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马蹄肾合并Wilms 瘤的畸胎瘤型:罕见病例报告。

Horseshoe kidney with teratoid type of Wilms tumor: a rare case report.

机构信息

Department of Pathology, School of Medicine, Iran University of Medical Sciences, Tehran, Iran.

Department of Pediatric, School of Medicine, Iran University of Medical Sciences, Tehran, Iran.

出版信息

BMC Nephrol. 2024 Aug 23;25(1):267. doi: 10.1186/s12882-024-03709-5.

Abstract

BACKGROUND

Horseshoe kidney is the most common renal fusion anomaly, and Wilms tumor is the most frequent renal malignancy in children. The occurrence of Wilms tumor in association with horseshoe kidney is a scarce anomaly. However, the arising of a teratoid type, which is a rare variant of Wilms tumor in a horseshoe kidney, is exceptionally unique.

CASE PRESENTATION

This report presents a 5-year-old male admitted with horseshoe kidney involved by a large heterogeneous calcified mass that was diagnose on biopsy as Wilms tumor blastemal dominant. According to the local and regional extension and metastatic tumor in the lungs, the patient underwent neoadjuvant chemotherapy and then surgery. Post-operative pathologic findings confirmed the diagnosis of teratoid Wilms tumor.

CONCLUSIONS

The occurrence of renal anomalies associated with a malignancy might be more frequent in the clinical environment. There are numerous differential diagnoses for renal tumors and masses, but the possibility of exceptional anomalies should not be denied, and clinicians should be prepared for these occasions. Although studies propose that chemotherapy has a trivial effect on teratoid Wilms tumors, it is essential to evaluate the tumor for any possibility of regression in non-teratoid regions before proceeding to upfront tumoral resection.

摘要

背景

马蹄肾是最常见的肾脏融合异常,而肾母细胞瘤是儿童最常见的肾脏恶性肿瘤。马蹄肾合并肾母细胞瘤的发生是一种罕见的异常。然而,在马蹄肾中发生的一种畸胎瘤型,是肾母细胞瘤的一种罕见变异,是极其独特的。

病例介绍

本报告介绍了一名 5 岁男性,因马蹄肾受累的大异质性钙化肿块就诊,活检诊断为Wilms 肿瘤成胚芽型为主。根据局部和区域扩展以及肺部的转移肿瘤,患者接受了新辅助化疗,然后进行了手术。术后病理检查结果证实了畸胎瘤 Wilms 肿瘤的诊断。

结论

在临床环境中,与恶性肿瘤相关的肾脏异常的发生可能更为频繁。肾肿瘤和肿块有许多鉴别诊断,但不应排除异常的可能性,临床医生应该为此做好准备。虽然研究表明化疗对畸胎瘤型 Wilms 肿瘤的影响微不足道,但在进行肿瘤切除前,必须评估肿瘤在非畸胎瘤区域是否有任何消退的可能。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a648/11342624/1e8f83f388af/12882_2024_3709_Fig1_HTML.jpg

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