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骨外周原始神经外胚层肿瘤的影像学及临床特征

Radiological and clinical findings of osseous peripheral primitive neuroectodermal tumors.

作者信息

Wang Chao, Li Bin, Yu Xin-Feng, Xuan Min, Gu Quan-Quan, Qian Wei, Qiu Tian-Tian, Shen Zhu-Jing, Zhang Min-Ming

机构信息

Department of Radiology, The Second Affiliated Hospital of Zhejiang University School of Medicine, Hangzhou, Zhejiang 310009, P.R. China.

出版信息

Oncol Lett. 2015 Jul;10(1):553-559. doi: 10.3892/ol.2015.3233. Epub 2015 May 19.

Abstract

Peripheral primitive neuroectodermal tumor (pPNET) is a rare and highly malignant undifferentiated type of tumor. The aim of the present study was to analyze the computed tomography (CT), magnetic resonance imaging (MRI) and clinical findings of osseous pPNET. The present study retrospectively analyzed the clinical data and CT findings from 15 patients with osseous pPNET; the MRI findings from 11 of these 15 patients were confirmed by histopathological examination. The 15 patients included 9 men and 6 women. The mean patient age was 29 years (range, 16-64 years) and 11 cases were aged <30 years. A CT scan was performed in 15 cases and the findings included a lytic lesion (13 cases), a lytic lesion with irregular sclerosis and dilation (2 cases), a soft tissue mass (15 cases), calcification (2 cases) and periosteal reaction (5 cases). A total of 9 cases of soft tissue mass were heterogeneous, with different sizes of lower-density necrotic areas. An enhanced MRI scan was performed in 11 cases. On T1-weighted images (WI), the soft tissue mass was isointense (8 cases) and marginally hyperintense (3 cases). On T2WI, aggressive soft tissue masses were heterogeneous iso- or hyperintense (11 cases). On contrast-enhanced T1WI, marked heterogeneous enhancement was present in 10 cases and intermediate heterogeneous enhancement in 1 case. The results indicated that osseous pPNET mainly affects male adolescents and young adults. The CT findings of osseous pPNET were destructive lesions with a soft tissue mass and, occasionally, with periosteal reaction. The tumor was often isodense, with patchy hypodense areas. Tumor calcification was uncommon. The MRI findings were those of an aggressive soft tissue mass exhibiting isointensity on T1WI and iso- or hyperintensity on T2WI, with marked heterogeneous enhancement. Although the imaging characteristics of pPNETs may be non-specific, CT and MRI may be useful in delineating the extent of the tumor, identifying distant metastases, predicting resectability and monitoring treatment.

摘要

外周原始神经外胚层肿瘤(pPNET)是一种罕见的高度恶性未分化型肿瘤。本研究的目的是分析骨pPNET的计算机断层扫描(CT)、磁共振成像(MRI)及临床特征。本研究回顾性分析了15例骨pPNET患者的临床资料和CT表现;其中11例患者的MRI表现经组织病理学检查证实。15例患者中,男性9例,女性6例。患者平均年龄为29岁(范围16 - 64岁),11例年龄小于30岁。15例患者均行CT扫描,表现为溶骨性病变(13例)、伴有不规则硬化及扩张的溶骨性病变(2例)、软组织肿块(15例)、钙化(2例)及骨膜反应(5例)。9例软组织肿块密度不均匀,有不同大小低密度坏死区。11例患者行增强MRI扫描。在T1加权像(WI)上,软组织肿块呈等信号(8例),边缘呈稍高信号(3例)。在T2WI上,侵袭性软组织肿块呈不均匀等或高信号(11例)。在增强T1WI上,10例呈明显不均匀强化,1例呈中等不均匀强化。结果表明,骨pPNET主要影响男性青少年及青年成人。骨pPNET的CT表现为伴有软组织肿块的破坏性病变,偶见骨膜反应。肿瘤常呈等密度,有斑片状低密度区。肿瘤钙化少见。MRI表现为侵袭性软组织肿块,T1WI呈等信号,T2WI呈等或高信号,明显不均匀强化。尽管pPNET的影像学特征可能不具有特异性,但CT和MRI有助于明确肿瘤范围、识别远处转移、预测可切除性及监测治疗效果。

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