PDF(Pubmed)
Abstract：
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.
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.
摘要：
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。
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。
