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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., (© 2019 Japan Pediatric Society.)

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, highly aggressive hematopoietic malignancy, characterized by cutaneous and bone marrow involvement and leukemic spread. Cutaneous involvement is the most common presentation in BPDCN. At present, the diagnosis and management of BPDCN are still challenging. Due to its rarity, the pediatric experience with BPDCN is especially limited. Herein, we report a special case of BPDCN with diffuse nodular lung metastases and a cutaneous lesion, which achieved a dramatic response to non-Hodgkin lymphoma regimen and remained with complete remission for 2 years. To date, acute lymphoblastic leukemia (ALL)-type chemotherapy followed by hematopoietic stem cell transplantation (SCT) is commonly thought to be related to a favorable outcome in adults with BPDCN. In contrast to it, ALL or non-Hodgkin lymphoma-type therapy alone seems enough in children with BPDCN with or without cutaneous lesions. SCT should only be performed for children who relapse and achieve a second remission. For pediatric BPDCN, further study of larger numbers of cases is needed to better define prognostic factors and optimal treatment strategy, and understand the underling differences in pathogenesis between children and adults.