Luan Tianji, Mei Hongliang, Yuan Ling, Hu Yilin
Department of General Surgery, General Hospital of Central Theater Command, Wuhan, China.
Department of Pathology, General Hospital of Central Theater Command, Wuhan, China.
Transl Cancer Res. 2022 Dec;11(12):4465-4473. doi: 10.21037/tcr-22-2689.
Undifferentiated embryonic sarcoma of the liver (UESL) is a rare primary liver sarcoma that mainly affects children. Since it was first reported in 1978, less than 100 cases of UESL in adults have been reported. Due to the lack of specificity of clinical symptoms and imaging findings, the misdiagnosis rate of UESL is very high, and the definite diagnosis is usually in the late stage. Paraneoplastic syndrome (PNS) is a clinical symptom caused by the production of hormones or hormone-like substances in some tumors. This report describes the clinical diagnosis and treatment of patients with these 2 rare disorders. The imaging, histological, and genetic manifestations of UESL are described. This study provides support for the clinical research progress of UESL.
We report the case of a 57-year-old male with UESL and a progressive abdominal mass. Preoperative fine needle aspiration cytology (FNAC) of intrahepatic mass and postoperative routine pathology showed UESL. Ultrasound examination showed solid intrahepatic mass. Both computed tomography (CT) and magnetic resonance imaging (MRI) showed a large necrotic lesion in the right lobe of the liver. Laboratory examination showed leukocytosis [white blood cells (WBC): 10,005/µL]. The percentage of neutrophils increased (Ne%: 85%). Blood glucose was increased [glucose (Glu): 19.27 mmol/L]. Moderate anemia was observed [hemoglobin (Hb): 64 g/L]. Serum tumor markers (including AFP, PIVIKA, CA19-9, and CEA) were normal. Hepatitis virus markers [hepatitis B surface antigen (HBsAg) and antibodies to hepatitis C virus (anti‑HCV)] were negative. The patient presented with paraneoplastic symptoms such as fever, cough, night sweats, hyperinflammation, and hyperglycemia before surgery. After right hemihepatectomy, the paraneoplastic symptoms disappeared completely, and there was no recurrence in the follow-up for six months.
UESL is rare, especially in adults. The clinical manifestations of UESL are very similar to those of liver abscess and liver cystic tumor at the initial stage of the disease, for which differentiation is necessary. UESL should be considered when there is large cystic liver disease. FNAC is a good way to confirm the diagnosis. Complete resection of the tumor and postoperative chemotherapy is still the main treatment for UESL. The long-term effects of the therapeutic agents identified by genetic testing in this patient on the disease remain to be seen.
肝未分化胚胎性肉瘤（UESL）是一种罕见的原发性肝肉瘤，主要影响儿童。自1978年首次报道以来，成人UESL的报道病例不足100例。由于临床症状和影像学表现缺乏特异性，UESL的误诊率很高，确诊通常在疾病晚期。副肿瘤综合征（PNS）是某些肿瘤产生激素或激素样物质引起的临床症状。本报告描述了这两种罕见疾病患者的临床诊断和治疗。描述了UESL的影像学、组织学和遗传学表现。本研究为UESL的临床研究进展提供了支持。
我们报告了一例57岁男性患有UESL并伴有进行性腹部肿块的病例。肝内肿块术前细针穿刺细胞学检查（FNAC）及术后常规病理显示为UESL。超声检查显示肝内实性肿块。计算机断层扫描（CT）和磁共振成像（MRI）均显示肝右叶有一个大的坏死病灶。实验室检查显示白细胞增多[白细胞（WBC）：10,005/µL]。中性粒细胞百分比升高（中性粒细胞%：85%）。血糖升高[葡萄糖（Glu）：19.27 mmol/L]。观察到中度贫血[血红蛋白（Hb）：64 g/L]。血清肿瘤标志物（包括甲胎蛋白、异常凝血酶原、糖类抗原19-9和癌胚抗原）均正常。肝炎病毒标志物[乙肝表面抗原（HBsAg）和丙肝病毒抗体（抗-HCV）]均为阴性。该患者术前出现发热、咳嗽、盗汗、炎症反应和高血糖等副肿瘤症状。右半肝切除术后，副肿瘤症状完全消失，随访6个月无复发。
UESL罕见，尤其是在成人中。UESL在疾病初期的临床表现与肝脓肿和肝囊性肿瘤非常相似，因此需要进行鉴别。当存在巨大肝囊性疾病时应考虑UESL。FNAC是确诊的好方法。肿瘤完整切除及术后化疗仍是UESL的主要治疗方法。该患者通过基因检测确定的治疗药物对疾病的长期影响还有待观察。
