病例報告：擬似腎細胞癌的腎臟原發纖維性小圓細胞瘤
林柏廷、虞凱傑、莊正鏗、馮思中、林柏宏、邵翊紘、甘弘成、曲元正
林口長庚紀念醫院外科部 泌尿科系
Desmoplastic small round cell tumor of the kidney mimicking renal cell carcinoma: a case report
Po-Ting Lin, Kai-Jie Yu, Cheng-Keng Chuang, See-Tong Pang, Po-Hung Lin, I-Hung Shao, Hung-Cheng Kan, Yuan-Cheng Chu
Division of Urology, Department of Surgery, Chang Gung Memorial Hospital, Linkou medical center, Taoyuan, Taiwan
Background: Desmoplastic small round cell tumors (DSRCT), a rare neoplasm with aggressive manifestation and poor prognosis, were typically seen in children and adolescent males. It usually presents abdominal serosa involvement, and a kidney-based primary was extremely rare in the world. Since the clinical presentation was similar to those of other renal tumors, histopathological features such as polyphenotypic differentiation helped distinguish DSRCT from others. Further molecular studies were able to confirm the diagnosis. Here, we presented a case of primary DSRCT of the kidney mimicking renal cell carcinoma (RCC).
Case presentation: A 41-year-old male presents with intermittent right flank pain and hematuria. The abdominal computed tomography revealed a heterogenous enhancing tumor in the upper to posterior aspects of the right kidney with an invasion of the pelvicalyceal region, suggesting renal cell carcinoma. Radical nephrectomy was performed, with the pathologic result favors a desmoplastic small round cell tumor after further immunohistochemical study.
Conclusions: DSRCT had a much worse prognosis than RCC, and the correct diagnosis is crucial for a proper therapeutic approach. Current case reports were mostly focused on the pathologic features and relied on the immunohistochemical and molecular differences to confirm the diagnosis. The specific clinical and radiologic manifestation of DSRCT should be explored to guide the impression before an invasive intervention such as surgery.