Babu Benoy I, Bigam David L, Gilmour Susan M, Dajani Khaled Z, Shapiro A M James, Kneteman Norman M
Department of Surgery, Section of Hepatobiliary, Pancreatic and Transplant Surgery, University of Alberta Hospital, Edmonton, AB, Canada.
Department of Pediatrics, Division of Pediatric Gastroenterology & Nutrition, Stollery Children's Hospital, Edmonton, AB, Canada.
Transplant Direct. 2021 Jan 15;7(2):e654. doi: 10.1097/TXD.0000000000001106. eCollection 2021 Feb.
Undifferentiated embryonal cell sarcoma (UESL) of the liver is the third most common malignant liver disease of childhood presenting as a rapidly enlarging intraabdominal mass. This systematic review explores the practicality of liver transplantation as a viable option in the treatment armamentarium for locally advanced undifferentiated embryonal cell sarcoma.
A systematic review of the literature was performed using Medline and Embase, from inception of databases to December 31, 2018. Keywords and MeSH headings used were embryonal sarcoma, mesenchymal sarcoma, and liver transplant. Reviews and manuscripts with incomplete data were excluded.
Twenty-eight patients had orthotopic liver transplantation (OLT) as a curative treatment option. The median age at presentation was 8 and 27 years in the pediatric and adult population, respectively, with a similar male to female ratio. A majority of the patients presented with abdominal pain, palpable mass, and a normal alpha-feto-protein. The median tumor size was 15 cm mainly affecting the right lobe (62%) of the liver. Eighty-two percent of the patients underwent primary OLT and 5 patients had salvage OLT. One death (3.6%) was due to initial misdiagnosis and management for hepatoblastoma. Recurrence was noted in 7.1% of the population. The median follow-up was noted to be 28.5 months. The documented survival rate post-liver transplant for UESL was 96%.
Based on available data and the very positive results therein, liver transplantation is a practical and justifiable use of a scarce resource as a treatment option for locally unresectable, undifferentiated embryonal cell sarcoma. The authors propose (accepting existence of different proposals) neoadjuvant therapy before curative resection, and if not achievable, then liver transplantation followed by adjuvant chemotherapy is an option for suitable candidates. For recurrent tumors after surgical resection, adjuvant therapy with salvage liver transplantation is an option.
肝脏未分化胚胎性细胞肉瘤（UESL）是儿童期第三常见的恶性肝脏疾病，表现为迅速增大的腹腔内肿块。本系统评价探讨了肝移植作为局部晚期未分化胚胎性细胞肉瘤治疗手段中一种可行选择的实用性。
使用Medline和Embase对文献进行系统评价，检索时间从数据库建立至2018年12月31日。使用的关键词和医学主题词为胚胎性肉瘤、间叶性肉瘤和肝移植。排除数据不完整的综述和手稿。
28例患者接受原位肝移植（OLT）作为根治性治疗选择。儿童和成人患者的中位发病年龄分别为8岁和27岁，男女比例相似。大多数患者表现为腹痛、可触及肿块，甲胎蛋白正常。中位肿瘤大小为15厘米，主要累及肝脏右叶（62%）。82%的患者接受了初次OLT，5例患者接受了挽救性OLT。1例死亡（3.6%）是由于最初将其误诊为肝母细胞瘤并进行了不恰当治疗。7.1%的患者出现复发。中位随访时间为28.5个月。记录的UESL肝移植后生存率为96%。
基于现有数据及其非常积极的结果，肝移植作为一种治疗选择，对局部不可切除的未分化胚胎性细胞肉瘤而言，是对稀缺资源的一种切实可行且合理的利用方式。作者建议（接受不同建议的存在）在根治性切除前进行新辅助治疗，如果无法实现，则对于合适的候选者，肝移植后进行辅助化疗是一种选择。对于手术切除后复发的肿瘤，挽救性肝移植辅助治疗是一种选择。
