神经母细胞瘤的影像学诊断

神经母细胞瘤是儿童颅外最常见的实体瘤,占儿童肿瘤的10%。其预后与肿瘤的分级密切相关。影像学的诊断能为临床的治疗和随访提供有价值的信息。核医学显像主要展示肿瘤及其转移灶的功能状况,而X线计算机体层摄影术和磁共振成像在结构方面能提供更详细和确切的资料。每一种方法都有独到之处,起互补作用。     

儿童神经母细胞瘤的CT诊断

目的：探讨ＣＴ对儿童神经母细胞瘤的诊断价值。材料与方法：分析经手术、病理证实的２６例神经母细胞瘤的ＣＴ表现，其中：神经母细胞瘤２１例，神经节神经母细胞瘤５例。胸部７例（２７％），肾上腺１６例（６２％），腹部、盆腔交感神经链３例（１１％）。全部病例行ＣＴ平扫与增强检查。结果：肾上腺和后纵隔近头端为本组两个最好发部位。肿瘤钙化发生率为７３％，肿瘤大小与钙化无关，神经母细胞瘤钙化明显多于神经节神经母细胞瘤，转移瘤可发生钙化。强化程度与瘤细胞分化程度相关。肿瘤向椎管内延伸２例，肾上腺神经母细胞瘤侵袭同侧肾脏７例，侵袭肝脏３例，肝转移１例，淋巴结转移１９例。１３例患者尿３－甲氧－４－羟苦杏仁酸（ＶＭＡ）阳性。本组术前诊断准确率为９２％。结论：ＣＴ是诊断儿童神经母细胞瘤最有价值的检查方法之一，好发部位、钙化、早期发生淋巴结转移，以及血、尿ＶＭＡ增高是诊断的重要依据。ＣＴ表现可为术前定性诊断、确定治疗方案及估计预后提供重要依据。     

Antenatal sonographic diagnosis of adrenal neuroblastoma

We report an infant with adrenal neuroblastoma diagnosed prenatally by ultrasonography. Early diagnosis is essential since survival is inversely related to the increasing age of the patient.     

Sonographic diagnosis and follow-up of abdominal neuroblastoma

Intraabdominal neuroblastomas were evaluated by sonography in 30 children, 18 of whom underwent 2–5 studies, for a total of 63 examinations. All tumors had a variable and inhomogeneous echo pattern, but the level of echogenicity and changes in echo texture with therapy were of no value in predicting the course of disease. Sonography was limited in delineating the extent of tumor and in following response to therapy. Total resolution of abdominal disease could not always be accurately diagnosed with sonography. Computed tomography is recommended to evaluate further those children in whom abdominal disease is thought to have resolved, since heavily calcified, residual neuroblastoma may be mistaken for bowel gas on sonography.     

Sonographic diagnosis and follow-up of abdominal neuroblastoma

Intraabdominal neuroblastomas were evaluated by sonography in 30 children, 18 of whom underwent 2-5 studies, for a total of 63 examinations. All tumors had a variable and inhomogeneous echo pattern, but the level of echogenicity and changes in echo texture with therapy were of no value in predicting the course of disease. Sonography was limited in delineating the extent of tumor and in following response to therapy. Total resolution of abdominal disease could not always be accurately diagnosed with sonography. Computed tomography is recommended to evaluate further those children in whom abdominal disease is thought to have resolved, since heavily calcified, residual neuroblastoma may be mistaken for bowel gas on sonography.     

儿童神经母细胞瘤的CT诊断

目的探讨儿童神经母细胞瘤的CT表现,以提高对本病的认识。方法回顾性分析11例经病理证实的儿童神经母细胞瘤的CT表现,包括肿瘤部位、大小、边缘、密度、强化程度、转移等。其中5例仅行CT平扫,6例行CT平扫及增强。结果11例中,源于肾上腺8例,源于腹部交感神经链2例,源于后纵隔交感神经链1例;10例肿块呈不规则分叶状,直径2．5-11cm。边界不清,10例肿块密度不均匀、可见囊变或钙化。在6例增强扫描中,6例都呈不均匀强化。转移常见,其中淋巴结转移3例,骨转移1例,肺转移1例。结论儿童神经母细胞瘤CT表现有一定的影像特征,有助于提高临床诊断。     

I-MIBG scintigraphy in the diagnosis of neuroblastoma in children

Since the early eighties, *I-MIBG (= *I-metaiodobenzylguanidine), which is stored in the neurotransmitter storage granules of chromaffin cells, has been increasingly used for the detection of pheochromocytomas and allied sympathoadrenal pathologies. The aim of this paper is to illustrate, by means of clinical examples, the role of *I-MIBG-scintigraphy in child neuroblastoma and to underline its original place, compared with other imaging techniques, in determining the neuro-ectodermal origin of a tumor as well as in establishing the extension of the lesions at the time of diagnosis and during follow-up.     

小儿神经母细胞瘤的CT诊断与病理分析

目的:探讨小儿神经母细胞瘤的CT征象及病理、免疫组化特征。方法:回顾性分析经手术、病理证实的28例小儿神经母细胞瘤的CT表现,并与手术病理作对比分析。结果:28例神经母细胞瘤位于腹膜后23例,胸椎椎旁2例,颈部3例。肿瘤瘤体较大,其中22例为单发,6例多发,23例密度不均匀,23例肿瘤内可见钙化及囊变坏死区。22例增强扫描病例中14例呈轻-中度不均匀强化,17例侵犯邻近组织、器官或发生转移。28例神经母细胞瘤的共同组织病理特点为肿瘤由大小较一致的小圆形或卵圆形细胞组成,核大且染色深,多呈菊花瓣样排列。所有病例均可见阳性表达的神经内分泌细胞标记物。结论:小儿神经母细胞瘤的CT及病理学表现有一定的特征性,对本病的诊断有较大价值。     

儿童纵隔神经母细胞瘤的CT与MRI诊断

目的 探讨儿童纵隔神经母细胞瘤的CT与MRI表现及其诊断价值.方法 回顾性分析21例经病理证实的儿童纵隔神经母细胞瘤的CT和MRI表现.19例行CT检查,8例行MRI检查.结果 20例肿瘤位于后纵隔,呈椭圆形或不规则形软组织肿块,突向肺野,边界较清楚.CT平扫示肿瘤密度较均匀12例,密度不均匀7例,15例肿瘤内伴有钙化.13例行增强检查,7例均匀强化,6例不均匀强化.8例MRI检查T1WI示肿瘤呈等或稍低信号,与胸壁肌肉相近,T2 WI呈不均匀稍高信号,3例肿瘤内伴出血、囊变呈混杂信号.结论 儿童患者,CT或MRI检查发现后纵隔肿物,特别是其内伴钙化者,诊断时应首先考虑神经母细胞瘤.     

Pulmonary metastases at diagnosis of neuroblastoma in pediatric patients: CT findings and prognosis

OBJECTIVE: We undertook this study to determine the frequency, CT appearance, and clinical implications of the rare occurrence of pulmonary metastases among children presenting with neuroblastoma. MATERIALS AND METHODS: A search of the Children's Cancer Group database revealed 21 of 567 children with reported lung metastases at original diagnosis of neuroblastoma. CT examinations available for 17 of these patients were analyzed retrospectively to determine if lung metastases were present, and if so, to characterize their radiographic features. RESULTS: Seventeen (3%) of 567 patients presenting with Evans stage IV neuroblastoma had confirmed pulmonary metastases at diagnosis. All had metastases to at least one site other than the lungs. The most common CT appearance of pulmonary lesions was of up to five, small, bilateral, noncalcified nodules. In nine patients (53%), the pulmonary nodules initially resolved with treatment. In this cohort, six children developed progressive disease and died, and three are still alive. All eight children whose lung lesion did not completely respond to treatment died. Overall, children with pulmonary metastases had unfavorable Shimada histology, a higher association with amplification of the MYCN oncogene (p = 0.0002), and a decreased event-free survival (p < 0.001) when compared with all children with stage IV neuroblastoma without pulmonary metastases. CONCLUSION: The search for neuroblastoma lung metastases, which occur more frequently than previously reported, is clinically important because their presence portends a poor prognosis.     

Pitfalls in the diagnosis of neuroblastoma by 99mTc-MDP scintigraphy: A case report

A routine bone scan was performed on an infant presenting with abdominal mass. Initially, the stasis of activity in the right ureter, which was compressed by a large abdominal meningocoele, was mistaken for activity in a neuroblastoma. A subsequent kidney scintigram led to the correct interpretation. The misinterpretation of abdominal ^99mTc-MDP accumulation can be prevented by the correlation of bone and kidney scintigrams. In this way, the correct site of the radiopharmaceutical concentration can be assessed, and the damage caused by any kind of abdominal mass to the compressed urinary tract and kidney can be assessed.     

儿童神经母细胞瘤肺转移的临床影像分析

目的：分析儿童神经母细胞瘤肺部转移的CT表现及其与临床的关系,提高对该病的认识。方法：搜集经手术病理或骨髓穿刺活检证实的神经母细胞瘤4例,均行胸部螺旋CT检查,其中3例随访复查。结果：4例神经母细胞瘤患儿中,有2例于首诊时发现肺部转移。神经母细胞瘤肺部转移的CT表现为肺内单发或多发、实性、磨玻璃密度或混合密度、无钙化的结节,边缘光滑或锐利。3例同时伴胸膜转移。结论：神经母细胞瘤肺转移的CT表现有一定的特征性,胸部CT检查可以为临床治疗及随访提供重要的影像学依据,并帮助临床判断预后。     

小儿腹膜后成神经细胞瘤影像学与病理的对照研究

目的 探讨ＣＴ、ＭＲＩ对小儿腹膜后成神经细胞瘤的诊断价值和限度。方法 对３２例中２０例术前同时行ＭＲＩ、ＣＴ检查者与术中所见对照,并对其中１９例术后离体肿瘤的ＭＲＩ、ＣＴ影像与病理大切片对照。结果 ＭＲＩ表现一般为Ｔ１Ｗ１中低信号,Ｔ２Ｗ１明显高信号,且可见低信号网格及肿瘤对血管的包绕。组织学基础：原始成神经细胞核大密集,无间质;瘤巢周围有神经纤维。ＣＴ表现肿瘤为不均匀低密度,可见钙化。术前判断肿     

The diagnosis of abdominal neuroblastoma: Relative roles of ultrasonography,CT, and MRI

Abdominal neuroblastoma is reviewed in terms of its diagnosis, including in utero, at birth, and through infancy into childhood. Age at diagnosis remains the best predictor of survival, with infants under 1 year of age having almost 100% cure. Ultrasonography and magnetic resonance imaging (MRI) are the recommended diagnostic modalities to stage the abdominal primary, although computed tomography (CT) (including myelography) is still widely and efficiently used. Examples are given of each stage.     

嗅神经母细胞瘤伴发神经内分泌副瘤综合征的诊疗分析

 目的 总结伴有神经内分泌副瘤综合征的嗅神经母细胞瘤临床及病理特点。方法 回顾性分析2010-08至2020-08医院伴有神经内分泌副瘤综合征的4例嗅神经母细胞瘤患者临床及病理资料。其中男2例,女2例。年龄22～48岁, 平均39.5岁。根据改良Kadish分期,4例病变均为C期;根据Dulguerov分期,T₃期1例,T₄期3例。结果 所有患者均行内镜下肿瘤切除术,并获得阴性切缘。围术期3例出现尿崩、电解质紊乱及激素改变。术后1例出现颅内积血,无脑脊液漏等并发症。术后2周内尿崩、电解质紊乱及激素改变大致恢复正常。术后随访6～125个月,未见肿瘤复发及转移。免疫组化染色见肿瘤细胞中突触素(Syn)、嗜铬素A(ChgA)等多呈阳性,Ki-67染色显示细胞增殖指数为1%～50%。结论 嗅神经母细胞瘤患者伴发的神经内分泌症状隐匿,可表现为电解质及激素水平紊乱,围术期风险高,需提高对该病的认识。随访中定期监测电解质水平,可及时发现肿瘤复发。