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原发性肾脏尤因肉瘤/原始神经外胚层肿瘤。一种罕见的发现。

Primary Ewing's sarcoma/primitive neuroectodermal tumor of the kidney. An infrequent finding.

作者信息

Parada David, Godoy All, Liuzzi Francisco, Pelia Karla B, Romero Aramis, Parada Arelí M

机构信息

Department of Anatomic Pathology, Vargas Hospital, Caracas, Venezuela.

出版信息

Arch Esp Urol. 2007 Apr;60(3):321-5. doi: 10.4321/s0004-06142007000300020.

DOI:10.4321/s0004-06142007000300020
PMID:17601313
Abstract

OBJECTIVE

Ewing's sarcoma/Primitive neuroectodermal tumor (ES/PNET) is an extraordinarily rare primary tumor in the kidney. We re-port herein the clinical, histological, and immunohisto-chemical features of a primary renal ES/PNET.

METHODS

A 19-year old male referred a two weeks history of constant, colic, left flank pain, and fever. A left radical nephrectomy was performed. Gross pathologic examination showed pink-tan, lobulated solid tumor, localized at the superior pole.

RESULTS

Histologically, the tumor was solid with necrosis. The neoplastic cells showed a small amount of clear cytoplasm, and had vesicular nuclei with small nucleoli. Immunohistochemical studies showed strongly and diffusely positive staining for CD99 in a membranous pattern.

CONCLUSIONS

This case represents a typical ES/ PNET affecting a young male patient. Adequate diagnosis is important because this neoplasm has an aggressive behaviour.

摘要

目的

尤因肉瘤/原始神经外胚层肿瘤(ES/PNET)是一种极其罕见的原发性肾脏肿瘤。我们在此报告一例原发性肾ES/PNET的临床、组织学和免疫组织化学特征。

方法

一名19岁男性患者,有持续两周的绞痛性左腰痛和发热病史。行左肾根治性切除术。大体病理检查显示为粉红色至棕褐色、分叶状实性肿瘤,位于上极。

结果

组织学上,肿瘤为实性伴坏死。肿瘤细胞胞质少量清亮,核呈泡状,核仁小。免疫组织化学研究显示CD99呈强弥漫性膜性阳性染色。

结论

该病例代表了一例影响年轻男性患者的典型ES/PNET。由于这种肿瘤具有侵袭性,准确诊断很重要。

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