Jiang Maoyan, Hu Xianwen, Qian Kai, Yang Peiqing, Tang Yang, Wang Pan, Cai Jiong
Department of Nuclear Medicine, Affiliated Hospital of Zunyi Medical University, Zunyi, China.
Department of Thoracic Surgery, Affiliated Hospital of Zunyi Medical University, Zunyi, China.
Transl Pediatr. 2022 Jul;11(7):1267-1273. doi: 10.21037/tp-22-205.
The neuroblastoma (NB) is a highly malignant tumor of the ectoderm of sympathetic nerve cells and one of the most common malignant tumors in children, which can occur in any part of the sympathetic nerve plexus distribution, however it is less common in the kidney.
Here we present a case of a 4-year-old boy who came to our hospital for medical help because of "abdominal distension for 1 day". Abdominal computed tomography (CT) revealed a huge retroperitoneal soft tissue mass with unclear boundaries with kidneys and adrenal glands, and low-density cystic necrosis areas were seen in the tumor. Contrast-enhanced scan showed that the mass showed mild heterogeneous enhancement. Based on these findings in the patient, he was initially considered to have Wilms tumor, but was finally pathologically confirmed as renal NB. The purpose of this article is to provide the clinicopathological features and CT manifestations of renal NB, and to discuss its differential diagnosis with other renal tumors, in order to better provide clinicians with a better understanding of the rare solid tumor. The renal NB is usually a large lobulated soft tissue mass with unclear boundary and incomplete capsule, which is prone to hemorrhage, necrosis, cystic degeneration and calcification. On CT, the mass is usually isodensity or slightly hypodense, and the cystic degeneration and necrosis area is hypodense, and on contrast-enhanced scan, it can be mildly to significantly enhanced, but the cystic degeneration and necrosis area have no enhancement.
The CT findings of our case were atypical and overlapped with those of Wilms, so our case suggests that renal NB should be considered as one of the differential diagnoses of Wilms, the most common malignancy of the kidney in children.
神经母细胞瘤（NB）是一种起源于交感神经细胞外胚层的高度恶性肿瘤，是儿童最常见的恶性肿瘤之一，可发生于交感神经丛分布的任何部位，但较少见于肾脏。
在此，我们报告一例4岁男孩，因“腹胀1天”前来我院就诊。腹部计算机断层扫描（CT）显示腹膜后巨大软组织肿块，与肾脏和肾上腺边界不清，肿瘤内可见低密度囊性坏死区。增强扫描显示肿块呈轻度不均匀强化。根据患者的这些表现，最初考虑为肾母细胞瘤，但最终经病理确诊为肾NB。本文旨在介绍肾NB的临床病理特征及CT表现，并探讨其与其他肾脏肿瘤的鉴别诊断，以便更好地为临床医生提供对这种罕见实体瘤的认识。肾NB通常为大的分叶状软组织肿块，边界不清，包膜不完整，容易发生出血、坏死、囊性变和钙化。CT上，肿块通常呈等密度或稍低密度，囊性变和坏死区呈低密度，增强扫描时，肿块可呈轻度至明显强化，但囊性变和坏死区无强化。
我们病例的CT表现不典型，与肾母细胞瘤的表现重叠，因此我们的病例提示肾NB应被视为儿童最常见的肾脏恶性肿瘤肾母细胞瘤的鉴别诊断之一。
