Kawano N, Inayama Y, Nagashima Y, Miyagi Y, Uemura H, Saitoh K, Kubota Y, Hosaka M, Tanaka Y, Nakatani Y
Division of Anatomic and Surgical Pathology, Hospital of Yokohama City University, Yokohama, Japan.
Mod Pathol. 1999 Jul;12(7):729-34.
Desmoplastic small round-cell tumor typically occurs in the abdomen of young men, but it can also develop at other anatomic sites and in older people, presenting greater diagnostic difficulties. We report a case of this tumor arising from the paratesticular region in a 43-year-old man. The tumor showed histologic, immunohistochemical, and ultrastructural evidence of multilineage differentiation, including epithelial, mesenchymal, and neuronal features. In addition, the presence of an EWS and WT1 chimeric messenger RNA was demonstrated by the reverse transcriptase-polymerase chain reaction using an EWS exon 7 primer and WT1 exon 8 and exon 9 primers, which revealed single polymerase chain reaction products with a junction of EWS exon 7 to WT1 exon 8. Our study demonstrates that desmoplastic small round-cell tumors of the paratesticular region share not only morphologic but also molecular genetic features with those of the abdomen and that reverse transcriptase-polymerase chain reaction analysis using paraffin sections is useful for a conclusive diagnosis.
促结缔组织增生性小圆细胞肿瘤通常发生于年轻男性的腹部，但也可发生于其他解剖部位以及老年人，这会带来更大的诊断困难。我们报告一例发生于一名43岁男性睾丸旁区域的该肿瘤病例。该肿瘤显示出多谱系分化的组织学、免疫组化及超微结构证据，包括上皮、间充质和神经特征。此外，使用EWS外显子7引物以及WT1外显子8和外显子9引物通过逆转录聚合酶链反应证实了EWS和WT1嵌合信使核糖核酸的存在，其显示出单个聚合酶链反应产物，具有EWS外显子7与WT1外显子8的连接。我们的研究表明，睾丸旁区域的促结缔组织增生性小圆细胞肿瘤不仅与腹部的肿瘤具有形态学特征，还具有分子遗传学特征，并且使用石蜡切片进行逆转录聚合酶链反应分析有助于做出明确诊断。
