成人肾原始神经外胚层肿瘤：一例报告
Primitive neuroectodermal tumor of the kidney in an adult: a case report.
作者信息
Businger Adrian, Zettl Andreas, Sonnet Stefan, Ruszat Robin, von Flüe Markus
机构信息
Department of Surgery, St. Claraspital, Kleinriehenstrasse 30, CH-4016 Basel, Switzerland.
出版信息
Cases J. 2009 Jun 5;2:6791. doi: 10.4076/1757-1626-2-6791.
INTRODUCTION
Primitive neuroectodermal tumors (PNETs) occur predominantly in childhood preferentially in the soft tissues of the lower extremity and the paraspinal region. We present here a rare case of a PNET of the kidney in an adult.
CASE PRESENTATION
A tumor adjacent to the right kidney was detected by ultrasound coincidentally at a routine check-up in a 46-year-old woman with irritable bowel syndrome in her medical history. The patient had no clinical signs. Contrast-enhanced computerized tomography scan of the abdomen demonstrated a highly vascularized renal tumor. A retroperitonealectomy with en-bloc resection of the kidney was performed, and histopathological work-up showed a primitive neuroectodermal tumor of the kidney with the characteristic translocation t(11;22)(q24;q12).
CONCLUSION
This tumor entity must be accurately distinguished from other renal neoplasms because of the prognostic and therapeutic impact.
引言
原始神经外胚层肿瘤（PNETs）主要发生于儿童期，好发于下肢软组织和脊柱旁区域。我们在此报告一例罕见的成人肾PNET病例。
病例介绍
一名46岁患有肠易激综合征的女性在常规体检时，经超声偶然发现右肾旁有一个肿瘤。患者无临床症状。腹部增强计算机断层扫描显示一个血管高度丰富的肾肿瘤。实施了肾整块切除的腹膜后切除术，组织病理学检查显示为具有特征性易位t(11;22)(q24;q12)的肾原始神经外胚层肿瘤。
结论
由于该肿瘤实体对预后和治疗有影响，必须将其与其他肾肿瘤准确区分开来。
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