PDF(Pubmed)
Abstract：
BACKGROUND: 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.
METHODS: 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.
CONCLUSIONS: 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.
METHODS: 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.
CONCLUSIONS: 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.
摘要：
背景：Wilms肿瘤（WT），也被称为肾母细胞瘤，在成年人中很少见，仅占所有肾母细胞瘤的3%或每百万个体0.2例。肾外Wilms肿瘤（ERWT）出现在肾脏边界之外，占所有WT病例的0.5％至1％，成年人的发病率更低。与异位肾病性休息（NR）相关的致癌突变可能有助于ERWT的发展。诊断包括手术切除和病理检查。由于病例稀少，成年人通常依赖儿科指南。我们彻底搜查了PubMed,Scopus,和WebofScience数据库来建立我们案例的独特性。据我们所知,这是在成人人群中首次有文献记载的椎管内肾外肾母细胞瘤的发病率.
方法：一名22岁女性，有先天性脂肪-脊髓膜膨出手术史，婴儿时出现6个月的背痛史。这种疼痛逐渐导致肢体无力，轻瘫,膀胱和肠道失控.MRI显示L4-S1水平有6×5×3cm的椎管肿块。因此,在L4-L5水平进行椎板切除术以切除髓内肿瘤.手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。
结论：本报告强调了成人肾外肾母细胞瘤（ERWT）的罕见性，挑战关于其典型发生年龄的传统假设。它强调了对此类罕见病例的临床认识的重要性。此外，脊髓ERWT的同时发生和脊髓异常的病史值得进一步调查.
方法：一名22岁女性，有先天性脂肪-脊髓膜膨出手术史，婴儿时出现6个月的背痛史。这种疼痛逐渐导致肢体无力，轻瘫,膀胱和肠道失控.MRI显示L4-S1水平有6×5×3cm的椎管肿块。因此,在L4-L5水平进行椎板切除术以切除髓内肿瘤.手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。
结论：本报告强调了成人肾外肾母细胞瘤（ERWT）的罕见性，挑战关于其典型发生年龄的传统假设。它强调了对此类罕见病例的临床认识的重要性。此外，脊髓ERWT的同时发生和脊髓异常的病史值得进一步调查.
