Sullivan Patrick S, Belfi Lily M, Garcia Roberto A, Fufa Duretti T, Bartolotta Roger J
Boston Medical Center, One Boston Medical Center Place, Boston, MA, 02118, USA.
Weill Cornell Medical College, 1300 York Avenue, New York, NY, 10021, USA.
Skeletal Radiol. 2025 Jul;54(7):1531-1535. doi: 10.1007/s00256-024-04849-3. Epub 2024 Dec 4.
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.
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。