Ammani A, Ghadouane M, Hajji F, Janane A, Ameur A, Abbar M
Service d'urologie, hôpital militaire d'instruction Mohammed-V, Hay-Riad, 10101 Rabat, Maroc.
Prog Urol. 2009 Sep;19(8):579-81. doi: 10.1016/j.purol.2009.02.007. Epub 2009 Apr 3.
Primitive neuroectodermal tumors (PNETs) are rare and aggressive malignant small round cell sarcomas. Primitive urogenital location of PNETs is rare and occurs most frequently in the kidney. PNETs of the upper-urinary tract are exceptional (only one case reported in the literature). Its diagnosis is almost postoperative within pathological study of the operatory specimen, supported by immunohistochemistry and cytogenetics. Treatment is similar in all to that of Ewing's sarcoma and involves surgery, chemotherapy and radiotherapy. We report a new case of upper-urinary tract PNET and discuss the diagnostic and therapeutic problems posed by this particular tumor.
原始神经外胚层肿瘤（PNETs）是罕见且侵袭性强的恶性小圆细胞肉瘤。PNETs原发于泌尿生殖系统的情况罕见，最常发生于肾脏。上尿路的PNETs极为罕见（文献中仅报道过1例）。其诊断几乎均在对手术标本进行病理研究后得出，免疫组织化学和细胞遗传学检查可为诊断提供支持。其治疗方法总体上与尤因肉瘤相似，包括手术、化疗和放疗。我们报告1例新的上尿路PNET病例，并讨论这种特殊肿瘤所带来的诊断和治疗问题。
