Department of Neurosurgery, Poursina Hospital, Guilan University of Medical Sciences, Rasht, Iran.
Neuroscience Research Center, Guilan University of Medical Sciences, Rasht, Iran.
BMC Urol. 2024 Jun 10;24(1):119. doi: 10.1186/s12894-024-01508-6.
Wilms tumor (WT), also known as nephroblastoma, is rare in adults, accounting for merely 3% of all nephroblastomas or 0.2 cases per million individuals. Extrarenal Wilms tumor (ERWT) emerges outside the renal boundaries and comprises 0.5 to 1% of all WT cases, with even rarer incidences in adults. Oncogenic mutations associated with ectopic nephrogenic rests (NR) may contribute to ERWT development. Diagnosis involves surgical resection and pathology examination. Due to scarce cases, adults often rely on pediatric guidelines. We thoroughly searched PubMed, Scopus, and Web of Science databases to establish our case's uniqueness. To the best of our knowledge, this is the first documented incidence of extrarenal Wilms tumor within the spinal canal in the adult population.
A 22-year-old woman with a history of congenital lipo-myelomeningocele surgery as an infant presented with a 6-month history of back pain. This pain gradually resulted in limb weakness, paraparesis, and loss of bladder and bowel control. An MRI showed a 6 × 5 × 3 cm spinal canal mass at the L4-S1 level. Consequently, a laminectomy was performed at the L4-L5 level to remove the intramedullary tumor. Post-surgery histopathology and immunohistochemistry confirmed the tumor as ERWT with favorable histology without any teratomatous component.
This report underscores the rarity of extrarenal Wilms tumor (ERWT) in adults, challenging conventional assumptions about its typical age of occurrence. It emphasizes the importance of clinical awareness regarding such uncommon cases. Moreover, the co-occurrence of spinal ERWTs and a history of spinal anomalies warrants further investigation.
Wilms 瘤（WT），又称肾母细胞瘤，在成人中罕见，仅占所有肾母细胞瘤的 3%或每百万人中 0.2 例。肾外 Wilms 瘤（ERWT）出现在肾外边界，占所有 WT 病例的 0.5%至 1%，在成人中更为罕见。与异位肾源性遗迹（NR）相关的致癌突变可能导致 ERWT 的发生。诊断包括手术切除和病理检查。由于病例稀少，成人通常依赖儿科指南。我们彻底搜索了 PubMed、Scopus 和 Web of Science 数据库，以确定我们的病例的独特性。据我们所知，这是首例在成人椎管内发生的肾外 Wilms 瘤病例。
一名 22 岁女性，出生时患有先天性脂肪脊膜脊髓膨出，曾行手术治疗。她因背痛病史 6 个月就诊。这种疼痛逐渐导致下肢无力、截瘫和膀胱及肠道控制丧失。MRI 显示 L4-S1 水平椎管内有一个 6×5×3cm 的肿块。因此，在 L4-L5 水平行椎板切除术以切除髓内肿瘤。术后组织病理学和免疫组织化学证实肿瘤为 ERWT，组织学良好，无任何畸胎瘤成分。
本报告强调了成人肾外 Wilms 瘤（ERWT）的罕见性，挑战了其典型发病年龄的传统观念。它强调了对这种罕见病例保持临床意识的重要性。此外，脊髓 ERWT 与脊髓异常的共存需要进一步调查。
