摘要
目的:通过探讨儿童外周原始神经外胚层肿瘤(peripheral primitive neuroectodermal tumor,pPNET)的临床病理学及影像学特点,旨在提高对此类少见疾病的认识及影像学诊断水平。方法:回顾并分析27例由病理学检查证实为pPNET的患儿的临床病理学及影像学资料,比较分析不同起源pPNET患者临床病理学及影像学特征的差异。结果:27例pPNET患者中,12例来源于骨组织,包括椎体3例,四肢骨3例,髂骨2例,下颌骨、颞骨、颧骨及眼眶各1例;15例源于软组织,包括腹盆腔3例,肾脏3例,胸部3例,下肢肌间隙2例,肾上腺、胰腺、腮腺及颈部各1例。本研究中起源于软组织的pPNET较起源于骨组织的病灶更大(7.9 vs 4.2,P<0.001)、Syn阳性率更高(80.0%vs 25.0%,P=0.007)、更易发生淋巴结转移(5 vs 0,P=0.047)。在计算机体层成像(computed tomography,CT)平扫上,91.7%的病灶表现为不均匀低密度肿块影,起源于骨组织的肿瘤则伴有溶骨性骨质破坏,4例软组织起源的pPNET病灶内见钙化影;增强后所有肿块样病灶呈不均匀强化,并伴不同程度的囊变影。骨组织起源的PNET患者在平扫图像上较软组织起源的PNET更易表现为边界不清(100.0%vs 58.3%,P=0.037)。9例起源于骨组织pPNET患者术前磁共振成像(magnetic resonance imaging,MRI)均显示溶骨性骨质破坏和不规则软组织肿块,T1加权成像(T1-weighted imaging,T1WI)呈等或低信号,T2加权成像(T2-weighted imaging,T2WI)呈不均匀高信号,增强后呈不均匀强化。结论:pPNET可累及身体的各个部位,若CT或MRI上表现为巨大、边界不清、伴有或不伴有骨质破坏的不均匀强化的侵袭性软组织肿块时应考虑本病的可能。
Objective:To investigate the clinicopathological and imaging features of peripheral primitive neuroectodermal tumors(pPNET)in children,then to improve the recognization of this rare disease and the level of imaging diagnosis.Methods:The clinicopathological and imaging findings of 27 patients confirmed with pPNET by pathology were reviewed retrospectively.The differences of clinicopathological and imaging characteristics between patients with different pPNET origins were analyzed.Results:Of 27 pPNET patients,12 cases originated from bone tissue,including 3 cases of centrum,3 cases of limbs,2 cases of ilium,each one case of mandible,temporal bone,cheekbone and orbit;15 cases originated from soft tissue,including 3 cases of abdominal pelvic cavity,3 cases of kidney,3 cases of chest,2 cases of lower limb muscle space and each one case of adrenal gland,pancreas,parotid gland and neck.Compared with the lesions originating from bone in our study,the lesions originating from soft tissue were larger in size(7.9 vs 4.2,P<0.001),higher positive rate of Syn(80.0%vs 25.0%,P=0.007)and higher metastatic rate of lymph node(5 vs 0,P=0.047).On plain computed tomography(CT)images,91.7%lesions showed heterogeneous hypo-dense mass and osteolytic destruction combined if lesions originating from the bone,and calcifications were found in 4 lesions originating from soft tissue.All lesions enhanced heterogeneously with varying areas of cystic changes.Patients with pPNET originating from the bone were more likely to have indistinct borderline than lesions originating from soft tissue(100.0%vs 58.3%,P=0.037)on plain images.Preoperative magnetic resonance imaging(MRI)of 9 patients with pPNET originating from bone showed osteolytic bone destruction and irregular soft tissue mass,with equal or low signal on T1-weighted imaging(T1WI),heterogeneous high signal on T2-weighted imaging(T2WI)and heterogeneous enhancement after contrast injection.Conclusion:pPNET in children can involve any part of the body,and a large,ill-defined,aggressive soft tis
作者
林呈祥
胡培安
乔中伟
沈茜刚
LIN Chengxiang;HU Peian;QIAO Zhongwei;SHEN Xigang(Department of Radiology,Children’s Hospital of Fudan University,National Children’s Medical Center,Fudan University,Shanghai,China;Department of Radiology,Fudan University Shanghai Cancer Center,Department of Oncology,Shanghai Medical College,Fudan University,Shanghai,China)
出处
《肿瘤影像学》
2024年第2期143-149,共7页
Oncoradiology
关键词
小圆细胞肿瘤
原始神经外胚层肿瘤
磁共振成像
计算机体层成像
Small round cell tumors
Primitive neuroectodermal tumor
Magnetic resonance imaging
Computed tomography
