Delahunt B, Beckwith J B, Eble J N, Fraundorfer M R, Sutton T D, Trotter G E
Department of Pathology, Wellington School of Medicine, University of Otago, Wellington South, New Zealand.
Cancer. 1998 Jun 15;82(12):2427-33. doi: 10.1002/(sici)1097-0142(19980615)82:12<2427::aid-cncr17>3.0.co;2-r.
Morphologic analysis of malignant renal tumors of childhood and adolescence has resulted in the identification of a variety of tumor types with characteristic histology and clinical behavior. The authors report a case of renal sarcoma in a 19-year-old male that differs in morphology from the various established categories of primitive renal tumors.
Sections taken from the nephrectomy specimen were stained by routine methods and by immunohistochemistry for stromal and epithelial markers, and for proliferation markers. In addition, ultrastructural studies were undertaken.
The tumor, which the authors termed cystic embryonal sarcoma, was comprised of poorly differentiated malignant mesenchyme in a myxoid stroma. Numerous epithelial-lined cysts were present. The distribution of the cysts and proliferation kinetics of cyst-lining epithelial cells suggested that they were derived from entrapped renal tubules. The tumor showed early recurrence postoperatively and after aggressive chemotherapy. The pathologic features and clinical behavior of the tumor resemble those of 2 previously reported cases and an additional 25 cases from the files of the National Wilms' Tumor Study Pathology Center.
The clinical and histologic features of cystic embryonal sarcoma differ from those of other renal tumors of childhood and adolescence, and the tumor appears to be a novel form of renal malignancy.
对儿童和青少年恶性肾肿瘤的形态学分析已识别出多种具有特征性组织学和临床行为的肿瘤类型。作者报告了1例19岁男性肾肉瘤病例，其形态与各种已确定的原发性肾肿瘤类别不同。
对肾切除标本切片进行常规染色及免疫组织化学染色，检测间质和上皮标志物以及增殖标志物。此外，还进行了超微结构研究。
作者将该肿瘤称为囊性胚胎性肉瘤，由黏液样基质中分化差的恶性间充质组成。存在大量内衬上皮的囊肿。囊肿的分布及囊肿内衬上皮细胞的增殖动力学表明它们源自被困的肾小管。该肿瘤术后及积极化疗后早期复发。该肿瘤的病理特征和临床行为与之前报告的2例病例以及国家肾母细胞瘤研究病理中心档案中的另外25例病例相似。
囊性胚胎性肉瘤的临床和组织学特征与儿童和青少年的其他肾肿瘤不同，该肿瘤似乎是一种新型肾恶性肿瘤。
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