成人原发性横纹肌肉瘤的临床、病理及影像学特征分析

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目的:探讨成人原发性横纹肌肉瘤(rhabdomyosarcoma,RMS)患者的临床及影像学特征。方法:回顾性分析4例经手术病理证实的成人原发性RMS患者的临床资料和影像学表现。结果:4例患者中,病变位于头颈部2例,位于盆腔1例,位于纵隔1例。所有患者均因相应部位肿胀或压迫症状就诊。CT显示病灶为软组织密度肿块,密度欠均匀,周围组织受压推移或侵犯;增强后病灶呈明显不均匀强化,4例患者的病灶内均可见坏死,但未见钙化成分。MRI检查T1WI图像上可见病灶呈等信号,T2WI图像上则以稍高信号为主;增强后呈不均匀持续强化。4例患者的病理组织行免疫组织化学染色,结果肌细胞生成素(Myogenin)均(+),波形蛋白(Vimentin)均(+),1例患者结蛋白Desmin(+)。结论:成人原发性RMS罕见,其在CT及MRI图像上具有一定特征性,结合患者的临床表现进行分析,可提高诊断的准确率。 Objective: To investigate the clinical and imaging features of adult patients with rhabdomyosarcoma (RMS). Methods: The clinical data and imaging findings of 4 adult patients with primary RMS confirmed by pathology were retrospectively analyzed. Results: Among the 4 patients, the lesions were located in the head and neck in 2 cases, in the pelvic cavity in 1 case and in the mediastinum in 1 case. All patients due to swelling or oppression of the corresponding parts of the doctor. CT showed that the lesions were soft tissue density lumps, the density is not uniform, the surrounding tissue was pushed or inflated; enhanced lesions were significantly uneven enhancement, 4 patients were lesions within the visible necrosis, but no calcification. MRI showed similar lesions on the T1WI images and T2 slightly higher signals on the T2WI images. After the enhancement, uneven enhancement was observed. Immunohistochemical staining was performed on the histopathology of 4 patients. Results Myogenin (+), Vimentin (+) and Desmin (+) were all found in 1 patient. Conclusion: The primary RMS is rare in adults. It has certain characteristics on CT and MRI images. Combined with the clinical manifestations of patients, it can improve the diagnostic accuracy.
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