Whole Lung Irradiation after High-Dose Busulfan/Melphalan in Ewing Sarcoma with Lung Metastases: An Italian Sarcoma Group and Associazione Italiana Ematologia Oncologia Pediatrica Joint Study.

PURPOSE: To analyze toxicity and outcome predictors in Ewing sarcoma patients with lung metastases treated with busulfan and melphalan (BU-MEL) followed by whole-lung irradiation (WLI). METHODS: This retrospective study included 68 lung metastatic Ewing Sarcoma patients who underwent WLI after BU-MEL with autologous stem cell transplantation, as part of two prospective and consecutive treatment protocols. WLI 12 Gy for <14 years old and 15 Gy for ≥14 years old patients were applied at least eight weeks after BU-MEL. Toxicity, overall survival (OS), event-free survival (EFS) and pulmonary relapse-free survival (PRFS) were estimated and analyzed. RESULTS: After WLI, grade 1-2 and grade 3 clinical toxicity was reported in 16.2% and 5.9% patients, respectively. The five-year OS, EFS and PRFS with 95% confidence interval (CI) were 69.8% (57.1-79.3), 61.2% (48.4-71.7) and 70.5% (56.3-80.8), respectively. Patients with good histological necrosis of the primary tumor after neoadjuvant chemotherapy showed a significant decreased risk of pulmonary relapse or death compared to patients with poor histological necrosis. CONCLUSIONS: WLI at recommended doses and time interval after BU-MEL is feasible and might contribute to the disease control in Ewing sarcoma with lung metastases and responsive disease. Further studies are needed to explore the treatment stratification based on the histological response of the primary tumor.

Ante situm liver resection with inferior vena cava replacement under hypothermic cardiopolmunary bypass for hepatoblastoma: Report of a case and review of the literature.

INTRODUCTION: Hepatoblastoma with tumour thrombi extending into inferior-vena-cava and right atrium are often unresectable with an extremely poor prognosis. The surgical approach is technically challenging and might require major liver resection with vascular reconstruction and extracorporeal circulation. However, which is the best surgical technique is yet unclear. PRESENTATION OF CASE: A 11-months-old boy was referred for a right hepatic lobe mass(90×78mm) suspicious of hepatoblastoma with tumoral thrombi extending into the inferior-vena-cava and the right atrium, bilateral lung lesions and serum alpha-fetoprotein level of 50.795IU/mL. After 8 months of chemotherapy (SIOPEL 2004-high-risk-Protocol), the lung lesions were no longer clearly visible and the hepatoblastoma size decreased to 61×64mm. Thus, ante situm liver resection was planned: after hepatic parenchymal transection, hypothermic cardiopulmonary bypass was started and en bloc resection of the extended-right hepatic lobe, the retro/suprahepatic cava and the tumoral trombi was performed with concomitant cold perfusion of the remnant liver. The inferior-vena-cava was replaced with an aortic graft from a blood-group compatible cadaveric donor. The post-operative course was uneventful and after 8 months of follow-up the child has normal liver function and an alpha-fetoprotein level and is free of disease recurrence with patent vascular graft. CONCLUSIONS: We report for the first time a case of ante situ liver resection and inferior-vena-cava replacement associated with hypothermic cardiopulmonary bypass in a child with hepatoblastoma. Herein, we extensively review the literature for hepatoblastoma with thumoral thrombi and we describe the technical aspects of ante situm approach, which is a realistic option in otherwise unresectable hepatoblastoma.