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目的分析小儿外周原始神经外胚层肿瘤(pPNET)的MSCT表现,提高对该病的认识。方法回顾性分析11例经病理确诊的pPNET的MSCT表现。结果 8例巨大软组织肿块伴骨质破坏,其中3例表现为骨质放射状增生伴局部骨质破坏,其中2例位于胸壁,1例位于肩胛骨;5例为溶骨性骨质破坏,其中2例位于后纵隔、1例发生于副鼻窦、1例发生在腰椎和1例发生在骶椎。3例为未伴骨破坏的软组织肿块,其中2例发生于腹膜后,1例发生于皮下脂肪层内。除发生在皮下脂肪层内病灶较小者,其余病灶都较大,平均直径为8 cm。软组织肿块以压迫推移邻近组织的方式生长。瘤体内钙化少见。结论 pPNET的MSCT典型表现为巨大软组织肿块,伴或不伴骨质破坏,增强呈均匀或非均匀的团絮状强化,大血管穿行于瘤体内较少见。有一定的特点,需与其他恶性软组织肿瘤鉴别。MSCT能较好显示病变内部结构,血供状况以及与邻近组织的关系,有助于术前分期、手术方案的确定、有无远处转移和治疗效果评价。
Objective To analyze the MSCT manifestations of pediatric peripheral neuroectodermal tumors (pPNET) and to improve their understanding of the disease. Methods Retrospective analysis of 11 cases of pathologically confirmed pPNET MSCT performance. Results Eight cases of massive soft tissue mass with bone destruction were found. Three cases showed radial bone hyperplasia with local bone destruction, of which 2 cases were located in the chest wall and 1 case was located in the scapula; 5 cases were osteolytic osteolysis, of which 2 cases Located in the posterior mediastinum, one occurred in the paranasal sinus, one in the lumbar spine and one in the sacral vertebra. Three cases of soft tissue mass without the destruction of the bone, of which two cases occurred in the retroperitoneum, one case occurred in the subcutaneous fat layer. Except for the lesion in the subcutaneous fat layer smaller, the remaining lesions are larger, with an average diameter of 8 cm. Soft tissue mass grows in a manner that oppresses the adjacent tissue. Tumor in vivo rare. Conclusion MSCT of pPNET is characterized by massive soft tissue mass with or without destruction of bone mass, enhancement of uniform or nonuniform flocculent enhancement, and less vascular invasion of the tumor. Have certain characteristics, need to distinguish with other malignant soft tissue tumors. MSCT can better display the internal structure of the lesion, blood supply and the relationship with the adjacent tissues, help to determine the preoperative staging, surgical plan, with or without distant metastasis and the evaluation of the therapeutic effect.