摘要
目的：分析外周性原始神经外胚层肿瘤（PPNET）的X线平片、CT及MRI的影像学表现，并与病理结果对照研究，提高PPNET的诊断水平。资料与方法：回顾性分析10例经病理证实的PPNET X线平片、ＣＴ、ＭＲＩ资料，并与病理结果结合分析。结果：10例PPNET中，3例位于胫骨，1例位于肱骨，2例位于胸壁，2例位于骨盆，1例位于腹股沟，1例位于腹膜后。X线表现为溶骨性骨质破坏4例，伴有骨质硬化和轻度膨胀的溶骨性骨质破坏1例；CT扫描中，表现为不规则的溶骨性骨质破坏4例，伴有环状骨质硬化和轻度膨胀的溶骨性骨质破坏1例，5例发生于软组织的PPNET表现为边界不清且不规则的软组织肿块影，肿块内密度多不均匀伴坏死、囊变，增强后呈中等程度以上不均匀强化；MRI中，10例发生于骨骼及软组织的PPNET，均表现为T1WI均呈中等或稍高信号，T2WI呈不均匀高信号，增强后病灶均呈不均匀明显强化，部分病例可见分隔样强化。病理形态上Homer-Wright菊形团为其特异性表现，免疫组织化学结果，肿瘤均表达CD99，并不同程度地表达（神经元特异性烯醇化酶）NSE、突触素（Syn）、S-100、Vimentin（波形蛋白）等。结论：合理应用X线、CT、MRI检查，可提高PPNET诊断的准确性，确诊仍依赖于病理和免疫组织化学检查。
Abstract
Objective: To explore the pathology and X-ray, CT and MR images of peripheral primitive neuroectodermal tumors(PPNET) as well as correlate with pathologic results, to improve the diagnostic accuracy of peripheral primitive neuroectodermal tumors. Materials and Methods: X-ray, CT and MRI findings in 10 patients with PPNET were retrospectively analyzed, then correlated with pathology. Results: In the ten lesions 3 were located in the tibia, 1 in humerus, 2 in chest wall, 2 in pelvic bone, 1 in inguinal region, 1 in retroperitoneum. The X-ray findings of 4 cases were lytic bone destruction, lytic lesion with irregular sclerosis and expansion(1 case). The CT findings included irregular lytic lesion(4 cases), lytic lesion with rim-like sclerosis and expansion(1 case). Five tumors in soft tissue showed ill-defined, irregular and heterogenous cystic areas. The tumors showed moderate heterogeneous enhancement after injection of contrast agents. The 10 cases in bone and soft tissue MRI findings showed iso- or hyperintensity on T1WI, and heterogeneous hyperintensity on T2WI. The tumors showed heterogeneous enhancement and reticular enhancement after injection of contrast agents. Homer-Wright daisy-group was a specific pathologic feature of PPNETs. According to immunohistochemical results, all tumor cells showed positive for CD99, and showed various degrees of antigen in epithelium, including NSE, Syn, S-100 and Vimentin. Conclusion: If X-ray, CT and MRI can be used reasonably, the accuracy of diagnosis of PPNET can be improved, the definite diagnosis is depended on the pathological and immunohistochemical results.
关键词
神经外胚瘤 /
原始 /
外周 /
体层摄影术 /
螺旋计算机 /
磁共振成像
Key words
Neuroectodermal tumors /
primitive /
peripheral /
Tomography /
spiral computed /
Magnetic resonance imaging
黄 斌.
外周性原始神经外胚层肿瘤的影像学表现及其病理基础研究[J]. 中国临床医学影像杂志. 2012, 23(9): 617-621
HUANG Bin.
Imaging features and pathologic study of peripheral primitive neuroectodermal tumors[J]. Journal of China Clinic Medical Imaging. 2012, 23(9): 617-621
中图分类号：
Ｒ738.1
R739.43
Ｒ445.2
R814.42
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