Multidisciplinary effort in treating children with hepatoblastoma in China.

The purpose of this study is to report the first nationwide protocol (Wuhan Protocol) developed by Chinese Children's Cancer Group and the results of multidisciplinary effort in treating hepatoblastoma. In this study, we reported the final analysis, which includes 153 hepatoblastoma patients in 13 hospitals from January 2006 to December 2013. The 6-year overall survival and event-free survival rates were 83.3 ± 3.1% and 71.0 ± 3.7%, respectively, in this cohort. The univariate analysis revealed that female (P = 0.027), under 5 years of age (P = 0.039), complete surgical resection (P = 0.000), no metastases (P = 0.000), and delayed surgery following neoadjuvant chemotherapy (P = 0.000) had better prognosis. In multivariate analysis, male, 5 years of age or above, stage PRETEXT III or IV, and incomplete surgical resection were among the some adverse factors contributing to poor prognosis. The preliminary results from this study showed that patients who underwent treatment following Wuhan Protocol had similar OS and EFS rates compared to those in developed countries. However, the protocol remains to be further optimized in standardizing surgical resection (including liver transplantation), refining risk stratification and risk-based chemotherapy.

Experience of fluoroscopy-aided thoracoscopic resection of pulmonary nodule localised with Lipiodol in a child.

We report the case of a 12-year-old boy with a huge liver tumour 20 cm in diameter with multiple lung metastases. Six months after systemic chemotherapy was initiated, all tumours had disappeared with the exception of the liver tumour and a tiny lung tumour 2.5 mm in diameter. Fluoroscopy-assisted thoracoscopic resection of the pulmonary nodule was performed to evaluate whether viable tumour tissue remained in the lung lesion. Before moving the patient to the operating room, the nodule was marked by Lipiodol under CT fluoroscopic guidance with the patient under local anaesthesia. This procedure allowed correct visualisation of the area that should be resected.

Hepatoblastoma presenting with lung metastases: treatment results of the first cooperative, prospective study of the International Society of Paediatric Oncology on childhood liver tumors.

BACKGROUND: The prognosis of children who are affected by hepatoblastoma (HB) that presents with lung metastases has always been considered very poor. In light of the overall improvement in the survival of HB patients since the introduction of cisplatin (CDDP) in the therapeutic armament of this tumor, the question has been raised whether patients with metastatic HB also would benefit from this drug. The purpose of the current study was to address this issue by analyzing the treatment outcome of those patients presenting with metastases who entered into the first HB study on childhood liver tumors conducted by the International Society of Paediatric Oncology (SIOPEL 1). METHODS: SIOPEL 1 was a prospective, international, multicentric, single-arm study based on preoperative chemotherapy that was open to patient registration from January 1990 to February 1994. After undergoing a biopsy, patients received four courses of CDDP (80 mg/m(2) in a 24-hour, continuous infusion) on Day 1 followed by doxorubicin (60 mg/m(2) in a 48-hour, continuous infusion) on Days 2 and 3 (PLADO). Surgery was performed after four courses of PLADO and was followed by two more courses. Untreated children age < 16 years with biopsy-proven HB were eligible for the study. Metastatic spread was assessed by chest X-ray and, where available, lung computed tomography scan. RESULTS: Thirty-one of 154 children that entered into the trial presented with metastases. Eight children presently are alive with no evidence of disease (NED) after being treated with protocol therapy only (median follow-up, 60 months); nine children are alive with NED after having failed PLADO and having been rescued with alternative therapies (median follow-up, 80 months). The 5-year overall and event free survival rates for these children were 57% (95% confidence interval, 39-75%) and 28% (95% confidence interval, 12-44%), respectively. Persistent lung disease was the main reason for PLADO failure (17 of 23 patients; 74%). CONCLUSIONS: The SIOPEL 1 therapeutic strategy seems to cure 25% of the HB patients who present with metastases. However, further chemotherapy and the use of thoracotomies still can save significant numbers of these children.