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手部肌周细胞瘤

Myopericytoma of the hand.

作者信息

Sullivan Patrick S, Belfi Lily M, Garcia Roberto A, Fufa Duretti T, Bartolotta Roger J

机构信息

Boston Medical Center, One Boston Medical Center Place, Boston, MA, 02118, USA.

Weill Cornell Medical College, 1300 York Avenue, New York, NY, 10021, USA.

出版信息

Skeletal Radiol. 2025 Jul;54(7):1531-1535. doi: 10.1007/s00256-024-04849-3. Epub 2024 Dec 4.

DOI:10.1007/s00256-024-04849-3
PMID:39633156
Abstract

Myopericytoma is a rare benign smooth-muscle cell neoplasm of the subcutaneous soft tissues that is primarily found in young adults with a male predominance. We present the clinical, radiologic, and histopathologic features of a longstanding myopericytoma of the hand. We emphasize the features that help the radiologist and pathologist to accurately characterize this entity. Radiologic evaluation, particularly MRI, plays a crucial role in differentiating myopericytoma from other soft tissue masses. Characteristic MR features include a well-demarcated lesion with intense post-contrast enhancement, isointensity to muscle on T1-weighted imaging, and hyperintensity on T2-weighted imaging, often with a central vascular pedicle. Histopathologic examination reveals a concentric perivascular growth pattern, which helps to distinguish myopericytoma from other soft tissue tumors. Immunohistochemically, these myoid cell neoplasms stain positive for alpha-smooth muscle actin and h-caldesmon, lack nuclear atypia, and are not highly proliferative (relatively low Ki-67 index and very low mitotic activity). The vast majority stain negative for desmin and CD34. Treatment typically involves complete surgical resection, with excellent prognosis and rare recurrence. Despite its rarity, awareness of this entity is important for clinicians who encounter soft tissue masses of the extremities.

摘要

肌周细胞瘤是一种罕见的皮下软组织良性平滑肌细胞肿瘤,主要见于年轻成年人,男性居多。我们报告了一例长期存在的手部肌周细胞瘤的临床、放射学和组织病理学特征。我们强调有助于放射科医生和病理科医生准确识别该实体的特征。放射学评估,尤其是磁共振成像(MRI),在鉴别肌周细胞瘤与其他软组织肿块方面起着关键作用。典型的MRI特征包括边界清晰的病变,增强后强化明显,在T1加权成像上与肌肉等信号,在T2加权成像上呈高信号,常伴有中央血管蒂。组织病理学检查显示为同心性血管周围生长模式,这有助于将肌周细胞瘤与其他软组织肿瘤区分开来。免疫组织化学方面,这些肌样细胞肿瘤α平滑肌肌动蛋白和h-钙调蛋白染色阳性,无核异型性,增殖不活跃(Ki-67指数相对较低,有丝分裂活性极低)。绝大多数对结蛋白和CD34染色阴性。治疗通常包括完整的手术切除,预后良好,复发罕见。尽管其罕见,但对于遇到四肢软组织肿块的临床医生来说,了解这个实体很重要。

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本文引用的文献

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Rare Case of Intravascular Myopericytoma-Imaging Characteristics and Review of the Literature.血管内肌周细胞瘤罕见病例——影像学特征及文献综述
Diagnostics (Basel). 2022 Oct 13;12(10):2473. doi: 10.3390/diagnostics12102473.
2
Malignant myopericytoma: Case report and review of the literature.恶性肌纤维母细胞瘤:病例报告及文献复习。
J Cutan Pathol. 2023 Jan;50(1):39-42. doi: 10.1111/cup.14313. Epub 2022 Sep 12.
3
Myopericytoma: A Review of Twenty-Three Cases Over Twelve Years and a Case Report of a Rare Neoplasm.肌纤维母细胞肉瘤:十二年 23 例回顾及一种罕见肿瘤的病例报告。
Am J Dermatopathol. 2022 Sep 1;44(9):623-631. doi: 10.1097/DAD.0000000000002130. Epub 2022 Apr 27.
4
Fast growing myopericytoma of the hand: Case report and literature review.手部快速生长的肌周细胞瘤:病例报告及文献复习
Int J Surg Case Rep. 2021 Aug;85:106220. doi: 10.1016/j.ijscr.2021.106220. Epub 2021 Jul 19.
5
Imaging features of myopericytoma of the breast: A case report and review of the literature.乳腺肌周细胞瘤的影像学特征:1例病例报告并文献复习
Radiol Case Rep. 2020 Nov 5;16(1):98-102. doi: 10.1016/j.radcr.2020.10.051. eCollection 2021 Jan.
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Radiopathological correlations of myopericytomas of the hand: emphasis on the MRI perivascular pushing growth pattern.手部肌周细胞瘤的放射病理学相关性:着重于MRI血管周围推挤生长模式
BJR Case Rep. 2020 Sep 29;6(2):20190074. doi: 10.1259/bjrcr.20190074. eCollection 2020 Sep.
7
Myopericytoma/myopericytomatosis of the lower extremity in two young patients: a recently designated rare soft tissue neoplasm.两名年轻患者下肢的肌周细胞瘤/肌周细胞瘤病:一种新近命名的罕见软组织肿瘤。
Radiol Case Rep. 2017 Nov 13;13(1):275-280. doi: 10.1016/j.radcr.2017.10.010. eCollection 2018 Feb.
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Medicine (Baltimore). 2017 Dec;96(49):e9064. doi: 10.1097/MD.0000000000009064.
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