四肢原发性胚胎性横纹肌肉瘤的影像学表现及病理特征

Research of imaging findings in primary embryonal rhabdomyosarcoma of the limbs and pathological features

目的探讨四肢原发性胚胎性横纹肌肉瘤的影像特点,以提高对此肿瘤的影像诊断水平。方法选取7例经病理证实的四肢原发性胚胎性横纹肌肉瘤的临床资料、病理结果及影像学表现。结果7例四肢原发性胚胎性横纹肌肉瘤中,2例原发于骨组织,5例原发于软组织,其中1例侵犯相邻骨质。位于骨组织者,X线平片示髓腔呈溶骨性骨质破坏,轻度膨胀,可见骨膜反应;MRI示肿瘤呈长T_(1)、长T_(2)信号改变,增强扫描病灶呈明显不均匀强化。位于软组织者,CT平扫示肿瘤边界清晰,增强扫描病灶轻度强化;MRI示肿瘤呈长T_(1)、长T_(2)信号改变,增强扫描病灶明显强化。结论四肢原发性胚胎性横纹肌肉瘤发病率低,临床症状与影像表现缺乏特异性,确诊依赖于细胞形态学与免疫组织化学检查。

Objective To explore imaging diagnosis of primary embryonal rhabdomyosarcoma of the limbs and improve diagnostic capacity.Methods A retrospective analysis was made on clinical data,pathological results and imaging findings of 7 cases with pathologically approved primary embryonal rhabdomyosarcoma of the limbs.Results Among the 7 cases of primary embryonal rhabdomyosarcoma of the limbs,2 cases were located in bone,5 cases were located in soft tissue and 1 case destroyed adjacent bone.In case of bone with X-ray plain film examination,the marrow cavity of the tumor showed lytic bone destruction and slight expansion,and periosteal reaction could be seen.MRI showed long T_(1) and long T_(2) signal shadows,and the tumor could be heterogeneous enhanced obviously.In case of soft tissue with CT examination,the tumor showed clear tumor boundary,and mild enhancement of lesions in enhanced scan;MRI showed long T_(1) and long T_(2) signal shadows,and the tumor could be enhanced obviously.Conclusion The incidence of primary embryonal rhabdomyosarcoma of the limbs is low,and the clinical symptoms and imaging manifestations are lack of specificity,and histomorphology combined with immunohistochemistry can make the diagnosis.