The St George Hospital, Kogarah, Sydney, Australia.
J Pediatr Surg. 2009 Dec;44(12):e13-6. doi: 10.1016/j.jpedsurg.2009.09.015.
INTRODUCTION/BACKGROUND: Ectopic nephrogenic rests (ENR) are rare. The incidental discovery of these lesions in children has particular clinicosurgical implications, especially given the association between ENR and the development of extrarenal Wilms' tumors (ERWT).
We reviewed the hospital records of patients with ERWT and ENR treated at our hospital over a 10-year period to identify those patients with histopathologically confirmed ENR and/or ERWT.
Ninety-five children with Wilms' tumor (WT) were identified, but only 1 case of ENR and ERWT. This patient was a 14-month-old boy who was incidentally found to have a mass in the left inguinal canal during orchiopexy. After histology, a provisional diagnosis of ENR was made. Six months later, the child went on to develop an ERWT at the same site. Periodic postsurgical follow-up has been uneventful.
This was the only case of ENR and ERWT in child in a 10-year review of patients with WT at our hospital. Our experience stresses the importance of including ENR in any working differential diagnosis of unexpected masses in the inguinal canal in children and underscores why careful long-term follow-up is mandatory. The reasons for the malignant transformation of ENR into primary ERWT are unknown, but our experience lends support for the theory that ENR are precursor lesions to the development of WT even in ectopic sites. The case also provides an example of the kind of technical difficulties presented by paratesticular masses during laparoscopy.
引言/背景:异位肾源性遗迹(ENR)很少见。这些病变在儿童中的偶然发现具有特殊的临床手术意义,特别是鉴于 ENR 与肾外 Wilms 瘤(ERWT)的发展之间存在关联。
我们回顾了我院 10 年来治疗 ERWT 和 ENR 患者的医院记录,以确定那些具有组织病理学证实的 ENR 和/或 ERWT 的患者。
共发现 95 例肾母细胞瘤(WT)患儿,但仅发现 1 例 ENR 和 ERWT。该患者为 14 个月大的男孩,在施行睾丸固定术时偶然发现左侧腹股沟管内有肿块。经过组织学检查,初步诊断为 ENR。6 个月后,患儿在同一部位发展为 ERWT。术后定期随访无异常。
这是我院 10 年来对 WT 患儿进行回顾性研究中唯一一例 ENR 和 ERWT 病例。我们的经验强调了在儿童腹股沟管意外肿块的任何鉴别诊断中包括 ENR 的重要性,并且强调了为什么需要进行仔细的长期随访。ENR 恶性转化为原发性 ERWT 的原因尚不清楚,但我们的经验支持这样一种理论,即即使在异位部位,ENR 也是 WT 发展的前体病变。该病例还提供了一个在腹腔镜检查中睾丸旁肿块带来的技术困难的例子。