Department of Interventional and Vascular surgery, Xinhua Hospital Affiliated to Shanghai Jiaotong University School of Medicine, Shanghai, China.
Department of Radiology, Xinhua Hospital Affiliated to Shanghai Jiaotong University School of Medicine, Shanghai, China.
Medicine (Baltimore). 2023 Dec 15;102(50):e35821. doi: 10.1097/MD.0000000000035821.
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.
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.
The diagnosis was primary hepatic YST with metastasis to the greater omentum.
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.
The AFP level decreased to within the normal range. No evidence of tumor collapse was observed during the 34-month follow-up period.
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.
成人原发性肝卵黄囊瘤（YST）较为罕见。肝切除术是治疗原发性肝 YST 的公认方法。肝移植可能为不可切除病例提供一种可能的治愈方法。
我们报告了 1 例 31 岁女性，因腹部肿块就诊，其甲胎蛋白（AFP）水平异常升高（31132ng/ml；正常值：0-7ng/ml）。增强 CT 显示肝脏两叶均有大肿瘤，动脉期增强，静脉期洗脱。氟-18 氟代脱氧葡萄糖（18F-FDG）正电子发射断层扫描（PET）/CT 显示肝肿瘤和左中腹腔内结节 18F-FDG 摄取增加（最大标准化摄取值，24.4）。
原发性肝 YST 伴大网膜转移。
患者在经肝动脉化疗栓塞（TACE）桥接后接受原位肝移植和腹腔内结节切除术。术中证实大网膜有腹腔内结节。肝肿瘤的组织病理学检查显示希勒-杜瓦尔小体。给予原肌球蛋白受体激酶（TRK）抑制剂拉罗替尼治疗，随后根据下一代测序结果进行 4 个周期博来霉素、依托泊苷和顺铂化疗。
AFP 水平降至正常范围。在 34 个月的随访期间，未观察到肿瘤塌陷的证据。
该病例提示以肝移植为主的多模式治疗，包括术前 TACE、术后辅助化疗和 TRK 抑制剂，是不可切除原发性肝 YST 的有效治疗方法。
