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肾尤文氏肉瘤/原始神经外胚层肿瘤(Ewing’s sarcoma/primitive neuroectodermal tumor,EWS/PNET)是一种罕见的肾肿瘤,恶性程度高,好发于儿童和青少年。患者多无特征性临床表现,常以腹痛或肉眼血尿而就诊。EWS/PNET临床进展迅速,易早期出现远隔转移并导致死亡,预后差,因此,及时、准确地做出诊断尤为重要。患者一经确诊,需接受包括根治性手术切除、化学治疗及放射治疗在内的综合治疗。遗憾的是,EWS/PNET在超声声像图及其他影像学检查中并无特异性表现。目前,肾EWS/PNET的诊断主要依靠其典型的组织学特点和免疫组织化学分析结果,特征性染色体异常也可作为有力的诊断依据。免疫组织化学分析CD99强阳性是其基本特征,t(11;22)染色体异位发生于大约90%的EWS/PNET病例中。本文报道了1例右肾EWS/PNET患者,患者为青年女性,以突发右侧腹痛为主要临床表现。超声检查时发现右肾下极可见一大的混合回声包块,对集合系统造成挤压导致肾上极扩张、积水。彩色多普勒检测到肿瘤内部存在动脉样血流频谱。患者接受了右肾肿瘤根治性切除术,并同时行右肾静脉和下腔静脉癌栓切除术。术后影像学检查提示,肿瘤未累及其他组织和器官。患者随后接受了共计6个周期的化学治疗。通过该肿瘤的特征性组织学特点和免疫组织化学结果,最终确诊为EWS/PNET。患者目前随访5年无复发征象。EWS/PNET的声像图表现虽无特异性,但超声检查仍是肿瘤初始评估的主要方法,超声引导下细针穿刺活检也具有重要的术前诊断价值。
Ewing’s sarcoma / primitive neuroectodermal tumor (EWS / PNET) is a rare type of renal tumor that is highly malignant and occurs in children and adolescents. Patients with no more clinical manifestations, often with abdominal pain or gross hematuria and treatment. EWS / PNET clinical progress is rapid, early easily distant metastasis and lead to death, the prognosis is poor, therefore, timely and accurate diagnosis is particularly important. Once diagnosed, patients need to be treated with a combination of radical surgery, chemotherapy and radiation therapy. Unfortunately, EWS / PNET is not specific for sonography and other imaging studies. At present, the diagnosis of renal EWS / PNET depends mainly on its typical histological features and immunohistochemistry results, and characteristic chromosomal abnormalities can also be used as a powerful diagnostic basis. Immunohistochemical analysis of CD99 strongly positive is its basic feature, t (11; 22) chromosomal ectopic occurred in about 90% of EWS / PNET cases. This paper reports a case of right renal EWS / PNET patients, patients with young women, with sudden right abdominal pain as the main clinical manifestations. Ultrasound examination revealed a very large mixed echo mass in the lower right kidney, which caused the expansion of the renal upper pole and water accumulation on the collection system. Color Doppler detected arterial blood flow within the tumor spectrum. The patient underwent a radical nephrectomy of the right kidney and a right thrombectomy of the right renal vein and inferior vena cava was performed simultaneously. Postoperative imaging examination revealed that the tumor did not affect other tissues and organs. The patient subsequently received a total of 6 cycles of chemotherapy. Through the characteristic histological features of the tumor and immunohistochemical results, the final diagnosis of EWS / PNET. Patients are currently followed up for 5 years without signs of recurrence. Although EWS / PNET sonography is nonspecific, ultrasound is still the primary method of tumor initial evaluation. Ultrasound-guided fine needle aspiration biopsy also has important preoperative diagnostic value.