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具有不寻常基因融合的低级别纤维黏液样肉瘤:1例发生于腮腺的罕见病例报告

Low-Grade Fibromyxoid Sarcoma Featuring an Unusual Gene Fusion: Report of a Rare Case Arising in the Parotid Gland.

作者信息

Alayed Doaa M, Pharaon Majed M

机构信息

Department of Pathology and Laboratory Medicine, King Khalid University Hospital, Riyadh, Saudi Arabia.

Department of Pathology and Laboratory Medicine, Ministry of National Guard, Riyadh, Saudi Arabia.

出版信息

Avicenna J Med. 2022 Jun 21;12(2):87-92. doi: 10.1055/s-0042-1749611. eCollection 2022 Apr.

DOI:10.1055/s-0042-1749611
PMID:36051504
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9425117/
Abstract

Low-grade fibromyxoid sarcoma (LGFMS), also known as Evan's tumor, is a fibroblastic tumor with a deceptively bland morphology but a high metastasizing potential and late recurrence. It presents clinically as a slowly growing mass in the deep soft tissue of extremities and trunk. Morphologically, it shows a biphasic pattern with alternating fibrous and myxoid areas, whorling growth pattern, hypocellularity, and bland fibroblasts with curvilinear or arcuate vessels in between. Collagen rosettes with central hyalinization is a helpful feature but not specific. MUC-4 is the most sensitive immunostain in LGFMS. The majority of cases harbor a gene fusion in - Herein, we present a 23-year-old female patient with a slow growing painless mass in the left parotid gland, which was later diagnosed as LGFMS. Involvement of the head and neck region by LGFMS is uncommonly encountered. To date, only two cases within the parotid gland have been described.

摘要

低度纤维黏液样肉瘤(LGFMS),也称为埃文斯瘤,是一种成纤维细胞性肿瘤,形态看似温和,但具有高转移潜能和晚期复发的特点。临床上,它表现为四肢和躯干深部软组织中缓慢生长的肿块。形态学上,它呈现双相模式,有交替的纤维和黏液样区域、漩涡状生长模式、细胞稀少,以及其间有曲线或弧形血管的温和的成纤维细胞。中央玻璃样变的胶原玫瑰花结是一个有用的特征,但不具有特异性。MUC-4是LGFMS中最敏感的免疫染色。大多数病例存在基因融合——在此,我们报告一名23岁女性患者,其左侧腮腺有一个缓慢生长的无痛性肿块,后来被诊断为LGFMS。LGFMS累及头颈部区域的情况并不常见。迄今为止,仅描述过两例发生于腮腺内的病例。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6039/9425117/82a0100f8f3e/10-1055-s-0042-1749611-i210136-4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6039/9425117/0d6277c0d5c6/10-1055-s-0042-1749611-i210136-1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6039/9425117/eb7acbd97f71/10-1055-s-0042-1749611-i210136-2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6039/9425117/05f4b1ad95d7/10-1055-s-0042-1749611-i210136-3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6039/9425117/82a0100f8f3e/10-1055-s-0042-1749611-i210136-4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6039/9425117/0d6277c0d5c6/10-1055-s-0042-1749611-i210136-1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6039/9425117/eb7acbd97f71/10-1055-s-0042-1749611-i210136-2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6039/9425117/05f4b1ad95d7/10-1055-s-0042-1749611-i210136-3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6039/9425117/82a0100f8f3e/10-1055-s-0042-1749611-i210136-4.jpg

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本文引用的文献

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Primary orbital low-grade fibromyxoid sarcoma - A case report.原发性眼眶低度纤维黏液样肉瘤-病例报告。
Indian J Ophthalmol. 2019 Apr;67(4):568-570. doi: 10.4103/ijo.IJO_633_18.
2
Low-grade fibromyxoid sarcoma: Clinical, morphologic and genetic features.低度恶性纤维黏液样肉瘤:临床、形态学及遗传学特征
Ann Diagn Pathol. 2017 Jun;28:60-67. doi: 10.1016/j.anndiagpath.2017.04.001. Epub 2017 Apr 5.
3
Low-grade fibromyxoid sarcoma involving the mandible: A diagnostic dilemma.累及下颌骨的低度恶性纤维黏液样肉瘤:诊断难题。
J Oral Maxillofac Pathol. 2016 May-Aug;20(2):334. doi: 10.4103/0973-029X.185914.
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A low grade fibromyxoid sarcoma originating from the masseter muscle: a case report.一例起源于咬肌的低级别纤维黏液样肉瘤病例报告。
J Med Case Rep. 2015 Aug 21;9:176. doi: 10.1186/s13256-015-0658-9.
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Low-Grade Fibromyxoid Sarcoma of the Head and Neck: A Clinicopathologic Series and Review of the Literature.头颈部低度恶性纤维黏液样肉瘤:一项临床病理系列研究及文献综述
Head Neck Pathol. 2016 Jun;10(2):161-6. doi: 10.1007/s12105-015-0647-8. Epub 2015 Aug 15.
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A genetic dichotomy between pure sclerosing epithelioid fibrosarcoma (SEF) and hybrid SEF/low-grade fibromyxoid sarcoma: a pathologic and molecular study of 18 cases.纯硬化性上皮样纤维肉瘤(SEF)与混合性SEF/低度纤维黏液样肉瘤之间的遗传学二分法:18例病例的病理学和分子研究
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