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原发子宫肝样腺癌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
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摘要 肝样腺癌(hepatoid adenocarcinoma,HAC)是一种非常罕见的发生于肝外的特殊类型腺癌,因其形态学特征和免疫表型与肝细胞癌相似而得名。发生于子宫的HAC极为少见,检索国内外文献,目前原发于宫颈的HAC仅见2例报道^([1-2]),原发于子宫内膜的HAC共16例报道^([3-17])。本文收集2例原发性子宫HAC病例,并对以往文献进行了复习,探讨其临床病理特征,以期提高对子宫HAC的认识,为该病的治疗及预后提供依据。 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 TP53 gene.The final pathological diagnosis was cervical HAC combined with endocervical adenocarcinoma and endocervical adenocarcinoma in situ.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.
作者 罗丹 黄海建 陈新 陈小岩 LUO Dan;HUANG Haijian;CHEN Xin;CHEN Xiaoyan(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)
出处 《北京大学学报(医学版)》 CAS CSCD 北大核心 2024年第6期1126-1131,共6页 Journal of Peking University:Health Sciences
关键词 肝样腺癌 子宫 子宫颈 临床病理特征 Hepatoid adenocarcinoma Uterus Cervix Clinicopathological feature
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