Huang Ming-De, Hsu Li-Sheng, Chuang Huei-Chieh, Lin Wei-Yu, Lin Wei-Hsiu, Yen Chih-Wei, Chen Min-Lang
Department of Diagnostic Radiology, Chang Gung Memorial Hospital, Chiayi Chang Gung University College of Medicine, Taoyuan Department of Physical Education, Health and Recreation, National Chiayi University Department of Pathology Department of Urology Department of Gastroenterology and Hepatology, Chang Gung Memorial Hospital, Chiayi Department of Diagnostic Radiology, Antai Tian-Sheng Memorial Hospital, Pingtung, Taiwan.
Medicine (Baltimore). 2018 Apr;97(14):e0345. doi: 10.1097/MD.0000000000010345.
Adult renal neuroblastoma (NB) is extremely rare, and there have been only a few cases previously described in the literature. We report a case of adult renal NB and summarize the clinical and imaging features of the reported cases.
A 41-year-old female was admitted to our hospital with a chief complaint of gross hematuria that had persisted for a month. Nonenhanced computed tomography (CT) revealed a hypodense right renal mass without calcification. Enhanced CT showed an infiltrative, heterogeneously enhancing right renal mass with retrocaval lymphadenopathy and right renal vein thrombus. Magnetic resonance imaging (MRI) revealed that the right renal mass was isointense relative to the renal parenchyma on nonenhanced T1-weighted images; it showed mixed hypointensity and hyperintensity on T2-weighted images, and heterogeneous enhancement with a hyperintense rim on fat-saturated, enhanced T1W images. The initial impression was renal cell carcinoma (RCC).
Adult renal neuroblastoma.
Right nephroureterectomy with lymph node dissection was performed. The pathology and immunohistochemistry confirmed the diagnosis of renal NB with retrocaval lymphadenopathy and retroperitoneal metastasis.
After surgery, the patient received 6 courses of chemotherapy, and no recurrence was observed during a 24-month follow-up period.
The clinical picture of adult renal NB is that of a 44-year-old woman, presenting with an abdominal or renal mass about 13cm in size, accompanied by hypertension, hematuria, or pain. In contrast to CT features described in previous literature, no tumor calcification is mentioned in these adult renal NB cases. It is difficult to differentiate renal NB from RCC based on CT or MRI. However, biopsy, urinary catecholamine levels, and metaiodobenzylguanidine (MIBG) scan may aid in presurgical diagnosis.
成人肾神经母细胞瘤（NB）极为罕见，此前文献中仅报道过少数病例。我们报告一例成人肾NB病例，并总结所报道病例的临床及影像学特征。
一名41岁女性因持续一个月的肉眼血尿为主诉入院。非增强计算机断层扫描（CT）显示右肾有一个低密度肿块，无钙化。增强CT显示一个浸润性、不均匀强化的右肾肿块，伴有腔静脉后淋巴结肿大及右肾静脉血栓形成。磁共振成像（MRI）显示，在非增强T1加权图像上，右肾肿块与肾实质等信号；在T2加权图像上呈混合低信号和高信号，在脂肪饱和增强T1加权图像上呈不均匀强化且有高信号边缘。初步印象为肾细胞癌（RCC）。
成人肾神经母细胞瘤。
行右肾输尿管切除术及淋巴结清扫术。病理及免疫组化证实为肾NB伴腔静脉后淋巴结肿大及腹膜后转移。
术后患者接受了6个疗程的化疗，在24个月的随访期内未观察到复发。
成人肾NB的临床表现为一名44岁女性，表现为大小约13cm的腹部或肾肿块，伴有高血压、血尿或疼痛。与先前文献中描述的CT特征不同，这些成人肾NB病例中未提及肿瘤钙化。基于CT或MRI很难将肾NB与RCC区分开来。然而，活检、尿儿茶酚胺水平及间碘苄胍（MIBG）扫描可能有助于术前诊断。
