手部肌外皮细胞瘤。

IF 1.9 3区 医学 Q2 ORTHOPEDICS Skeletal Radiology Pub Date : 2024-12-04 DOI:10.1007/s00256-024-04849-3
Patrick S Sullivan, Lily M Belfi, Roberto A Garcia, Duretti T Fufa, Roger J Bartolotta
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引用次数: 0

摘要

肌外皮细胞瘤是一种罕见的良性皮下软组织平滑肌细胞肿瘤,主要见于男性为主的年轻人。我们提出的临床,放射学和组织病理学特征的长期肌外皮细胞瘤的手。我们强调有助于放射科医生和病理学家准确表征这种实体的特征。影像学评估,尤其是MRI,在鉴别肌外皮细胞瘤与其他软组织肿块中起着至关重要的作用。特征性MR表现为界限清晰的病变,增强后增强,t1加权成像与肌肉呈等强度,t2加权成像呈高强度,常伴有中央血管蒂。组织病理学检查显示同心血管周围生长模式,这有助于将肌外皮细胞瘤与其他软组织肿瘤区分开来。免疫组化,这些肌样细胞肿瘤α -平滑肌肌动蛋白和h-caldesmon染色阳性,缺乏核异型性,不高度增殖(相对较低的Ki-67指数和非常低的有丝分裂活性)。绝大多数desmin和CD34呈阴性。治疗通常包括完全手术切除,预后良好,很少复发。尽管罕见,但对于遇到四肢软组织肿块的临床医生来说,意识到这一点很重要。
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Myopericytoma of the hand.

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.

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来源期刊
Skeletal Radiology
Skeletal Radiology 医学-核医学
CiteScore
4.40
自引率
9.50%
发文量
253
审稿时长
3-8 weeks
期刊介绍: Skeletal Radiology provides a forum for the dissemination of current knowledge and information dealing with disorders of the musculoskeletal system including the spine. While emphasizing the radiological aspects of the many varied skeletal abnormalities, the journal also adopts an interdisciplinary approach, reflecting the membership of the International Skeletal Society. Thus, the anatomical, pathological, physiological, clinical, metabolic and epidemiological aspects of the many entities affecting the skeleton receive appropriate consideration. This is the Journal of the International Skeletal Society and the Official Journal of the Society of Skeletal Radiology and the Australasian Musculoskelelal Imaging Group.
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