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[原发性子宫肝样腺癌:2例临床病理分析及文献复习]

[Primary uterine hepatoid adenocarcinoma: Clinicopathological analysis of 2 cases and literature review].

作者信息

Luo Dan, Huang Haijian, Chen Xin, Chen Xiaoyan

机构信息

Department of Pathology, Fujian Maternity and Child Health Hospital, College of Clinical Medicine for Obstetrics & Gynecology and Pediatrics, Fujian Medical University, Fuzhou 350005, China.

Department of Pathology, Fujian Provincial Hospital, Provincial Clinical Medical College of Fujian Medical University, Fuzhou 350001, China.

出版信息

Beijing Da Xue Xue Bao Yi Xue Ban. 2024 Dec 18;56(6):1126-1131. doi: 10.19723/j.issn.1671-167X.2024.06.030.

Abstract

Primary hepatoid adenocarcinoma (HAC) of the uterus is a particular tumour that bears high similarity to hepatocellular carcinoma histologically, and may easily be misdiagnosed because it is rare if you don' t remember it. In this report, we describe two cases of alpha-fetoprotein (AFP)-producing HAC of the uterus. Case 1 was a 69-year-old postmenopausal woman who was presented to the hospital for a medical examination. Positron emission computed tomography and gross examination revealed an invasive mass on the cervix. Microscopically, the tumor cells grew in trabecularand and solid patterns with heteromorphic nuclei and abundant eosinophilic cytoplasm, and were stained positively for AFP, spalt-like transcription factor 4 (SALL-4), cytokeratin 7 (CK7), hepatocyte paraffin 1 (Hep Par 1), Glypican 3 and p16. The paired box protein 8 (PAX8), Vimentin, CK20, estrogen receptor (ER), progesterone receptor (PR) were negative. P53 protein was strongly diffuse staining, suggesting the possibility of potential mutation in the 53 gene. The final pathological diagnosis was cervical HAC combined with endocervical adenocarcinoma and endocervical adenocarcinoma . To the best of our knowledge, however, it is the third case confined to the uterine cervix reported in Chinese and English literature. Case 2 was a 57-year-old postmenopausal woman with abnormal vaginal bleeding for 4 months. Biopsy was considered as poorly differentiated endometrial carcinoma. Finally, pure HAC in endometrium was diagnosed in postoperative specimens. The histological features and immunohistochemical results were similar to those in case 1. A total abdominal hysterectomy, bilateral salpingo-oophorectomy, pelvic lymphadenectomy and pelvic adhesiolysis were carried out in both cases. Serum levels of AFP were increased remarkably in both cases pre-operation and decreased after surgery, which was proved to be closely related to tumor progression, recurrence, and also the patient' s response to treatment. The diagnosis of HAC is mainly based on the histological features, and immunohistochemistry is a good assistant, but it needs to be differentiated from metastatic hepatocellular carcinoma (HCC), germ cell tumors, and yolk sac tumor. Following surgery, both patients received chemotherapy, and case 1 also received radiotherapy, and has been free of disease for 25 months and 5 months, respectively.

摘要

原发性子宫肝样腺癌(HAC)是一种特殊的肿瘤,在组织学上与肝细胞癌高度相似,由于其罕见,若不加以留意很容易被误诊。在本报告中,我们描述了两例产生甲胎蛋白(AFP)的子宫HAC病例。病例1是一名69岁的绝经后女性,因体检入院。正电子发射计算机断层扫描及大体检查显示宫颈有一浸润性肿块。显微镜下,肿瘤细胞呈小梁状和实性生长,细胞核异型,胞质丰富嗜酸性,AFP、锌指样转录因子4(SALL-4)、细胞角蛋白7(CK7)、肝细胞石蜡1(Hep Par 1)、磷脂酰肌醇蛋白聚糖3和p16染色呈阳性。配对盒蛋白8(PAX8)、波形蛋白、CK20、雌激素受体(ER)、孕激素受体(PR)均为阴性。P53蛋白呈强弥漫性染色,提示53基因可能存在潜在突变。最终病理诊断为宫颈HAC合并宫颈管腺癌和宫颈管腺癌。然而,据我们所知,这是中英文文献中报道的第三例局限于宫颈的病例。病例2是一名57岁的绝经后女性,有4个月的异常阴道出血。活检被认为是低分化子宫内膜癌。最终术后标本诊断为子宫内膜单纯性HAC。组织学特征和免疫组化结果与病例1相似。两例均行全腹子宫切除术、双侧输卵管卵巢切除术、盆腔淋巴结清扫术及盆腔粘连松解术。两例患者术前血清AFP水平均显著升高,术后降低,这被证明与肿瘤进展、复发以及患者对治疗的反应密切相关。HAC的诊断主要基于组织学特征,免疫组化是很好的辅助手段,但需要与转移性肝细胞癌(HCC)、生殖细胞肿瘤和卵黄囊瘤相鉴别。术后,两名患者均接受了化疗,病例1还接受了放疗,分别已无病生存25个月和5个月。

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本文引用的文献

1
Hepatoid Adenocarcinoma of the Stomach: Current Perspectives and New Developments.胃肝样腺癌:当前观点与新进展
Front Oncol. 2021 Apr 12;11:633916. doi: 10.3389/fonc.2021.633916. eCollection 2021.
2
Hepatoid Adenocarcinoma Presenting as Pancreatitis.表现为胰腺炎的肝样腺癌
ACG Case Rep J. 2020 May 7;7(5):e00381. doi: 10.14309/crj.0000000000000381. eCollection 2020 May.
4
Hepatoid adenocarcinoma of the lung and the review of the literature.肺肝样腺癌及文献综述
J Oncol Pharm Pract. 2020 Sep;26(6):1505-1510. doi: 10.1177/1078155220903360. Epub 2020 Feb 10.
5
Therapeutic Approaches to Gastric Hepatoid Adenocarcinoma: Current Perspectives.胃肝样腺癌的治疗方法:当前观点
Ther Clin Risk Manag. 2019 Dec 23;15:1469-1477. doi: 10.2147/TCRM.S204303. eCollection 2019.

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