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成人肝母细胞瘤:与肝细胞癌进行具有挑战性的鉴别诊断

Adult hepatoblastoma: making the challenging distinction from hepatocellular carcinoma.

作者信息

Pagarigan Allison Kaye L, Mendoza Paulo Giovanni L

机构信息

Department of Pathology and Laboratory Medicine, National Kidney and Transplant Institute, Quezon City, Philippines.

出版信息

J Liver Cancer. 2023 Mar;23(1):219-224. doi: 10.17998/jlc.2023.02.24. Epub 2023 Mar 13.

Abstract

Hepatoblastoma is an exceptionally rare malignancy in adults with just over 70 non-pediatric cases reported in literature. Recounted is a case of a 49-year-old female who presented with acute right upper quadrant abdominal pain, elevated serum alpha fetoprotein and a large liver mass on imaging. Hepatectomy was performed under clinical suspicion of hepatocellular carcinoma. Immunomorphologic characteristics of the tumor proved consistent with hepatoblastoma of mixed epithelial and mesenchymal type. Hepatocellular carcinoma remains to be the primary differential diagnosis for adult hepatoblastoma, however, distinguishing between these two neoplasms requires close histomorphologic assessment and immunohistochemical profiling as clinical, radiologic and gross pathologic findings typically overlap. Making this distinction is highly crucial in the timely initiation of surgical and chemotherapeutic interventions for this inherently aggressive and rapidly fatal disease.

摘要

肝母细胞瘤在成人中极为罕见,文献报道的非儿童病例仅有70多例。本文讲述了一例49岁女性患者,她因右上腹急性腹痛就诊,血清甲胎蛋白升高,影像学检查发现肝脏有巨大肿块。临床怀疑为肝细胞癌而行肝切除术。肿瘤的免疫形态学特征证实为混合上皮和间充质型肝母细胞瘤。肝细胞癌仍是成人肝母细胞瘤的主要鉴别诊断对象,然而,区分这两种肿瘤需要密切的组织形态学评估和免疫组化分析,因为临床、放射学和大体病理学表现通常相互重叠。对于这种具有侵袭性且迅速致命的疾病,及时进行手术和化疗干预,准确区分这两种肿瘤至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1346/10202245/0282d2956dc6/jlc-2023-02-24f1.jpg

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