Li Jian, He Lulu, Wu Qizi, Zhou Jianfeng, Zhou Li, Li Tao
Department of Oncology, Children's Hospital of Nanjing Medical University, Nanjing, China.
Department of Hematology, Children's Hospital of Nanjing Medical University, Nanjing, China.
Transl Pediatr. 2024 May 31;13(5):784-790. doi: 10.21037/tp-24-10. Epub 2024 May 27.
Undifferentiated embryonal sarcoma of the liver (UESL) is a rare and highly aggressive malignancy predominantly affecting children and adolescents. Managing UESL is particularly intricate due to its aggressive nature and the limited array of treatment options available. This study is dedicated to elucidating the efficacy of a multimodal therapeutic strategy in the successful management of UESL.
Four pediatric patients (two males, two females; aged 6-11 years) diagnosed with UESL were treated at the Children's Hospital of Nanjing Medical University between November 2019 and June 2023. Surgical resection with lymph node dissection achieved complete primary tumor eradication. Adjuvant chemotherapy tailored to each patient's needs was followed by localized radiation therapy. After 9-42 months of follow-up, one patient who did not undergo immediate radiotherapy experienced a relapse. Following a second operation coupled with radiotherapy, the patient achieved complete remission, and mirroring the status of the other three patients who are now presently in remission. The overall cohort exhibited commendable tolerance to the treatment regimen, with manageable chemotherapy-related toxicities.
This case series suggests that implementing a standardized protocol of resection, followed by adjuvant chemotherapy and radiation, can lead to favorable outcomes in pediatric patients diagnosed with UESL. Nevertheless, the need for comprehensive large-scale studies is imperative to substantiate the effectiveness of this approach.
肝未分化胚胎性肉瘤（UESL）是一种罕见且高度侵袭性的恶性肿瘤，主要影响儿童和青少年。由于其侵袭性本质以及可用治疗选择有限，管理UESL特别复杂。本研究致力于阐明多模式治疗策略在成功管理UESL中的疗效。
2019年11月至2023年6月期间，南京医科大学儿童医院对4例诊断为UESL的儿科患者（2例男性，2例女性；年龄6 - 11岁）进行了治疗。手术切除并清扫淋巴结实现了原发性肿瘤的完全根除。根据每位患者的需求进行辅助化疗，随后进行局部放疗。经过9 - 42个月的随访，1例未立即接受放疗的患者出现复发。在进行第二次手术并联合放疗后，该患者实现完全缓解，其他3例患者目前也处于缓解状态。整个队列对治疗方案表现出良好的耐受性，化疗相关毒性可控。
该病例系列表明，实施标准化的切除方案，随后进行辅助化疗和放疗，可使诊断为UESL的儿科患者获得良好预后。然而，迫切需要开展全面的大规模研究以证实该方法的有效性。
