First incidence of extrarenal wilms tumor within the spinal canal in the adult population: a novel case report and literature review

• Published in: BMC urology Vol. 24; no. 1; pp. 119 - 7
• Main Authors: Alijani, Babak, Abbaspour, Elahe, Karimzadhagh, Sahand, Reihanian, Zoheir, Haghani Dogahe, Mohammad, Jafari, Maryam, Jafari, Seifollah, Zaresharifi, Nooshin
• Format: Journal Article
• Language: English
• Published: England BioMed Central Ltd 10-06-2024; BioMed Central; BMC
• Subjects: Abdomen; Adults; Back pain; Backache; Case Report; Chemotherapy; Extra-renal nephroblastoma; Extrarenal Wilms tumor; Female; Females; Humans; Immunohistochemistry; Incidence; Kidney Neoplasms - diagnostic imaging; Kidney Neoplasms - pathology; Kidney Neoplasms - surgery; Kidneys; Literature reviews; Magnetic resonance imaging; Nephroblastoma; Online databases; Pain; Pediatrics; Socioeconomic factors; Spinal Canal - diagnostic imaging; Spinal Canal - pathology; Spinal cancer; Spinal cord; Spinal dysraphism; Spinal Neoplasms - diagnostic imaging; Spinal Neoplasms - surgery; Spinal tumors; Surgery; Tumors; Wilms tumor; Wilms Tumor - surgery; Young Adult; Extrarenal Wilms tumor; Nephroblastoma; Spinal dysraphism; Extra-renal nephroblastoma; Spinal tumors; Wilms tumor
• ISSN: 1471-2490; 1471-2490
• 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.