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一名年轻女性的非典型上皮样施万细胞瘤——形态学引发的难题;免疫组织化学解决——一例罕见病例报告

Atypical Epithelioid Schwannoma in a Young Female- A Dilemma Set by Morphology; Resolved by Immunohistochemistry-A Rare Case Report.

作者信息

Amita K, Rajani T

机构信息

From the Department of Pathology, Adichunchanagiri Institute of Medical Sciences, Adichunchanagiri University, BG Nagara, Nagamangala Taluk, Mandya, Karnataka, India.

出版信息

Indian J Dermatol. 2025 Mar-Apr;70(2):97-101. doi: 10.4103/ijd.ijd_584_23. Epub 2025 Feb 27.

DOI:10.4103/ijd.ijd_584_23
PMID:40162348
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11952709/
Abstract

Epithelioid morphology spawns myriad of benign and malignant tumors into differential diagnoses, which include epithelioid sarcoma, malignant melanoma, epithelioid malignant peripheral nerves sheath tumor, epithelioid hemangioendothelioma, myoepithelioma, etc. Epithelioid schwannoma (ES), especially the atypical variant has not received much attention in literature. Conclusive diagnosis, important for accurate management is, however, challenging. We describe a case of atypical ES in a 24-year-old female. On clinical and radiological examination, a provisional diagnosis of vascular malformation was made. Atypical epithelioid ESs are rare, but it is important to be aware of this variant to avoid misdiagnosis with other malignant epithelioid tumors. Apart from clinical and morphological features, immunohistochemistry aids in arriving at a decisive diagnosis. Perineurium-like capsule, circumscription, oblong shape of the lesion, and hyalinized blood vessels are pointers toward shwannomatous differentiation of this tumor.

摘要

上皮样形态会引发多种良恶性肿瘤的鉴别诊断,其中包括上皮样肉瘤、恶性黑色素瘤、上皮样恶性外周神经鞘瘤、上皮样血管内皮瘤、肌上皮瘤等。上皮样神经鞘瘤(ES),尤其是非典型变体,在文献中并未受到太多关注。然而,对于准确治疗至关重要的确诊却具有挑战性。我们描述了一例24岁女性的非典型ES病例。经临床和影像学检查,初步诊断为血管畸形。非典型上皮样ES很罕见,但了解这种变体以避免与其他恶性上皮样肿瘤误诊很重要。除了临床和形态学特征外,免疫组织化学有助于做出决定性诊断。类神经束膜样包膜、边界清晰、病变呈椭圆形以及血管玻璃样变是该肿瘤呈神经鞘瘤样分化的指标。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6421/11952709/8f7712307b50/IJD-70-97-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6421/11952709/58651b8ac88d/IJD-70-97-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6421/11952709/5f33a77c9125/IJD-70-97-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6421/11952709/8f7712307b50/IJD-70-97-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6421/11952709/58651b8ac88d/IJD-70-97-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6421/11952709/5f33a77c9125/IJD-70-97-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6421/11952709/8f7712307b50/IJD-70-97-g003.jpg

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本文引用的文献

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Myoepithelial carcinoma or epithelioid sarcoma - A rare diagnosis with poor prognosis. A case report and review of literature.肌上皮癌或上皮样肉瘤——一种罕见的诊断,预后不良。病例报告及文献综述。
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SMARCB1/INI1 Loss in Epithelioid Schwannoma: A Clinicopathologic and Immunohistochemical Study of 65 Cases.上皮样神经鞘瘤中SMARCB1/INI1缺失:65例临床病理及免疫组化研究
Am J Surg Pathol. 2017 Aug;41(8):1013-1022. doi: 10.1097/PAS.0000000000000849.
3
Epithelioid schwannoma of the skin displaying unique histopathological features: a teaching case giving rise to diagnostic difficulties on a morphological examination of a resected specimen, with a brief literature review.
皮肤上皮样施万细胞瘤表现出独特的组织病理学特征:一个教学案例,在对切除标本进行形态学检查时引发诊断困难,并附简要文献综述
Diagn Pathol. 2017 Jan 19;12(1):11. doi: 10.1186/s13000-017-0604-9.
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Epithelioid Schwannomas: An Analysis of 58 Cases Including Atypical Variants.上皮样神经鞘瘤:58例分析,包括非典型变体
Am J Surg Pathol. 2016 May;40(5):704-13. doi: 10.1097/PAS.0000000000000589.
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Epithelioid Schwannoma of a Spinal Nerve Root.脊神经根上皮样施万细胞瘤
Can J Neurol Sci. 2016 May;43(3):430-3. doi: 10.1017/cjn.2015.340. Epub 2016 Jan 7.
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Epithelioid hemangioendothelioma: an overview and update on a rare vascular tumor.上皮样血管内皮瘤:一种罕见血管肿瘤的概述与更新
Oncol Rev. 2014 Oct 13;8(2):259. doi: 10.4081/oncol.2014.259. eCollection 2014 Sep 23.
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Cutaneous benign epithelioid peripheral nerve sheath tumour: a rare entity.皮肤良性上皮样外周神经鞘瘤:一种罕见的实体。
Indian J Dermatol. 2013 Sep;58(5):409. doi: 10.4103/0019-5154.117354.
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Epithelioid sarcoma: a clinicopathologic and immunohistochemical analysis of 106 cases from the French sarcoma group.上皮样肉瘤:来自法国肉瘤研究组的106例临床病理及免疫组化分析
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Mucosal benign epithelioid nerve sheath tumors.黏膜良性上皮样神经鞘瘤。
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