He Min, Cai Jia-Bin, Lai Can, Mao Jun-Qing, Xiong Jie-Ni, Guan Zhong-Hai, Li Lin-Jie, Shu Qiang, Ying Mei-Dan, Wang Jin-Hu
Department of Surgical Oncology, The Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou 310052, Zhejiang Province, China.
Department of Radiology, The Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou 310052, Zhejiang Province, China.
World J Clin Cases. 2022 Jul 6;10(19):6437-6445. doi: 10.12998/wjcc.v10.i19.6437.
Undifferentiated embryonal sarcoma of the liver (UESL) is a rare and aggressive mesenchymal tumor in children. Herein, we describe our experience in neoadjuvant therapy (NAT) and subsequent surgery for the treatment of UESL in children.
To evaluate the efficacy of NAT and explore a new choice for successful operation of UESL in children.
We retrospectively analyzed six patients newly diagnosed with unresectable UESL who received NAT and then surgery at our center between January 2004 and December 2019. The tumor was considered unresectable if it involved a large part of both lobes of the liver or had invaded the main hepatic vessels or inferior vena cava. The NAT included preoperative transcatheter arterial chemoembolization (TACE) and systemic chemotherapy. The patients were 4 boys and 2 girls with a mean age of 7 years. The longest tumor at presentation ranged from 8.6 to 14.8 cm (mean, 12 cm). Extrahepatic metastases were present in 2 cases. Preoperative systemic chemotherapy was administered 3 wk after TACE. Tumor resection was performed 3 wk after one or two cycles of NAT. The patients received systemic chemotherapy after surgery.
All patients successfully underwent NAT and complete resection. The tumor volumes decreased by 18.2%-68.7%, with a mean decrease of 36% after 1 cycle of NAT ( = 3.524, = 0.017). According to the Response Evaluation Criteria In Solid Tumors criteria, 4 patients had a partial response and underwent surgery, while 2 had stable disease and received another cycle of NAT before surgery. Massive tumor necrosis was seen on pathological examination of the surgical specimen: > 90% necrosis in two, > 50% necrosis in three, and 25% necrosis in 1, with an average of 71.8%. Post-NAT complications included fever, nausea and vomiting, and mild bone marrow suppression. Elevated alanine transaminase levels occurred in all patients, which returned to normal within 7-10 d after treatment. No cardiac or renal toxicity, severe hepatic dysfunction, bleeding and non-target embolization were observed in the patients. The median follow-up period was 8 years with an overall survival of 100%.
NAT effectively reduced tumor volume, cleared the tumor margin, and caused massive tumor necrosis. This may be a promising choice for successful surgery of UESL in children.
肝未分化胚胎性肉瘤（UESL）是儿童中一种罕见且侵袭性强的间充质肿瘤。在此，我们描述我们在儿童UESL治疗中进行新辅助治疗（NAT）及后续手术的经验。
评估NAT的疗效，并探索儿童UESL成功手术的新选择。
我们回顾性分析了2004年1月至2019年12月期间在我们中心新诊断为不可切除UESL并接受NAT然后手术的6例患者。如果肿瘤累及肝脏两叶的大部分或侵犯了肝主要血管或下腔静脉，则认为该肿瘤不可切除。NAT包括术前经动脉化疗栓塞（TACE）和全身化疗。患者为4名男孩和2名女孩，平均年龄7岁。就诊时最长肿瘤范围为8.6至14.8厘米（平均12厘米）。2例存在肝外转移。TACE后3周给予术前全身化疗。在1个或2个周期的NAT后3周进行肿瘤切除。患者术后接受全身化疗。
所有患者均成功接受NAT并完全切除。肿瘤体积减少了18.2% - 68.7%，1个周期的NAT后平均减少36%（ = 3.524， = 0.017）。根据实体瘤疗效评价标准，4例患者部分缓解并接受手术，2例病情稳定，术前接受了另1个周期的NAT。手术标本的病理检查可见大量肿瘤坏死：2例坏死> 90%，3例坏死> 50%，1例坏死25%，平均坏死率为71.8%。NAT后的并发症包括发热、恶心呕吐和轻度骨髓抑制。所有患者均出现丙氨酸转氨酶水平升高，治疗后7 - 10天恢复正常。患者未观察到心脏或肾脏毒性、严重肝功能障碍、出血和非靶栓塞。中位随访期为8年，总生存率为100%。
NAT有效缩小了肿瘤体积，清除了肿瘤边界，并导致大量肿瘤坏死。这可能是儿童UESL成功手术的一个有前景的选择。
