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卵巢肝样癌:临床、组织病理学和免疫表型特征。

Hepatoid Carcinoma of the Ovary: Clinical, Histopathologic, and Immunophenotypic Features.

机构信息

From the University of Illinois at Chicago (Dr Acosta); the Chicago Medical School of Rosalind Franklin University of Medicine and Science, North Chicago, Illinois (Dr Pins); and the Advocate Lutheran General Hospital and the James R. & Helen D. Russell Institute for Research & Innovation, Park Ridge, Illinois (Dr Pins).

出版信息

Arch Pathol Lab Med. 2019 Jul;143(7):883-889. doi: 10.5858/arpa.2017-0485-RS. Epub 2019 Jan 10.

DOI:10.5858/arpa.2017-0485-RS
PMID:30628840
Abstract

Hepatoid carcinoma of the ovary (HCO) is a rare malignant tumor of uncertain histogenesis that was first described by Ishikura and Scully in the late 1980s. Unlike hepatoid yolk sac tumor (HYST), one of its main differential diagnoses, HCO usually presents in perimenopausal and postmenopausal women without gonadal dysgenesis. Most cases show advanced local disease at initial presentation, with diffuse intraperitoneal dissemination. Despite aggressive treatment, including surgery and adjuvant chemotherapy, 61.5% of patients either die of the disease (11 of 26; 42.3%) or are alive with recurrent or residual disease (5 of 26; 19.2%) after a median follow-up of 11.5 months (range, 1-60 months). Most HCOs are solid, with high-grade histology, significant nuclear pleomorphism, scattered giant cells, and a high mitotic index. Their immunophenotype is defined by the expression of broad-spectrum cytokeratins, α-fetoprotein, and hepatocellular antigens with absence of sex cord and germ cell markers. Although immunohistochemistry can be very helpful to distinguish between sex cord-stromal tumors and HCO, differentiation of the latter from HYST, metastatic hepatocellular carcinoma, and metastatic gastrointestinal tumors with hepatoid phenotype requires integration of clinical, radiologic, and pathologic information.

摘要

卵巢肝样细胞癌(HCO)是一种罕见的恶性肿瘤,其组织发生不确定,于 20 世纪 80 年代末由 Ishikura 和 Scully 首次描述。与它的主要鉴别诊断之一肝样卵黄囊瘤(HYST)不同,HCO 通常发生在围绝经期和绝经后妇女中,无性腺发育不全。大多数病例在初始表现时表现为晚期局部疾病,弥漫性腹膜内播散。尽管采用了包括手术和辅助化疗在内的积极治疗,61.5%的患者在中位随访 11.5 个月(范围为 1-60 个月)后死于该疾病(26 例中的 11 例;42.3%)或患有复发或残留疾病(26 例中的 5 例;19.2%)。大多数 HCO 为实性,组织学分级高,核异型性明显,散在分布的巨细胞和高有丝分裂指数。它们的免疫表型由广谱细胞角蛋白、α-胎蛋白和肝细胞抗原的表达定义,缺乏性索和生殖细胞标志物。尽管免疫组织化学对于区分性索-间质肿瘤和 HCO 非常有帮助,但将后者与 HYST、转移性肝细胞癌和具有肝样表型的转移性胃肠道肿瘤区分开来需要整合临床、影像学和病理学信息。

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