背景:原发性肝卵黄囊瘤(YSTs)在成人中很少见。肝切除术是原发性肝YST的公认治疗方式。肝移植可能为无法切除的病例提供可能的治愈方法。
方法:我们介绍了一例31岁的腹部包块妇女,甲胎蛋白(AFP)水平异常升高(31,132ng/mL;正常:0-7ng/mL)。对比增强计算机断层扫描(CT)显示位于两个肝叶的大肿瘤,动脉增强和静脉冲洗。氟-18氟脱氧葡萄糖(18F-FDG)正电子发射断层扫描(PET)/CT显示18F-FDG摄取增加(最大标准化摄取值,24.4)在肝脏肿瘤和左侧中部腹内结节中。
方法:诊断为原发性肝YST伴大网膜转移。
方法:患者经肝动脉化疗栓塞(TACE)后行原位肝移植和腹内结节切除术。术中,大网膜有腹内结节.肝肿瘤的组织病理学检查显示席勒-杜瓦尔体。给予原肌球蛋白受体激酶(TRK)抑制剂larotrectinib,接着是四个周期的博莱霉素化疗,依托泊苷,和顺铂基于下一代测序结果。
结果:AFP水平下降到正常范围内。在34个月的随访期间未观察到肿瘤塌陷的证据。
结论:本病例提示多模式治疗以肝移植为主,包括术前TACE,术后辅助化疗,和TRK抑制剂,是不可切除的原发性肝YST的有效治疗方式。
BACKGROUND: Primary hepatic yolk sac tumors (YSTs) are rare in adults. Liver resection is an acknowledged treatment modality for primary hepatic YST. Liver transplantation may offer a possible cure for unresectable cases.
METHODS: We present a case of a 31-year-old woman with an abdominal mass who had abnormally elevated alpha-fetoprotein (AFP) levels (31,132 ng/mL; normal: 0-7 ng/mL). Contrast-enhanced computed tomography (CT) revealed large tumors located in both lobes of the liver, with arterial enhancement and venous washout. Fluorine-18 fluorodeoxyglucose (18F-FDG) positron emission tomography (PET)/CT indicated increased 18F-FDG uptake (maximum standardized uptake value, 24.4) in the liver tumors and left middle intra-abdominal nodule.
METHODS: The diagnosis was primary hepatic YST with metastasis to the greater omentum.
METHODS: The patient underwent orthotopic liver transplantation and intra-abdominal nodule resection after transarterial chemoembolization (TACE) as a bridge. Intraoperatively, an intra-abdominal nodule was confirmed in the greater omentum. Histopathological examination of the liver tumors revealed Schiller-Duval bodies. The tropomyosin receptor kinase (TRK) inhibitor larotrectinib was administered, followed by four cycles of chemotherapy with bleomycin, etoposide, and cisplatin based on the next-generation sequencing results.
RESULTS: The AFP level decreased to within the normal range. No evidence of tumor collapse was observed during the 34-month follow-up period.
CONCLUSIONS: This case suggests that multimodal therapy dominated by liver transplantation, including preoperative TACE, postoperative adjuvant chemotherapy, and TRK inhibitors, is an effective treatment modality for unresectable primary hepatic YST.