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起源于神经鞘瘤的上皮样恶性外周神经鞘膜瘤。

Epithelioid malignant peripheral nerve sheath tumor arising in schwannoma.

作者信息

Heatley Nicholas, Kolson Kokohaare Eva, Strauss Dirk C, Hallin Magnus, Jones Robin L, Fisher Cyril, Thway Khin

机构信息

Sarcoma Unit, The Royal Marsden Hospital, London, UK.

The Institute of Cancer Research, London, UK.

出版信息

Rare Tumors. 2020 Aug 21;12:2036361320950862. doi: 10.1177/2036361320950862. eCollection 2020.

DOI:10.1177/2036361320950862
PMID:32913618
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7443986/
Abstract

Epithelioid malignant peripheral nerve sheath tumor (EMPNST, malignant epithelioid schwannoma) is a rare variant of malignant peripheral nerve sheath tumor that has morphologic and immunophenotypic overlap with a variety of epithelioid neoplasms. Because of its rarity it may be potentially underrecognized. We describe a case arising in the subcutis of the thigh in a 25 year-old female, and discuss the pathologic features and differential diagnosis.

摘要

上皮样恶性外周神经鞘瘤（EMPNST，恶性上皮样施万细胞瘤）是恶性外周神经鞘瘤的一种罕见变体，在形态学和免疫表型上与多种上皮样肿瘤存在重叠。由于其罕见性，它可能潜在地未被充分认识。我们描述了一例发生在一名25岁女性大腿皮下的病例，并讨论其病理特征和鉴别诊断。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/959e/7443986/50b04ccb1f8b/10.1177_2036361320950862-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/959e/7443986/50b04ccb1f8b/10.1177_2036361320950862-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/959e/7443986/50b04ccb1f8b/10.1177_2036361320950862-fig1.jpg

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Mod Pathol. 2016 Jun;29(6):582-90. doi: 10.1038/modpathol.2016.45. Epub 2016 Mar 18.
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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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Loss of H3K27me3 Expression Is a Highly Sensitive Marker for Sporadic and Radiation-induced MPNST.
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Front Neurol. 2023 May 25;14:1178651. doi: 10.3389/fneur.2023.1178651. eCollection 2023.
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H3K27me3表达缺失是散发性和辐射诱导性恶性外周神经鞘膜瘤的高度敏感标志物。
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4
Epithelioid Sarcoma: Diagnostic Features and Genetics.上皮样肉瘤：诊断特征与遗传学
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