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一名成人睾丸旁原始神经外胚层肿瘤:病例报告及文献复习

A para-testicular primitive neuroectodermal tumor in an adult: a case report and literature review.

作者信息

Cohen D D, Zorn K, Bernard C, Rajan R, Kuzmarov I W

机构信息

McGill University Health Center, Montreal, Quebec, Canada.

出版信息

Can J Urol. 2000 Aug;7(4):1081-4.

Abstract

OBJECTIVE

The authors describe the salient clinical, radiologic and histopathologic features of an extremely rare para-testicular primitive neuroectodermal tumor in a 25 year-old man.

INTERVENTION

Excisional biopsy of the tumor en bloc was performed. Adjuvant VAdriaC-based chemotherapy (Vincristine, Doxorubicin, and Cyclophosphamide) was given post-operatively.

MAIN OUTCOME MEASURES

Histopathologic examination and immunohistochemical studies were performed on formaldehyde-fixed, paraffin-embedded tumor tissue.

RESULTS

Histologic examination showed an undifferentiated small cell tumor. The tumor cells stained positively with MIC-2, a marker specific for primitive neuroectodermal tumors. The patient is 12 months post surgery and has completed adjuvant chemotherapy with no evidence of recurrent disease.

CONCLUSIONS

This highly unusual, peripheral primitive neuroectodermal tumor should be considered in the differential diagnosis of undifferentiated small cell neoplasms of the genitourinary system in adults, from the kidney to the testicle. We present a patient with a PNET treated based on a Ewing's family of tumors protocol.

摘要

目的

作者描述了一名25岁男性极为罕见的睾丸旁原始神经外胚层肿瘤的显著临床、放射学和组织病理学特征。

干预措施

对肿瘤进行了整块切除活检。术后给予基于VAdriaC的辅助化疗(长春新碱、阿霉素和环磷酰胺)。

主要观察指标

对用甲醛固定、石蜡包埋的肿瘤组织进行组织病理学检查和免疫组化研究。

结果

组织学检查显示为未分化小细胞肿瘤。肿瘤细胞对MIC-2呈阳性染色,MIC-2是原始神经外胚层肿瘤的特异性标志物。患者术后12个月,已完成辅助化疗,无疾病复发迹象。

结论

在鉴别诊断成人从肾脏到睾丸的泌尿生殖系统未分化小细胞肿瘤时,应考虑这种极为罕见的外周原始神经外胚层肿瘤。我们报告了一名根据尤因氏肿瘤家族方案治疗的原始神经外胚层肿瘤患者。

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