Department of Pathology, Indiana University School of Medicine, Riley Hospital for Children at IU Health, Riley Hospital Drive RM, Indianapolis, IN, USA.
Am J Surg Pathol. 2012 Jan;36(1):94-100. doi: 10.1097/PAS.0b013e318233083b.
Neuroblastoma is the most common extracranial solid tumor to occur during infancy and early childhood. However, primary renal neuroblastoma is rare, and only scattered case reports exist in the English medical literature. We report 8 cases that accumulated at our institution over the past 15 years and summarize their clinicopathologic features. The composite picture of a patient with renal neuroblastoma is that of a boy of 17 months of age, who presented with a large renal mass, about 9 cm in size, accompanied by hypertension. The mass was typically hemorrhagic, either encapsulated or unencapsuated, and infiltrating. A renal neuroblastoma can be undifferentiated, poorly differentiated, or differentiating; it falls into either the favorable or the unfavorable histology category, and presentation at higher stages is the rule. The N-myc is usually unamplified, and the bone marrow is usually not involved at presentation. Unless the tumor is undifferentiated or very poorly differentiated, patients with renal neuroblastoma fare well, although not without new and improved modalities of treatment. Primary renal neuroblastoma is perhaps more common than people realize; a higher level of awareness and early recognition are important for its prognosis and management, as they are very different from Wilms tumor.
神经母细胞瘤是婴儿期和幼儿期最常见的颅外实体瘤。然而，原发性肾神经母细胞瘤很少见，英文医学文献中仅有散在的病例报告。我们报告了过去 15 年来在我院积累的 8 例病例，并总结了其临床病理特征。肾神经母细胞瘤患者的综合特征是一个 17 个月大的男孩，表现为一个约 9 厘米大小的大肾脏肿块，并伴有高血压。肿块通常为出血性，有包膜或无包膜，浸润性生长。肾神经母细胞瘤可以是未分化的、低分化的或分化的；它分为有利或不利的组织学类型，通常处于较高分期。N-myc 通常不扩增，骨髓通常在初始阶段不受累。除非肿瘤是未分化的或非常低分化的，否则肾神经母细胞瘤患者预后良好，尽管并非没有新的和改进的治疗方法。原发性肾神经母细胞瘤可能比人们意识到的更为常见；提高认识和早期识别对于其预后和管理非常重要，因为它们与肾母细胞瘤有很大的不同。
