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两例成人肝母细胞瘤

Two Cases of Hepatoblastoma in Adults.

作者信息

Ihssan Elouarith, Hajar Elagouri, Salma Bekarsabein, Soumaya Ech-Charif, Youssef Mahdi, Mouna Khmou, Basma El Khannoussi

机构信息

Pathology Department, Oncology National Institute, Faculty of Medicine and Pharmacy, Mohammed V University, Rabat, Morocco.

出版信息

Clin Pathol. 2022 Oct 26;15:2632010X221129592. doi: 10.1177/2632010X221129592. eCollection 2022 Jan-Dec.

Abstract

Adult hepatoblastoma is a rare tumor whose etiology and mechanisms of development are still incompletely understood. Imaging and biological tests such as AFP and liver enzymes are non-specific. Histologically, there are 2 histological variants: pure epithelial with 5 types (pure fetal, embryonal, small cell undifferentiated, cholangioblastic, and macrotrabecular), a mixed epithelial and a mesenchymal variant with or without a teratoid contingent. The main differential diagnosis concerns hepatocellular carcinoma. The treatment of hepatoblastoma in adults is not yet standardized and surgery remains the mainstay of treatment. In this report we aim to describe the clinical, pathological, and immunohistochemical features of this rare entity in adult patients and discuss the elements allowing its distinction from hepatocellular carcinoma (HCC).

摘要

成人肝母细胞瘤是一种罕见肿瘤,其病因及发生机制仍未完全明确。影像学检查及甲胎蛋白(AFP)和肝酶等生物学检测均不具有特异性。组织学上,有两种组织学亚型:5种类型的纯上皮型(纯胎儿型、胚胎型、小细胞未分化型、胆管母细胞型和大小梁型)、上皮和间叶混合型,后者可有或无畸胎样成分。主要鉴别诊断涉及肝细胞癌。成人肝母细胞瘤的治疗尚未标准化,手术仍是主要治疗手段。在本报告中,我们旨在描述成年患者中这种罕见疾病的临床、病理和免疫组化特征,并讨论将其与肝细胞癌(HCC)区分开来的因素。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/abe1/9615437/9cffea003c02/10.1177_2632010X221129592-fig1.jpg

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