| 李静,林海月,戴桂红,等.恶性血管球瘤 11例临床病理特征并文献复习[J].安徽医药,2026,30(6):1135-1138. |
| 恶性血管球瘤 11例临床病理特征并文献复习 |
| Clinicopathological features of 11 cases of malignant glomus tumor and literature review |
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| DOI：10.3969/j.issn.1009-6469.2026.06.015 |
| 中文关键词: 恶性血管球瘤 h-caldesmon Dog-1 诊断 鉴别诊断 |
| 英文关键词: Malignant glomus tumor H-caldesmon Dog-1 Diagnosis Differential diagnosis |
| 基金项目:嘉定区自然科学研究课题( JDKW-2025-0024)；上海市嘉定区卫生健康委员会科研课题计划( 2020-KY-06) |
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| 中文摘要: |
| 目的探讨恶性血管球瘤( MGT)的临床病理学及分子遗传学特征并文献复习。方法回顾性分析 2008年 12月至 2024年 6月于上海健康医学院附属嘉定区中心医院、连云港市第一人民医院、南京医科大学附属泰州人民医院病理科存档的 11例 MGT的临床病理特征并文献复习。结果 11例 MGT发生部位:胃 4例，肺 3例，背部、腕部、腿部、结肠各 1例。肿瘤周边多数可找及良性血管球瘤( GT)的形态， GT肿瘤细胞形态一致，由细胞质红染或略空淡的圆形至卵圆形上皮样细胞呈巢片状或弥漫分布，常围绕血管周围同心圆样生长。良恶性区域间分界较清，恶性区表现为两种形态，一种为梭形细胞肉瘤，另一种为小圆细胞肉瘤。两种形态均可见浸润性生长至周边正常组织。免疫表型: h-caldesmon、Dog-1、白细胞分化抗原 34(CD34)、平滑肌肌动蛋白( SMA)、结蛋白和 S-100蛋白在 MGT中的阳性表达率分别为 90.91%(10/11)、 18.18%(2/11)、27.27%(3/11)、 100.00%(11/11)、 0%(0/11)、 0%(0/11)，Ki-67增殖数在 25%~60%。11例 MGT中有 9例可在肿瘤周边找见良性 GT的形态， 7例良性区为 h-caldesmon阴性，恶性区为 h-caldesmon弥漫阳性。分子遗传学特征: 1例 BRAF V600E突变(9.09%)。结论寻找肿瘤周边良性 GT组织学形态，并观察 h-caldesmon在良恶性区域的倒置表达现象对诊断 MGT有很大的辅助价值。 |
| 英文摘要: |
| Objective To investigate the clinicopathological and molecular genetic characteristics of malignant glomus tumor (MGT) and review the literature.Methods A retrospective analysis was conducted on the clinical and pathological characteristics of 11 casesof MGT that were archived in the Pathology Department of Shanghai Health Medical College Affiliated Jiading District Central Hospi-tal, The First People's Hospital of Lianyungang, and The Affiliated Taizhou People's Hospital of Nanjing Medical University from De.cember 2008 to June 2024. Literature review was also performed.Results The locations of 11 cases of MGT were as follows: 4 cases in the stomach, 3 cases in the lung,1 case each in the back, wrist, leg and colon. Benign glomus tumor (GT) morphology could be foundaround the majority of the tumors. The tumor cells of GT were uniform in shape, consisting of round to oval epithelioid cells with red-stained or slightly pale cytoplasm, distributed in nests, sheets or diffusely, and often grew in concentric circles around blood vessels.The boundaries between benign and malignant areas were relatively clear. The malignant areas presented two morphologies: one wasspindle cell sarcoma and the other was small round cell sarcoma. Both morphologies showed invasive growth into the surrounding nor.mal tissues. Immunotype: The positive expression rates of h-caldesmon, Dog-1, CD34, SMA, Desmin, and S-100 in MGT were 90.91% (10/11),18.18% (2/11),27.27% (3/11), 100.00% (11/11), 0% (0/11), and 0% (0/11), respectively, with Ki-67 ranging from 25% to 60%.Among the 11 cases of MGT, 9 cases showed benign GT morphology around the tumor, 7 cases were negative for h-caldesmon in the be. nign area, and diffuse positive for h-caldesmon in the malignant area. Molecular genetic features: 1 case of BRAF V600E mutation (9.09%) was detected.Conclusion Finding the histological morphology of benign GT tissue around tumors and observing the inverted expression of h-caldesmon in benign and malignant regions has great auxiliary value for the diagnosis of MGT. |
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