Primitive neuroectodermal tumor of the kidney

被引:1
作者
Ellinger J. [1 ,3 ]
Bastian P.J. [1 ]
Hauser S. [1 ]
Biermann K. [2 ]
Müller S.C. [1 ]
机构
[1] Klinik und Poliklinik für Urologie, Rheinische Friedrich-Wilhelms Universität, Bonn
[2] Institut für Pathologie, Rheinische Friedrich-Wilhelms Universität, Bonn
[3] Klinik und Poliklinik für Urologie, Rheinische Friedrich-Wilhelms Universität, 53105 Bonn
来源
Der Urologe | 2006年 / 45卷 / 6期
关键词
Chemotherapy; Kidney; PNET; Primitive neuroectodermal tumor;
D O I
10.1007/s00120-006-1029-3
中图分类号
学科分类号
摘要
Primitive neuroectodermal Tumor (PNET) is a rare malignant tumor of young adult. Patients often present with the classical trias of renal cancer (pain, haematuria, palpable tumor). Specific radiological signs are missing, and therefore a PNET is often diagnosed postoperatively. It is characterized by the expression of MIC2, neural markers (Vimentin, S-100, Synaptophysin) and EWS/FLI1-translocation. The tumor is often diagnosed in advanced stage, and prognosis is poor despite of multimodal treatment including radical nephrectomy, polychemotherapy (vincristine, adriamycin, cyclophosphamid, etoposide, ifosfamide) and radiation in case of metastases or incomplete resection of primary tumor. We represent two cases of metastatic renal PNET in a 28- and 39-year-old patient. Due to the multimodal treatment a partial and a complete remission was achieved, respectively. © Springer Medizin Verlag 2006.
引用
收藏
页码:735 / 738
页数:3
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