Almeida Maria Fernanda Arruda, Patnana Madhavi, Korivi Brinda Rao, Kalhor Neda, Marcal Leonardo
Diagnostic Radiology Resident, A. C. Camargo Hospital, Sao Paulo, SP, Brazil.
Department of Diagnostic Radiology, The University of Texas M. D. Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA.
Case Rep Radiol. 2014;2014:283902. doi: 10.1155/2014/283902. Epub 2014 Jan 9.
Ewing sarcoma and primitive peripheral neuroectodermal tumor (PNET) are high-grade malignant tumors typically found in children and adolescents. These tumors belong to the family of small round cell tumors and are of neuroectodermal origin. Primary Ewing sarcoma of the kidney is rare and because of that is an infrequent differential diagnosis in urologic malignancies. Renal PNET mostly presents with nonspecific symptoms such as hematuria and abdominal pain. The imaging findings are uncharacteristic. The diagnosis is based on the histology, immunohistochemistry, and molecular pathologic findings. Once PNET has been diagnosed, multimodal treatment is indicated. Despite all treatment options, the prognosis of those with metastatic disease is poor.
尤因肉瘤和原始外周神经外胚层肿瘤（PNET）是典型的发生于儿童和青少年的高级别恶性肿瘤。这些肿瘤属于小圆细胞肿瘤家族，起源于神经外胚层。肾脏原发性尤因肉瘤罕见，因此在泌尿系统恶性肿瘤中是不常见的鉴别诊断。肾PNET大多表现为血尿和腹痛等非特异性症状。影像学表现无特征性。诊断基于组织学、免疫组织化学和分子病理学结果。一旦诊断为PNET，就需要进行多模式治疗。尽管有所有的治疗选择，但转移性疾病患者的预后很差。
