腹盆部外周型原始神经外胚层瘤的CT 诊断与病理分析

目的：分析腹盆部外周型原始神经外胚层瘤(pPNET)的临床特点、CT 表现及其与病理的联系,提高对本病的认识。方法回顾性分析23例经病理证实的腹盆部 pPNET 的 CT 表现、临床资料及病理特征。结果(1)所有患者术前临床及影像资料完整。(2)23例病灶 CT 表现多为大小不一、边界模糊的肿块影,平均直径约10.72 cm；9例呈不同程度的囊变坏死,2例见小片状出血灶,4例出现点状钙化。CT 增强后多数呈不均匀轻中度强化,囊变、坏死更明显。12例出现不同程度的复发、周围侵犯或远处转移,5例合并癌栓形成,4例见肿瘤滋养血管。(3)免疫组化示19例 CD99表达,11例 CD56表达,12例 Vim(波形蛋白)表达,11例 Syn(突触素)表达,4例 NSE(神经元特异性烯醇化酶)表达,4例 FLI-1表达及4例 EMA(上皮细胞抗原)表达。结论腹盆部pPNET 发病年龄较小且恶性程度高,常为较大不均匀肿块,多伴囊变坏死,浸润性生长,增强后实性成分多呈渐进性轻中度强化。

CT and pathological analysis of peripheral primitive neuroectodermal tumors in abdomen-pelvis region

Objective To analyze the correlations of clinical characteristics,CT and pathology features of peripheral primitive neuroectrodermal tumors (pPNET)in abdomen-pelvis region,and to improve the understanding of the disease.Methods The clinical information,CT and pathological features of 23 confirmed pPNET patients in abdomen-pelvis region were analyzed retrospectively.Results (1)All patients had preoperative clinical and imaging data integrallty.(2)23 cases showed soft tissue masses with fuzzy boundary and the average diameter were 10.72 cm.Among all,9 cases showed with varying degrees of sac necrosis,while 2 cases showed with platelet hemorrhage and 4 cases with speckled calcifications.Most showed slight-to-moderate heterogeneous enhancement and the necrosis and cystic are-as showed more obvious.12 cases showed with different degree of recurrence,invasion or distant metastasis.5 cases combined with tumor emboli and 4 cases with tumor feeding vessels.(3)Immunohistochemical examination showed CD99 expressed in 1 9 patients, CD56 in 11,vimentin(Vim)in 12,synaptophysin(Syn)in 11,neuron specific enolase(NSE)in 4,FLI-1 in 4 and epithelial membrane antigen (EMA)in 4 patients.Conclusion pPNET is common among young patients with a high degree of malignancy.CT features include large mass with invasive growth,commonly combined heterogenous density,necrosis and cystic changes.Persistent enhancement is observed during nephrographic phase.