Novel treatment of refractory / recurrent pulmonary hepatoblastoma.

BACKGROUND: There is no consensus about how to manage pulmonary metastasis in patients with hepatoblastoma. We reviewed a treatment with a combination of oxaliplatin, vincristine, and topotecan (OVT) paired with radiofrequency ablation (RFA) of 12 patients with multiple refractory / recurrent pulmonary hepatoblastoma. METHODS: The medical records from patients with ≤21 years of age presenting with multiple deposits (≥2) of refractory / recurrent pulmonary hepatoblastoma were reviewed. The following data were extracted from each patient: age, gender, histological subtyping, cycles of OVT, tumor size, biomarkers, chemotherapy regimen and dosage, RFA details, treatment response, follow up, and patient outcomes. The primary outcome measure was the complete response (CR) of pulmonary diseases, and secondary outcomes were event-free survival rate and overall survival rate. RESULTS: Of 12 assessable patients, three (25%) (95% CI, 46.3-104) patients achieved PR (partial resopnse) after they finished OVT. After RFA, five (41.7%) (95% CI, 8.95-74.4) patients achieved CR (complete response). The 2 year event-free survival rate was 33% (95% CI, 20.5-64.6). The 2 year overall survival for the study group was 41.7% (95% CI, 8.9-74.4). All toxicity events were handled satisfactorily and no toxic related deaths were observed. CONCLUSIONS: Our review report shows that OVT combined with RFA can be a successful treatment modality for previously heavily treated refractory / recurrent pulmonary metastatic lesions from hepatoblastoma.

Unexpected vincristine-induced systemic capillary leak syndrome in patients with Wilm's tumor: A single institution experience.

Capillary leak syndrome (CLS) is a rare but fatal disease, which has been reported following the infusions of interleukin-2, tumor necrosis factor, granulocyte-colony stimulating factor, certain monoclonal antibodies, and gemcitabine, suggesting that drugs can also cause CLS. In this study, seven Wilm's tumor cases with CLS had been presented, which was suggested to be caused following administration of vincristine (VCR). From January 1st, 2014 to December 31st, 2016, medical records from Wilm's tumor patients were reviewed to identify those diagnosed with CLS. Moreover, the following data were extracted for each patient, including age, gender, histological subtyping, tumor stage, risk group, biomarkers, chemotherapy regimen and dosage, surgery details, clinical manifestation of CLS, treatment regimen of CLS, and patient outcomes. From January 1st, 2014 to December 31st, 2016, a total of seven patients with Wilms tumor were identified with a diagnosis of VCR-associated CLS. Typically, for these seven cases in our study, the predominant features of CLS included interstitial pneumonia and pulmonary edema. Moreover, steroid therapy was demonstrated as the most effective therapy in our study. The clinical features of VCR-induced CLS are distinct, and pediatric oncologists should be aware of CLS that manifests as interstitial pneumonia and pulmonary edema during the VCR treatment for patients with Wilm's tumor.