畸胎瘤样 Wilms 瘤：一例罕见变异型病例报告，其在化疗期间可模拟侵袭性生物学行为。

Teratoid Wilms' tumor: case report of a rare variant that can mimic aggressive biology during chemotherapy.

机构信息

Department of Pathology, Mahidol University, Bangkok, Thailand.

出版信息

J Pediatr Surg. 2011 Dec;46(12):e1-6. doi: 10.1016/j.jpedsurg.2011.09.049.

DOI:10.1016/j.jpedsurg.2011.09.049

Abstract

Teratoid Wilms' tumor is considered by some as a variant of Wilms' tumor containing at least 50% heterologous differentiated tissue. Fewer than 30 cases have been described. We report a 9-month-old boy with bilateral Wilms' tumors who did not respond to multiagent chemotherapy and underwent right nephrectomy that showed a teratoid Wilms' tumor. The patient continued to survive despite cessation of treatment. The overall predominance of differentiated stromal elements in this subtype of Wilms' tumor might explain the poor response to chemotherapy yet generally favorable outcome. Recognition of this subtype on biopsy might justify earlier surgical intervention that, for bilateral tumors, might allow for greater nephron sparing.

摘要

胚胎性肾母细胞瘤被一些人认为是肾母细胞瘤的一种变体，含有至少 50%的异源性分化组织。目前已经描述了不到 30 例。我们报告了一例 9 个月大的男孩，患有双侧肾母细胞瘤，对多药化疗没有反应，并进行了右肾切除术，显示为胚胎性肾母细胞瘤。尽管停止了治疗，患者仍继续存活。这种亚型的肾母细胞瘤中分化基质成分的总体优势可能解释了对化疗的不良反应，但总体预后良好。在活检中识别出这种亚型可能证明更早的手术干预是合理的，对于双侧肿瘤，可能允许更多的肾单位保留。