CT在儿童外周性原始神经外胚层肿瘤诊断中的应用

发布时间：2018-08-29 19:00

【摘要】：目的:分析儿童外周性原始神经外胚层肿瘤(Peripheral primitive neuroectodermal tumor,pPNET)的CT表现,结合临床病理特征,以提高对该病的认识及临床诊断方法。方法:回顾性分析经病理证实的6例儿童(男∶女=2:4)pPNET的CT表现,6例均行CT平扫,其中4例又行增强检查。结果 :6例pPNET均为软组织肿物,2例位于胸壁,2例位于腹膜后,1例位于手掌背侧,1例位于骨盆。软组织pPNET多表现为较大的、边界不清的软组织肿物,伴或不伴邻近的骨质破坏。肿物内密度不均匀,伴有囊变、坏死及分隔,增强后多呈不均匀强化。6例患儿均行手术治疗,术中完整切除肿物,术后病理回报均考虑pPNET。结论:pPNET目前仍为少见病例,影像学表现缺乏特异性,确诊需要依靠病理组织检查及免疫组化,但CT检查在显示肿瘤的位置、大小、来源、血供情况以及邻近组织有否受累等具有较明确的提示。在儿童中,当CT检查提示巨大的、界限不清的软组织肿物包含囊变、坏死及分隔时,应高度怀疑pPNET。HE染色镜下可见Homer-Wright菊形团结构,免疫组化染色肿瘤均表达CD99,更能进一步提示pPNET。
[Abstract]:Objective: to analyze the CT features of peripheral primitive neuroectodermal tumors (Peripheral primitive neuroectodermal tumor,pPNET) in children and to improve the understanding and clinical diagnosis of the disease. Methods: the CT findings of 6 pathologically proved children (male: female: 2: 4) with pPNET were analyzed retrospectively. CT plain scan was performed in all 6 cases, among which 4 cases were examined by contrast enhancement. Results all 6 cases of pPNET were soft tissue masses, 2 cases were located in chest wall, 2 cases were located in retroperitoneum, 1 case was located in dorsal palm of palm, 1 case was located in pelvis. Soft tissue pPNET was characterized by large, ill-defined soft tissue masses with or without adjacent bone destruction. The density of the tumor was uneven, accompanied by cystic change, necrosis and septum. After enhancement, 6 cases of children with heterogeneous enhancement were treated by operation. The tumor was removed completely during operation. The pathological results of all cases considered pPNET. after operation. Conclusion the diagnosis of the disease is still rare, and the imaging findings are not specific. The diagnosis depends on pathological examination and immunohistochemistry, but CT can show the location, size and origin of the tumor. The blood supply and the presence of involvement of adjacent tissues are clear indications. In children, it should be highly suspected that Homer-Wright chrysanthemum structure can be seen under pPNET.HE staining microscope when CT examination indicates that large, ill-defined soft tissue masses contain cystic change, necrosis and septum. Immunohistochemical staining of CD99, in all tumors can further suggest pPNET..
【作者单位】：中国医科大学附属盛京医院小儿外科;
【分类号】：R730.44;R739.4

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