Children's Hospital in Belgrade, between January 1995 and December 2007 were retrospectively analyzed. We analyzed mode of therapy, surgical management and patients' survival according to Kaplan Mayer statistical test. RESULTS Eleven patients were treated (ten boys and one girl), aged from 6 months to 13 years, mean 32 months, median 12 months. The most frequent presenting sign was abdominal distension. Routine laboratory examination showed elevated alpha-fetoprotein (AFP), range 147 to 44880 ng/ml, mean 8667 ng/ml. Ten patients (91%) had elevated platelet count, range 450 to 909x109/l. Initial lung metastasis developed in 3 patients (27%). The diagnosis was established by an open surgical biopsy in 6 patients and after complete tumour resection in 4 patients, namely 2 patients after preoperative chemotherapy and in 2 patients with no previous chemotherapy. Histopathology verified hepatoblastoma in 10 patients: in 7 patients with epithelial type and in 3 patients with mixed epithelial-mesenchyme type. Six patients were in continuous remission with mean follow up of 58 months. One patient was lost to follow up. Four patients died; three patients due to disease progression and one patient due to sepsis after the first cycle of chemotherapy. CONCLUSION Combined preoperative chemotherapy with complete tumour resection followed by postoperative chemotherapy results in a high percentage of definitive cures.

Keywords: hepatoblastoma, children, survival