Background Hepatoblastoma is the most common primary malignant liver tumor in children and 20% patients have pulmonary metastasis at diagnosis, which highly accepted abnormal AFP as the marker of diagnosis and recurrence, but no one pays attention to other serum markers. Methods Clinical data and serological markers were collected from pediatric HB patients who received systematic treatment at our hospital. Multivariate logistic regression was performed to identify independent risk factors for pulmonary metastasis in HB. Cox proportional hazards regression was further applied to analyze prognostic factors in the pulmonary metastasis group. Results A total of 146 HB patients were included in this study, comprising 117 non-metastasis cases and 29 pulmonary metastasis cases. Subgroup analysis of the 28 pulmonary metastasis cases showed that presence of extrapulmonary metastasis and pulmonary metastasis resection were significant prognostic factors. Cox regression analysis further indicated that Initial D-D/FIB >1.6, GPR >1.35, postoperative 1-month AFP >324.4 ng/ml, postoperative 2-month AFP >185.9 ng/ml, presence of extrapulmonary metastasis were prognostic risk factors. Conclusions The pulmonary metastasis group showed significantly lower OS and EFS compared to the non-metastasis group. Multifocal liver tumors, initial tumor diameter >10cm, and vascular tumor thrombus were identified as independent risk factors for pulmonary metastasis in HB. Additionally, presence of extrapulmonary metastasis, postoperative 1-month AFP and postoperative 2-month AFP were independent prognostic predictors for HB patients with pulmonary metastasis. Serological markers may serve as valuable indicators for monitoring disease progression and guiding prognosis in HB with pulmonary metastasis.

Purpose: To evaluate and compare the efficacy of corticosteroid and vincristine (VCR) in the treatment of kaposiform hemangioendothelioma (KHE) and tufted angioma (TA). Methods: This was a multicenter prospective randomized controlled study. All patients with KHE/TA who meet the diagnostic criteria were included. The patients were randomized to methylprednisolone (MP) group and VCR group. The primary outcome was the single main parameter effective rate (SMPE) and overall effective rate (OE) of corticosteroid and VCR over one month after treatment. The single main parameters included platelets, fibrinogen, tumor size, texture and appearance. Results: In single main parameters, VCR was superior to corticosteroid in the relief of platelet (80.0% vs 44.0, P = 0.019) and tumor texture (68.9% vs 30.8%, P = 0.007). Although the efficacy of VCR on fibrinogen (23.3% vs 20.7%, P=1.000), tumor size (23.3% vs 13.8%, P=0.273) and appearance (65.5% vs 46.2%, P=0.120) were higher than that of corticosteroid, there was no significant difference (P > 0.05). And the overall effective rate of VCR was higher than that of corticosteroid (31.0% and 56.7% vs 31.0%), but the differences were also not statistically significant. (P=0.067). Conclusions: Our prospective data show that the therapeutic effect of VCR was significantly greater than that of corticosteroid with regard to treating thrombocytopenia and improving tumor texture. So, we recommend that VCR could be an option for first-line treatment in KHE/TA patients.

Objective: To investigate the long-term outcomes of extended hepatectomy for POST-TEXT III and IV hepatoblastoma. Methods: The clinical data of 35 patients with POST-TEXT III and IV hepatoblastoma from January 2009 to June 2019 were analyzed retrospectively. Results: There were 34 cases of POST-TEXT III and 1 cases of POST-TEXT IV, respectively. All 35 cases underwent hepatic resection, including 7 cases of hepatic trisectionectomy, 8 cases of extended hemihepatectomy, 4 cases of irregular hepatectomy, 12 cases of mesohepatectomy and 4 cases of ALPPS procedure. Pringle maneuver and Glissonean approach was used in 4 and 22 patients, respectively. The average blood loss of patients with Glissonean approach (147.73 ± 137.46ml) was significantly less than that of patients without the approach (387.69 ± 235.69ml; P = 0.001). The 5-year overall survival rate and event-free survival rate was 79.2% and 73.7%, respectively. According to the classification of tumor margin distance, the 5-year overall survival rates of the tumors with margin > 1cm, 0.5-1cm, < 0.5cm and close to the tumor margin were 100%, 78.0%, 83.3% and 53.3%, respectively (P=0.371). Seven patients developed recurrence which occurred within 1 year after tumor resection. Conclusion: Non-transplant extended hepatic resection is a feasible approach for POST-TEXT III and IV hepatoblastomas. On the basis of mastering the Glisson approach and ensuring a certain resection margin, it is possible to achieve a similar oncological outcome to liver transplantation.