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子宫原发性上皮样血管肉瘤:一种具有高度侵袭性的罕见肿瘤。

Primary Epithelioid Angiosarcoma of the Uterus: A Rare Tumor with Very Aggressive Behavior.

作者信息

Majeed Nasma K, Adley Brian, Guzman Grace, Mehta Vikas

机构信息

Department of Pathology, University of Illinois at Chicago, Illinois, USA.

Department of Pathology, Advocate Lutheran General Hospital, Illinois, USA.

出版信息

Case Rep Pathol. 2020 Feb 22;2020:5461782. doi: 10.1155/2020/5461782. eCollection 2020.

DOI:10.1155/2020/5461782
PMID:32158582
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7060455/
Abstract

Angiosarcoma is a high-grade vascular tumor arising from endothelial cells of blood vessels. It represents less than 1% of the mesenchymal tumors. Uterine angiosarcoma is an extremely rare tumor with less than 25 cases reported in the literature. It usually presents in postmenopausal women as uterine mass and rarely can arise in a leiomyoma. It is included in the group of tumors of aggressive behavior and poor prognosis. Herein, we present a case of primary uterine angiosarcoma in a 56-year-old female patient with a history of fibroids presenting with pelvic pain and weight loss. Abdominal CT scan showed a large uterine mass and enlarged pelvic lymph nodes. Total abdominal hysterectomy with bilateral salpingo-oophorectomy was performed, and on histopathologic examination, it was found to be primary epithelioid angiosarcoma of the uterus. Immunohistochemical stains for CD31, keratin MAK-6, and keratin AE1/AE3 confirmed the diagnosis. In conclusion, uterine angiosarcoma should be suspected in any rapidly growing hemorrhagic uterine mass, and appropriate sampling and immunohistochemical stains should be considered.

摘要

血管肉瘤是一种起源于血管内皮细胞的高级别血管肿瘤。它占间叶性肿瘤的比例不到1%。子宫血管肉瘤是一种极其罕见的肿瘤,文献报道不足25例。它通常在绝经后女性中表现为子宫肿块,很少可起源于平滑肌瘤。它属于具有侵袭性生物学行为和预后不良的肿瘤类别。在此,我们报告一例56岁女性原发性子宫血管肉瘤病例,该患者有肌瘤病史,表现为盆腔疼痛和体重减轻。腹部CT扫描显示子宫有一个大肿块以及盆腔淋巴结肿大。实施了全腹子宫切除术及双侧输卵管卵巢切除术,经组织病理学检查,发现为原发性子宫上皮样血管肉瘤。CD31、角蛋白MAK-6和角蛋白AE1/AE3的免疫组化染色证实了诊断。总之,对于任何快速生长的出血性子宫肿块都应怀疑子宫血管肉瘤,并应考虑进行适当的取材及免疫组化染色。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8513/7060455/479d21ced091/CRIPA2020-5461782.003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8513/7060455/3e0994ee7e61/CRIPA2020-5461782.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8513/7060455/91459da3681e/CRIPA2020-5461782.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8513/7060455/479d21ced091/CRIPA2020-5461782.003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8513/7060455/3e0994ee7e61/CRIPA2020-5461782.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8513/7060455/91459da3681e/CRIPA2020-5461782.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8513/7060455/479d21ced091/CRIPA2020-5461782.003.jpg

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本文引用的文献

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Epithelioid Angiosarcoma Arising from a Huge Leiomyoma: A Case Report and a Literature Review.源于巨大平滑肌瘤的上皮样血管肉瘤:一例报告及文献复习
Case Rep Obstet Gynecol. 2018 Jun 4;2018:7591769. doi: 10.1155/2018/7591769. eCollection 2018.
2
Uterine Angiosarcoma: A Case Report and Literature Review.子宫血管肉瘤:一例报告及文献综述
Int J Gynecol Pathol. 2016 May;35(3):264-8. doi: 10.1097/PGP.0000000000000219.
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Ovarian angiosarcoma: a case report and review of the literature.卵巢血管肉瘤:一例病例报告及文献综述
J Med Case Rep. 2014 Feb 12;8:47. doi: 10.1186/1752-1947-8-47.
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Malignant vascular tumors--an update.恶性血管肿瘤——最新进展
Mod Pathol. 2014 Jan;27 Suppl 1:S30-8. doi: 10.1038/modpathol.2013.176.
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Angiosarcomas of primary gynecologic origin: a clinicopathologic review and quantitative analysis of survival.原发性妇科来源的血管肉瘤:临床病理回顾和生存的定量分析。
Int J Gynecol Cancer. 2014 Jan;24(1):4-12. doi: 10.1097/IGC.0000000000000020.
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Breakages at YWHAE, FAM22A, and FAM22B loci in uterine angiosarcoma: a case report with immunohistochemical and genetic analysis.子宫血管肉瘤中YWHAE、FAM22A和FAM22B基因座的断裂:一例免疫组织化学和基因分析病例报告
Pathol Res Pract. 2014 Feb;210(2):130-4. doi: 10.1016/j.prp.2013.09.009. Epub 2013 Sep 26.
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Vaginal epithelioid angiosarcoma: a potential pitfall in gynecologic pathology.阴道上皮样血管肉瘤:妇科病理学中的一个潜在陷阱。
J Low Genit Tract Dis. 2014 Apr;18(2):E38-42. doi: 10.1097/LGT.0b013e31829863b3.
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Distinct histological features characterize primary angiosarcoma of bone.原发性骨血管肉瘤具有独特的组织学特征。
Histopathology. 2011 Jan;58(2):254-64. doi: 10.1111/j.1365-2559.2011.03750.x.
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Ann Diagn Pathol. 2008 Jun;12(3):217-21. doi: 10.1016/j.anndiagpath.2007.08.001. Epub 2008 Jan 11.