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肺泡型孤立性纤维瘤:一种罕见的形态学类型。

Alveolar solitary fibrous tumor: an uncommon morphological form.

作者信息

Song Lin, Lin Dong-Liang, Zhang Zhao-Fen, Wang Zhou, Zong Yuan-Yuan

机构信息

Department of Pathology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, No. 324 JingWu Road, Jinan, 250021, Shandong, China.

Department of Pathology, The Affiliated Hospital of Qingdao University, Qingdao, 266003, China.

出版信息

Diagn Pathol. 2025 Aug 26;20(1):98. doi: 10.1186/s13000-025-01698-z.

DOI:10.1186/s13000-025-01698-z
PMID:40859272
Abstract

Solitary fibrous tumor (SFT) is a fibroblastic tumor characterized by a prominent staghorn vasculature and collagen deposition. However, little is known about SFTs with alveolar structures. Herein, we present a case of an alveolar pattern SFT in a 55-year-old woman. The tumor was present in the lumbosacral spinal canal and showed an alveolar architecture composed of ovoid to spindle-shaped cells. Immunohistochemical examination showed that the tumor cells were positive for STAT6 (nuclear expression), CD34, CD99, and Bcl-2, but negative for cytokeratins (CK-pan and AE1/AE3), EMA, GFAP, CD31, progesterone receptor, S-100 protein, and smooth muscle actin. Furthermore, NAB2::STAT6 fusion was detected using DNA-based next-generation sequencing, which established the diagnosis of SFT at a molecular level. The present case expands the morphological categories of SFT.

摘要

孤立性纤维瘤(SFT)是一种以显著的鹿角状血管和胶原沉积为特征的纤维母细胞性肿瘤。然而,对于具有肺泡结构的SFT了解甚少。在此,我们报告一例55岁女性的肺泡型SFT病例。肿瘤位于腰骶部椎管,呈现出由卵圆形至梭形细胞组成的肺泡结构。免疫组织化学检查显示肿瘤细胞STAT6(核表达)、CD34、CD99和Bcl-2呈阳性,但细胞角蛋白(泛细胞角蛋白和AE1/AE3)、EMA、GFAP、CD31、孕激素受体、S-100蛋白和平滑肌肌动蛋白呈阴性。此外,通过基于DNA的二代测序检测到NAB2::STAT6融合,在分子水平上确立了SFT的诊断。本病例扩展了SFT的形态学范畴。

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Alveolar solitary fibrous tumor: an uncommon morphological form.肺泡型孤立性纤维瘤:一种罕见的形态学类型。
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本文引用的文献

1
Ependymoma: Evaluation and Management Updates.室管膜瘤:评估与管理的最新进展。
Curr Oncol Rep. 2022 Aug;24(8):985-993. doi: 10.1007/s11912-022-01260-w. Epub 2022 Apr 6.
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Primary endodermal hemangiopericytoma/solitary fibrous tumor of the cervical spine: a case report and literature review.颈椎原发性内胚层窦血管外皮细胞瘤/孤立性纤维瘤:病例报告及文献复习。
BMC Surg. 2021 Nov 27;21(1):405. doi: 10.1186/s12893-021-01399-6.
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The 2021 WHO Classification of Tumors of the Central Nervous System: a summary.2021 年世卫组织中枢神经系统肿瘤分类:概述。
Neuro Oncol. 2021 Aug 2;23(8):1231-1251. doi: 10.1093/neuonc/noab106.
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The many faces of solitary fibrous tumor; diversity of histological features, differential diagnosis and role of molecular studies and surrogate markers in avoiding misdiagnosis and predicting the behavior.孤立性纤维瘤的多面性;组织学特征的多样性、鉴别诊断以及分子研究和替代标志物在避免误诊和预测行为中的作用。
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Cancer Stem Cells in Soft-Tissue Sarcomas.软组织肉瘤中的癌症干细胞。
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6
Papillary Solitary Fibrous Tumor/Hemangiopericytoma: An Uncommon Morphological Form With NAB2-STAT6 Gene Fusion.乳头状孤立性纤维性肿瘤/血管外皮细胞瘤:一种具有NAB2-STAT6基因融合的罕见形态学类型
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7
Solitary fibrous tumor: An evolving and unifying entity with unsettled issues.孤立性纤维性肿瘤:一个仍存在未解决问题且不断演变并趋于统一的实体。
Histol Histopathol. 2019 Apr;34(4):313-334. doi: 10.14670/HH-18-064. Epub 2018 Nov 15.
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Dedifferentiated Solitary Fibrous Tumor: A Concise Review.去分化孤立性纤维性肿瘤:简要综述。
Arch Pathol Lab Med. 2018 Jun;142(6):761-766. doi: 10.5858/arpa.2016-0570-RS.
9
Extrapleural solitary fibrous tumor: A distinct entity from pleural solitary fibrous tumor. An update on clinical, molecular and diagnostic features.胸膜外孤立性纤维瘤:一种与胸膜孤立性纤维瘤不同的实体。临床、分子及诊断特征的最新进展
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10
NAB2-STAT6 Gene Fusion in Meningeal Hemangiopericytoma and Solitary Fibrous Tumor.脑膜血管外皮细胞瘤和孤立性纤维性肿瘤中的NAB2-STAT6基因融合
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