Introduction: Embryonal sarcoma of the liver (ESL) is a rare neoplasm of the liver occurring mainly in paediatric ages. Making the correct diagnosis can be challenging as the laboratory and radiological findings that are often nonspecific, and the tumour immunophenotype is poorly defined and even somewhat variable. Case Presentation: A large epigastric mass was detected in a computerized tomography scan of a 43-year-old woman presenting with abdominal pain and bloating. The mass was biopsied and submitted to histopathological study. Microscopically the tumour had sarcomatoid features and showed multinucleated cells with periodic acid-Schiff (PAS)-positive globules. Immunostaining revealed positivity for vimentin, CD10, glypican-3, and α1-antitrypsin and negativity for keratins, muscle, adipocytic, and melanocytic differentiation markers. The patient was then submitted to a left hepatectomy with similar histological findings. Discussion: ESL in adults is a rarity and its diagnosis requires the exclusion of other entities. While some microscopic features are very common, they remain nonspecific. The main feature is the presence of multinucleated cells with PAS-positive hyaline globules. While ancillary testing is key, the immunophenotype also lacks specificity and ESL may have variable staining for glypican-3 and epithelial or muscle differentiation markers. Although it has been described for more than 3 decades, the prognosis and optimal treatment are still not well defined, but surgery has yielded favourable results.
GE Port J Gastroenterol

中文翻译：

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成人肝脏胚胎肉瘤：罕见实体的诊断挑战

简介：肝脏胚胎肉瘤 (ESL) 是一种罕见的肝脏肿瘤，主要发生在儿科年龄。做出正确的诊断可能具有挑战性，因为实验室和放射学检查结果通常是非特异性的，并且肿瘤免疫表型定义不明确甚至有些可变。案例介绍：一名 43 岁的女性因腹痛和腹胀而接受计算机断层扫描，发现上腹部有一个大肿块。对肿块进行活组织检查并进行组织病理学研究。在显微镜下，肿瘤具有肉瘤样特征，并显示具有高碘酸席夫 (PAS) 阳性小球的多核细胞。免疫染色显示波形蛋白、CD10、glypican-3 和 α1-抗胰蛋白酶呈阳性，而角蛋白、肌肉、脂肪细胞和黑素细胞分化标志物呈阴性。然后患者接受了左肝切除术，组织学结果相似。讨论：成人 ESL 很少见，其诊断需要排除其他疾病。虽然一些微观特征非常普遍，但它们仍然是非特异性的。主要特征是存在具有 PAS 阳性透明小球的多核细胞。虽然辅助测试是关键，但免疫表型也缺乏特异性，ESL 可能对磷脂酰肌醇蛋白聚糖 3 和上皮或肌肉分化标记物进行可变染色。虽然已经描述了 3 多年，但预后和最佳治疗仍未明确，但手术取得了良好的效果。
GE Port J 胃肠醇