关键词: ALPPS PRETEXT III case series hepatoblastoma liver transplantation

来  源:   DOI:10.3389/fped.2024.1350697   PDF(Pubmed)

Abstract:
UNASSIGNED: Hepatoblastoma is the most common malignant primary liver tumor in the pediatric population, accounting for 67% of cases in the United States. Surgical resection is the only curative treatment option; however, it can be performed in only 10% of patients with primary tumors. The two most common limitations for resection are the need for extensive resections and tumors in central locations. The therapeutic hypertrophy of healthy tissue achieved with ALPPS (Associating Liver Partition and Portal vein ligation for Staged Hepatectomy) enables larger resections and has been successfully employed in the pediatric population in recent years.
UNASSIGNED: To present three cases of patients with centrally located PRETEXT II or III hepatoblastomas who underwent ALPPS procedure as a viable therapeutic alternative to liver transplantation.
UNASSIGNED: Central PRETEXT III hepatoblastomas are typically indications for liver transplantation. Transplantation offers high five-year survival rates (73%). However, the associated morbidity, healthcare system costs, and limited availability make it necessary to explore alternative options. Series have reported the successful application of the ALPPS procedure in PRETEXT II and PRETEXT III hepatoblastomas in other locations. Therapeutically induced hypertrophy, characterized by an increase in the volume of healthy tissue in unaffected lobes or segments, enabled the resection of previously deemed unresectable lesions. The patients experienced uncomplicated postoperative courses and expected reduction in tumor markers. Chemotherapy selection followed the guidelines outlined in Block C of the SIOPEL IV protocol.
UNASSIGNED: ALPPS hepatectomy is a viable therapeutic option for patients with centrally located PRETEXT III or II hepatoblastomas.
摘要:
肝母细胞瘤是儿科人群中最常见的恶性原发性肝肿瘤,占美国病例的67%。手术切除是唯一的治疗选择;然而,它可以在只有10%的原发性肿瘤患者中进行。切除的两个最常见的限制是需要广泛切除和中央位置的肿瘤。使用ALPPS(联合肝分区和门静脉结扎用于分期肝切除术)实现的健康组织的治疗性肥大可以进行更大的切除,并且近年来已成功用于儿科人群。
介绍3例位于中央的PRETEXTII或III型肝母细胞瘤患者接受ALPPS手术作为肝移植的可行治疗替代方案。
中央PRETEXTIII型肝母细胞瘤通常是肝移植的适应症。移植提供了很高的五年生存率(73%)。然而,相关的发病率,医疗保健系统成本,和有限的可用性使得有必要探索替代方案。系列报道了ALPPS程序在其他位置的PRETEXTII和PRETEXTIII肝母细胞瘤中的成功应用。治疗性肥大,特征在于未受影响的叶或段中健康组织的体积增加,能够切除先前认为不可切除的病变。患者经历了简单的术后过程和预期的肿瘤标志物减少。化疗选择遵循SIOPLIV方案的第C部分概述的指南。
ALPPS肝切除术是位于中央的PRETEXTIII或II型肝母细胞瘤患者的可行治疗选择。
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