First Incidence of Extrarenal Wilms Tumor within the Spinal Canal in the Adult Population: A Novel Case Report and Literature Review

First Incidence of Extrarenal Wilms Tumor within the Spinal Canal in the Adult Population: A Novel Case Report and Literature Review | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Case Report First Incidence of Extrarenal Wilms Tumor within the Spinal Canal in the Adult Population: A Novel Case Report and Literature Review Babak Alijani, Elahe Abbaspour, Sahand Karimzadhagh, Zoheir Reihanian, and 4 more This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-4242104/v1 This work is licensed under a CC BY 4.0 License Status: Published Journal Publication published 10 Jun, 2024 Read the published version in BMC Urology → Version 1 posted 14 You are reading this latest preprint version Abstract Background: Wilms tumor (WT), also known as nephroblastoma, is a rare occurrence in adults, accounting for merely 3% of all nephroblastomas or 0.2 cases per million individuals. Extrarenal Wilms tumor (ERWT) emerges outside the renal confines and comprises 0.5 to 1% of all WT cases. Although ERWT is more prevalent in children, its incidence in adults signifies an even rarer incidence. 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 conducted a thorough search on 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. Case presentation: 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 6x5x3 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. Conclusion: This report underscores a rare case of ERWT in an adult, challenging conventional diagnostics. Timely surgical intervention is recommended for similar masses, emphasizing the need for further research and clinical awareness. Wilms tumor Nephroblastoma Extrarenal Wilms tumor Extra-renal nephroblastoma Spinal dysraphism Spinal tumors Figures Figure 1 Figure 2 Figure 3 Introduction Wilms tumor (WT) or nephroblastoma occurrence in adults is uncommon; only 3% of all nephroblastomas are reported in adults, equating to 0.2 cases per million individuals ( 1 ). Extrarenal Wilms' tumor (ERWT) is an exceptionally scarce variant of Wilms' tumor, presenting outside the renal boundaries. While more prevalent in children, ERWT in adults signifies an even rarer incidence ( 2 ). ERWT occurrences within the spinal cord are exceptionally rare, with only a handful of documented cases reported, primarily in pediatric patients. To our knowledge, no prior instances of an intramedullary ERWT within the adult population have been reported. Remarkably, our case represents the first occurrence of such an entity, found in a 22-year-old female patient. The precise origins of ERWT's development still need to be fully understood, prompting the exploration of numerous hypotheses. The most widely embraced hypothesis proposes that ectopic nephrogenic rests (NR), aggregates of embryonal renal tissue persisting beyond the 35th week of development, can be attributed to the development of ERWTs through oncogenic mutation.Moreover, existing studies suggest that incomplete neural tube closure in the fourth week of embryonic life can hinder the migration of developing metanephric blastema, coinciding with the onset of metanephric kidney formation ( 3 , 4 ). Due to the limited reported cases, finding an optimal treatment for this tumor and predicting the prognosis is challenging. While childhood Wilms tumors have a high cure rate, outcomes in adults are less successful( 5 ). Here, we present the first incidence of a unique case involving a 22-year-old female with a history of lipo-myelomeningocele surgery during the neonatal period who presented with ERWT in the spine after 22 years. Case Presentation A 22-year-old female patient was admitted to the hospital with a history of back pain that had been ongoing for six months. Over time, her lower limb weakness progressively worsened, eventually leading to paraparesis and a loss of bladder and bowel control. Notably, the patient had a medical history of congenital lipo-myelomeningocele, which had been surgically repaired in infancy. On physical examination, her vital signs were stable; however, a neurological assessment revealed flaccid paralysis in both lower limbs, absent deep tendon reflexes, and sensory deficits in the lower limbs. Radiological assessment, which included spinal magnetic resonance imaging (MRI), identified a well-defined mass measuring 6x5x3 cm located within the spinal canal at the L4-S1 level. Considering the patient's history of myelomeningocele, the differential diagnosis included primary spinal cord tumors and secondary lesions. ( Fig. 1 ) Subsequently, the patient underwent a laminectomy at the L4-L5 level to surgically excise the intramedullary tumor, with the primary goals being the relief of spinal cord compression and the preservation of neurological function. The postoperative period proceeded without any notable complications. The postoperative histopathologic examination identified an extrarenal Wilms tumor displaying a favorable histological pattern. Distinctive histological features included a triphasic pattern characterized by 1. blastemal elements consisting of primitive cells with sporadic mitotic figures, 2. stromal components comprising spindle-shaped cells, and 3. small areas of epithelial tissue displaying glomeruloid and tubular structures. No signs of anaplasia or teratoma were observed. The immunohistochemistry study (IHC) confirmed the histologic diagnosis with a strong WT1 expression in the blastemal region. ( Fig. 2 ) Later on, additional imaging studies, including abdominal ultrasonography (USG), were conducted, revealing kidneys of normal size with a healthy cortex and sinus. However, both kidneys exhibited increased parenchymal thickness. Furthermore, a 4mm stone was detected in the mid-pole of the right kidney, with no signs of hydronephrosis. Aside from these findings, no other significant observations were made. Computed tomography (CT) scans of the chest, abdomen, and pelvis were also performed to exclude the possibility of a primary renal tumor or any tumor originating outside the kidneys. The results were normal and without any abnormalities. Regular follow-up assessments, including clinical examinations and imaging studies, were scheduled to monitor for any potential recurrence. Post-operative follow-up revealed a significant improvement in lower limb strength up to 3/5, both proximally and distally. However, the issue of incontinence remains unresolved at this time. The patient is currently undergoing further evaluation to determine the most appropriate approach for adjuvant therapy. Discussion We report a case of a 22-year-old female with a history of repaired lipo-myelomeningocele presented with progressive back pain and lower limb weakness. The subsequent MRI revealed an intramedullary tumor at L4-S1. Following laminectomy, tumor excision, and histopathological examination, a diagnosis of ERWT was confirmed. Postoperatively, despite persisting incontinence, lower limb strength improved significantly. Additionally, follow-up assessments ruled out any potential recurrences. Currently, regular monitoring continues for the unresolved incontinence issue. In approximately 90% of cases, the diagnosis is established before the age of 7, with most cases occurring before 5 ( 6 ). Therefore, to explore the rarity of our case, we conducted an extensive search across PubMed, Scopus, and Web of Science databases using specific keywords, including "Extrarenal," "ERWT," "Wilms," "Nephroblastoma," "spine," "spinal," "intraspinal," and "intramedullary." Our most recent search update on September 10, 2023, identified a total of 272 articles since inception. After excluding duplicates (82 articles), non-English articles, reviews, and cases involving non-human subjects mentioned solely in the article titles and abstracts, we narrowed our focus to case series and case reports. Thoroughly examining the full text of the remaining 45 studies, we identified 10 cases of spinal ERWTs. Based on the information available in the literature, our case presents a novel occurrence of extrarenal Wilms tumor within the spinal canal in an adult. Table 1 provides an overview of reported cases of ERWT within the spinal canal as documented in the existing literature. Table 1 Overview of ERWT cases reported within the spine in the literature. Author Patient Age Location Anomaly/Birth stigmata Outcome and follow-up Present Case Female 22-year-old L4-S1 Spinal dysraphism Unresolved incontinency Karim et al.( 27 ), 2022 Male 4-year-old T12-S3 Spinal dysraphism (posterior spina bifida) The patient died in the intensive care unit two years later. Tokuç et al.( 28 ), 2022 Female 3-year-old L2-S2 Dermal sinus and tethered cord diagnoses weakness in her right lower extremity (muscle strength 4/5)) Igbaseimokumo et al.( 23 ), 2017 Female Newborn L5 Occult dysraphism/ Dorsal Lipoma with hypertrichosis No recurrence at 1 years Wu et al.( 4 ), 2014 Male 9-month-old T7-L1 Vertebral malformation, meningomyelocele, diastematomyelia and tethered cord syndrome No recurrence at 6 months Sharma et al.( 29 ), 2009 Male 20-day-old L4-L5 Lumbar spina bifida/ tethered cord No recurrence at 9 months Deshpande et al.( 30 ), 2002 Male 1-year-old L2-L4 No previous stigmata Receiving treatment at the time of the report 3 months later Govender et al.( 31 ), 2000 Female 4-year-old T10 Spina bifida occulta Palliative care only Fahner et al.( 32 ), 1993 Female 2.5-year-old L5 Lipoma with skin dimple No recurrence at 2 years Mirkin et al.( 33 ), 1990 Female 4- year-old T12 - L4 Diastematomyelia /Spina Bifida with hypertrichosis Metastasis to cerebellum 1 year later Fernbach et al.( 34 ), 1984 Female 2- year-old L1 Diastematomyelia/ hypertrichosis No recurrence at 1 year ERWT was initially documented by Moyson et al. in 1961, representing approximately 0.5 to 1% of all Wilms tumor diagnoses ( 7 ). Adult WTs are rare and share histological characteristics with their pediatric counterparts. The treatment approach in adults and children is similar. However, there is a disparity in their behavior, as adult WTs tend to exhibit more aggressive behavior and show limited responses to treatment ( 8 ). Most ERWTs are diagnosed following surgical resection and pathological assessment. Since there are no distinguishing histological features between adult and pediatric Wilms' tumors, three diagnostic criteria have been established to identify ERWTs. First, it is essential to rule out a primary intrarenal tumor with secondary extrarenal metastases or a supernumerary kidney ( 9 – 11 ). Second, the pathologic assessment should reveal Wilms’ tumor’s characteristic triphasic histologic pattern. Lastly, a comprehensive examination of the entire tumor should demonstrate the absence of teratoma or renal carcinoma ( 12 ). [ Figure 3 ] Microscopic analysis typically reveals classical histological features characterized by a triphasic composition encompassing blastema, epithelial, and stromal areas. The blastema, which is the least differentiated component, comprises small, round blue cells with active mitotic activity and overlapping nuclei. This tends to be more prominent in adults and correlates with an unfavorable prognosis. The epithelial part shows variable differentiation, ranging from primitive tubular formations to structures resembling nephrogenesis at different stages. The stromal component may consist of densely packed mesenchymal cells or myxoid areas, which exhibit less aggressive behavior ( 13 , 14 ). To confirm the presence of ERWT, a thorough evaluation of the kidneys using multi-slice spiral CT images is essential to rule out any intrarenal tumors. A double-contrast CT scan is often recommended to ascertain the tumor's location and assess its resectability. MRI can also be advantageous, particularly for paraspinal and thoracic tumors, when there are symptoms of spinal cord compression ( 15 ). However, relying solely on these imaging modalities cannot provide a conclusive and definite diagnosis or accurately differentiate ERWT from other malignancies that may be considered in the differential diagnoses. These may include primary intrarenal tumors with metastasis to extrarenal sites, teratomas containing nephroblastoma elements, and various other primitive mesenchymal tumors, among others ( 16 , 17 ). Staging ERWTs is challenging, especially when following the National Wilms' Tumor Study (NWTS) guidelines. These guidelines recommend categorizing all such tumors as stage II or higher due to their location beyond the renal capsule, which necessitates chemotherapy for all patients. While treatment strategies align with those for renal Wilms' tumors, the unique locations and proximity to adjacent organs introduce distinctive considerations when planning surgical and adjuvant approaches ( 18 ). Managing these complex tumors requires a multidisciplinary approach, integrating diagnostic precision, surgical planning, intraoperative guidance, and postoperative care to address intricacies effectively ( 19 , 20 , 18 , 21 ). Nephrogenic rests (NR), originating from persistent nephrogenic blastema, are proposed as precursors to Wilms' tumor ( 22 ). The exact embryological origins of ectopic NRs remain elusive. However, studies indicate that approximately 67% of NRs located in the lumbosacral region are linked with spinal dysraphism. ( 3 ) It is hypothesized that these tumors may develop from NRs trapped between the dura and the developing spinal cord, possibly due to neural tube defects impeding renal tissue migration ( 23 ). While the exact pathogenesis remains debatable, the embryonic rest theory emerges as a plausible explanation, elucidating its unusual anatomical location. Due to the limited number of cases, standard management approaches for adults with this condition are lacking. In many specific cases, it is based on pediatric guidelines ( 24 ). Management protocols for this condition typically adhere to guidelines established by the NWTS and the Society of Paediatric Oncology (SIOP) in Europe. The NWTS approach advocates for surgery as the initial step, followed by adjuvant chemotherapy and radiotherapy based on surgical staging. In contrast, SIOP's approach involves universal neoadjuvant chemotherapy, followed by surgery and subsequent adjuvant therapies determined by staging. However, the effectiveness of these guidelines is still questioned due to the rarity of extrarenal adult Wilms' tumors ( 25 , 26 ). Conclusion In conclusion, this report highlights a unique case of ERWT in an adult, a rare occurrence that challenges conventional diagnostic approaches due to the absence of hallmark imaging features. Given the distinct nature of this case, we strongly recommend timely surgical intervention upon encountering unusual masses in comparable anatomical locations. In addition, this pioneering case highlights the crucial necessity for additional research and increased clinical awareness regarding ERWT. Declarations Ethics approval and Consent to participate This study received approval from the Institutional Review Board at Guilan University of Medical Sciences, with the ethics approval code IR.GUMS.REC.1402.369. Consent for publication Written informed consent was obtained from the patient to publish this case report and accompanying images. A copy of written consent is available for review by the editor-in-chief of this journal on request. Acknowledgment The authors created the illustration using www.biorender.com Availability of data and materials The data used to support the findings of this case report are available from the corresponding author upon request. Competing interests There are no competing interests. Funding None. Authors Contributions B.A. and E.A. co-authored in writing the manuscript. E.A. and S.K. wrote the first draft and collected data; M.H. and Z.R. conceived the idea and revised the manuscript, aided by input from S.J., M.J., and N.Z., who provided valuable feedback and contributed to the manuscript's refinement. References Mitry E, Ciccolallo L, Coleman MP, Gatta G, Pritchard-Jones K, EUROCARE Working Group. Incidence of and survival from Wilms’ tumour in adults in Europe: data from the EUROCARE study. Eur J Cancer Oxf Engl 1990. 2006;42(14):2363–8. Armanda V, Culić S, Pogorelić Z, Kuljiš D, Budimir D, Kuzmić-Prusac I. Rare localization of extrarenal nephroblastoma in 1-month-old female infant. 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Zaresharifi","email":"data:image/png;base64,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","orcid":"","institution":"Guilan University of Medical Sciences","correspondingAuthor":true,"prefix":"","firstName":"Nooshin","middleName":"","lastName":"Zaresharifi","suffix":""}],"badges":[],"createdAt":"2024-04-09 13:05:19","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-4242104/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-4242104/v1","draftVersion":[],"editorialEvents":[{"content":"https://doi.org/10.1186/s12894-024-01508-6","type":"published","date":"2024-06-10T14:49:21+00:00"}],"editorialNote":"","failedWorkflow":false,"files":[{"id":54929251,"identity":"f040c752-4752-42ac-977f-cf2e184c368c","added_by":"auto","created_at":"2024-04-18 17:46:44","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":1223297,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cem\u003e\u003cstrong\u003e(a)\u003c/strong\u003e\u003c/em\u003e\u003cem\u003e T1-weighted lumbar MRI image showed a well-defined hypointense mass (arrow) within the spinal canal at the L4-S1 level. \u003c/em\u003e\u003cem\u003e\u003cstrong\u003e(b)\u003c/strong\u003e\u003c/em\u003e\u003cem\u003e In the sagittal T2-weighted MRI image, the lesion measuring 6x5x3 cm appeared hyperintense.\u003c/em\u003e\u003c/p\u003e","description":"","filename":"1.png","url":"https://assets-eu.researchsquare.com/files/rs-4242104/v1/69628868aeff795cbc068318.png"},{"id":54928437,"identity":"a548b779-b029-4fe1-a3e7-09569f718dd8","added_by":"auto","created_at":"2024-04-18 17:38:44","extension":"png","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":3561427,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cem\u003e\u003cstrong\u003e(a)\u003c/strong\u003e\u003c/em\u003e\u003cem\u003e Microscopic examination showing triphasic histology (H\u0026amp;E×100) (\u003c/em\u003e\u003cem\u003e\u003cstrong\u003eb) 1- \u003c/strong\u003e\u003c/em\u003e\u003cem\u003eepithelial component with glomeruloid and tubular structures (H\u0026amp;E×400) (\u003c/em\u003e\u003cem\u003e\u003cstrong\u003ec)\u003c/strong\u003e\u003c/em\u003e\u003cem\u003e \u003c/em\u003e\u003cem\u003e\u003cstrong\u003e2\u003c/strong\u003e\u003c/em\u003e\u003cem\u003e- Blastema component in the stromal background featuring primitive round/oval blastomatous cells and \u003c/em\u003e\u003cem\u003e\u003cstrong\u003e3-\u003c/strong\u003e\u003c/em\u003e\u003cem\u003espindle-shaped stromal cells, without any teratomatous elements (H \u0026amp; E, 100x). (\u003c/em\u003e\u003cem\u003e\u003cstrong\u003ed)\u003c/strong\u003e\u003c/em\u003e\u003cem\u003e within the same specimen at a higher magnification (H \u0026amp; E, 400x) \u003c/em\u003e\u003cem\u003e\u003cstrong\u003e(e, f)\u003c/strong\u003e\u003c/em\u003e\u003cem\u003e WT-1 positive reaction of the blastema component, immunohistochemical staining (×100 \u0026amp; ×400)\u003c/em\u003e\u003c/p\u003e","description":"","filename":"2.png","url":"https://assets-eu.researchsquare.com/files/rs-4242104/v1/d8bc639289a91d0206aa089b.png"},{"id":54928435,"identity":"5bdf8554-48ef-42ab-891f-1400e8f58dd0","added_by":"auto","created_at":"2024-04-18 17:38:44","extension":"png","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":439220,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cem\u003eSchematic Presentation of normal kidney development and tumourigenesis of Wilms tumor. The Classical histopathological feature is the triphasic pattern comprised of Blastemal, epithelial, and stromal components.\u003c/em\u003e\u003c/p\u003e","description":"","filename":"3.png","url":"https://assets-eu.researchsquare.com/files/rs-4242104/v1/014ded5ff6e0a9e9cbcbfc31.png"},{"id":58822138,"identity":"cdd57a0b-23be-4683-9687-d2ca730f80a8","added_by":"auto","created_at":"2024-06-21 16:32:52","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":5263186,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-4242104/v1/038419e2-f329-4cff-bf2b-0b91f438ad85.pdf"},{"id":54928438,"identity":"dc4cbbae-a7eb-412d-9560-ff38a848e407","added_by":"auto","created_at":"2024-04-18 17:38:44","extension":"docx","order_by":6,"title":"","display":"","copyAsset":false,"role":"supplement","size":14454,"visible":true,"origin":"","legend":"","description":"","filename":"Highlights.docx","url":"https://assets-eu.researchsquare.com/files/rs-4242104/v1/96e8923d40e367ca069e6612.docx"}],"financialInterests":"No competing interests reported.","formattedTitle":"First Incidence of Extrarenal Wilms Tumor within the Spinal Canal in the Adult Population: A Novel Case Report and Literature Review","fulltext":[{"header":"Introduction","content":"\u003cp\u003eWilms tumor (WT) or nephroblastoma occurrence in adults is uncommon; only 3% of all nephroblastomas are reported in adults, equating to 0.2 cases per million individuals (\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e). Extrarenal Wilms' tumor (ERWT) is an exceptionally scarce variant of Wilms' tumor, presenting outside the renal boundaries. While more prevalent in children, ERWT in adults signifies an even rarer incidence (\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e). ERWT occurrences within the spinal cord are exceptionally rare, with only a handful of documented cases reported, primarily in pediatric patients. To our knowledge, no prior instances of an intramedullary ERWT within the adult population have been reported. Remarkably, our case represents the first occurrence of such an entity, found in a 22-year-old female patient.\u003c/p\u003e \u003cp\u003eThe precise origins of ERWT's development still need to be fully understood, prompting the exploration of numerous hypotheses. The most widely embraced hypothesis proposes that ectopic nephrogenic rests (NR), aggregates of embryonal renal tissue persisting beyond the 35th week of development, can be attributed to the development of ERWTs through oncogenic mutation.Moreover, existing studies suggest that incomplete neural tube closure in the fourth week of embryonic life can hinder the migration of developing metanephric blastema, coinciding with the onset of metanephric kidney formation (\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e, \u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e). Due to the limited reported cases, finding an optimal treatment for this tumor and predicting the prognosis is challenging. While childhood Wilms tumors have a high cure rate, outcomes in adults are less successful(\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e). Here, we present the first incidence of a unique case involving a 22-year-old female with a history of lipo-myelomeningocele surgery during the neonatal period who presented with ERWT in the spine after 22 years.\u003c/p\u003e"},{"header":"Case Presentation","content":"\u003cp\u003eA 22-year-old female patient was admitted to the hospital with a history of back pain that had been ongoing for six months. Over time, her lower limb weakness progressively worsened, eventually leading to paraparesis and a loss of bladder and bowel control. Notably, the patient had a medical history of congenital lipo-myelomeningocele, which had been surgically repaired in infancy. On physical examination, her vital signs were stable; however, a neurological assessment revealed flaccid paralysis in both lower limbs, absent deep tendon reflexes, and sensory deficits in the lower limbs.\u003c/p\u003e \u003cp\u003eRadiological assessment, which included spinal magnetic resonance imaging (MRI), identified a well-defined mass measuring 6x5x3 cm located within the spinal canal at the L4-S1 level. Considering the patient's history of myelomeningocele, the differential diagnosis included primary spinal cord tumors and secondary lesions. \u003cb\u003e(\u003c/b\u003eFig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003e\u003cb\u003e)\u003c/b\u003e\u003c/p\u003e \u003cp\u003eSubsequently, the patient underwent a laminectomy at the L4-L5 level to surgically excise the intramedullary tumor, with the primary goals being the relief of spinal cord compression and the preservation of neurological function. The postoperative period proceeded without any notable complications.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003eThe postoperative histopathologic examination identified an extrarenal Wilms tumor displaying a favorable histological pattern. Distinctive histological features included a triphasic pattern characterized by 1. blastemal elements consisting of primitive cells with sporadic mitotic figures, 2. stromal components comprising spindle-shaped cells, and 3. small areas of epithelial tissue displaying glomeruloid and tubular structures. No signs of anaplasia or teratoma were observed. The immunohistochemistry study (IHC) confirmed the histologic diagnosis with a strong WT1 expression in the blastemal region. \u003cb\u003e(\u003c/b\u003eFig.\u0026nbsp;\u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e2\u003c/span\u003e\u003cb\u003e)\u003c/b\u003e\u003c/p\u003e \u003cp\u003eLater on, additional imaging studies, including abdominal ultrasonography (USG), were conducted, revealing kidneys of normal size with a healthy cortex and sinus. However, both kidneys exhibited increased parenchymal thickness. Furthermore, a 4mm stone was detected in the mid-pole of the right kidney, with no signs of hydronephrosis. Aside from these findings, no other significant observations were made.\u003c/p\u003e \u003cp\u003eComputed tomography (CT) scans of the chest, abdomen, and pelvis were also performed to exclude the possibility of a primary renal tumor or any tumor originating outside the kidneys. The results were normal and without any abnormalities.\u003c/p\u003e \u003cp\u003eRegular follow-up assessments, including clinical examinations and imaging studies, were scheduled to monitor for any potential recurrence. Post-operative follow-up revealed a significant improvement in lower limb strength up to 3/5, both proximally and distally. However, the issue of incontinence remains unresolved at this time. The patient is currently undergoing further evaluation to determine the most appropriate approach for adjuvant therapy.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003eWe report a case of a 22-year-old female with a history of repaired lipo-myelomeningocele presented with progressive back pain and lower limb weakness. The subsequent MRI revealed an intramedullary tumor at L4-S1. Following laminectomy, tumor excision, and histopathological examination, a diagnosis of ERWT was confirmed. Postoperatively, despite persisting incontinence, lower limb strength improved significantly. Additionally, follow-up assessments ruled out any potential recurrences. Currently, regular monitoring continues for the unresolved incontinence issue.\u003c/p\u003e\n\u003cp\u003eIn approximately 90% of cases, the diagnosis is established before the age of 7, with most cases occurring before 5 (\u003cspan class=\"CitationRef\"\u003e6\u003c/span\u003e). Therefore, to explore the rarity of our case, we conducted an extensive search across PubMed, Scopus, and Web of Science databases using specific keywords, including \u0026quot;Extrarenal,\u0026quot; \u0026quot;ERWT,\u0026quot; \u0026quot;Wilms,\u0026quot; \u0026quot;Nephroblastoma,\u0026quot; \u0026quot;spine,\u0026quot; \u0026quot;spinal,\u0026quot; \u0026quot;intraspinal,\u0026quot; and \u0026quot;intramedullary.\u0026quot; Our most recent search update on September 10, 2023, identified a total of 272 articles since inception. After excluding duplicates (82 articles), non-English articles, reviews, and cases involving non-human subjects mentioned solely in the article titles and abstracts, we narrowed our focus to case series and case reports. Thoroughly examining the full text of the remaining 45 studies, we identified 10 cases of spinal ERWTs. Based on the information available in the literature, our case presents a novel occurrence of extrarenal Wilms tumor within the spinal canal in an adult. Table \u003cspan class=\"InternalRef\"\u003e1\u003c/span\u003e provides an overview of reported cases of ERWT within the spinal canal as documented in the existing literature.\u003c/p\u003e\n\u003cdiv class=\"gridtable\"\u003e\u0026nbsp;\u003c/div\u003e\n\u003cdiv class=\"gridtable\"\u003e\n \u003cdiv class=\"colspec\" align=\"left\"\u003e\u0026nbsp;\u003c/div\u003e\n \u003ctable id=\"Tab2\" border=\"1\"\u003e\n \u003ccaption\u003e\n \u003cdiv class=\"CaptionNumber\"\u003eTable 1\u003c/div\u003e\n \u003cdiv class=\"CaptionContent\"\u003e\n \u003cp\u003eOverview of ERWT cases reported within the spine in the literature.\u003c/p\u003e\n \u003c/div\u003e\n \u003c/caption\u003e\n \u003cthead\u003e\n \u003ctr\u003e\n \u003cth align=\"left\"\u003e\n \u003cp\u003eAuthor\u003c/p\u003e\n \u003c/th\u003e\n \u003cth align=\"left\"\u003e\n \u003cp\u003ePatient\u003c/p\u003e\n \u003c/th\u003e\n \u003cth align=\"left\"\u003e\n \u003cp\u003eAge\u003c/p\u003e\n \u003c/th\u003e\n \u003cth align=\"left\"\u003e\n \u003cp\u003eLocation\u003c/p\u003e\n \u003c/th\u003e\n \u003cth align=\"left\"\u003e\n \u003cp\u003eAnomaly/Birth stigmata\u003c/p\u003e\n \u003c/th\u003e\n \u003cth align=\"left\"\u003e\n \u003cp\u003eOutcome and follow-up\u003c/p\u003e\n \u003c/th\u003e\n \u003c/tr\u003e\n \u003c/thead\u003e\n \u003ctbody\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003ePresent Case\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eFemale\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e22-year-old\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eL4-S1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eSpinal dysraphism\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eUnresolved incontinency\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eKarim et al.(\u003cspan class=\"CitationRef\"\u003e27\u003c/span\u003e),\u003c/p\u003e\n \u003cp\u003e2022\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eMale\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e4-year-old\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eT12-S3\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eSpinal dysraphism (posterior spina bifida)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eThe patient died in the intensive care unit two years later.\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eToku\u0026ccedil; et al.(\u003cspan class=\"CitationRef\"\u003e28\u003c/span\u003e),\u003c/p\u003e\n \u003cp\u003e2022\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eFemale\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e3-year-old\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eL2-S2\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eDermal sinus and tethered cord diagnoses\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eweakness in her right lower extremity (muscle strength 4/5))\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eIgbaseimokumo et al.(\u003cspan class=\"CitationRef\"\u003e23\u003c/span\u003e), 2017\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eFemale\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eNewborn\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eL5\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eOccult dysraphism/ Dorsal Lipoma with hypertrichosis\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eNo recurrence at 1 years\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eWu et al.(\u003cspan class=\"CitationRef\"\u003e4\u003c/span\u003e),\u003c/p\u003e\n \u003cp\u003e2014\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eMale\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e9-month-old\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eT7-L1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eVertebral malformation, meningomyelocele, diastematomyelia and tethered cord syndrome\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eNo recurrence at 6 months\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eSharma et al.(\u003cspan class=\"CitationRef\"\u003e29\u003c/span\u003e),\u003c/p\u003e\n \u003cp\u003e2009\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eMale\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e20-day-old\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eL4-L5\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eLumbar spina bifida/ tethered cord\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eNo recurrence at 9 months\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eDeshpande et al.(\u003cspan class=\"CitationRef\"\u003e30\u003c/span\u003e),\u003c/p\u003e\n \u003cp\u003e2002\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eMale\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e1-year-old\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eL2-L4\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eNo previous stigmata\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eReceiving treatment at the time of the report\u003c/p\u003e\n \u003cp\u003e3 months later\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eGovender et al.(\u003cspan class=\"CitationRef\"\u003e31\u003c/span\u003e),\u003c/p\u003e\n \u003cp\u003e2000\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eFemale\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e4-year-old\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eT10\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eSpina bifida occulta\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003ePalliative care only\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eFahner et al.(\u003cspan class=\"CitationRef\"\u003e32\u003c/span\u003e),\u003c/p\u003e\n \u003cp\u003e1993\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eFemale\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e2.5-year-old\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eL5\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eLipoma with skin dimple\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eNo recurrence at 2 years\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eMirkin et al.(\u003cspan class=\"CitationRef\"\u003e33\u003c/span\u003e),\u003c/p\u003e\n \u003cp\u003e1990\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eFemale\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e4- year-old\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eT12 - L4\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eDiastematomyelia /Spina Bifida with hypertrichosis\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eMetastasis to cerebellum 1 year later\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eFernbach et al.(\u003cspan class=\"CitationRef\"\u003e34\u003c/span\u003e),\u003c/p\u003e\n \u003cp\u003e1984\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eFemale\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e2- year-old\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eL1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eDiastematomyelia/ hypertrichosis\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eNo recurrence at 1 year\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003c/tbody\u003e\n \u003c/table\u003e\n\u003c/div\u003e\n\u003cp\u003eERWT was initially documented by Moyson et al. in 1961, representing approximately 0.5 to 1% of all Wilms tumor diagnoses (\u003cspan class=\"CitationRef\"\u003e7\u003c/span\u003e). Adult WTs are rare and share histological characteristics with their pediatric counterparts. The treatment approach in adults and children is similar. However, there is a disparity in their behavior, as adult WTs tend to exhibit more aggressive behavior and show limited responses to treatment (\u003cspan class=\"CitationRef\"\u003e8\u003c/span\u003e). Most ERWTs are diagnosed following surgical resection and pathological assessment. Since there are no distinguishing histological features between adult and pediatric Wilms\u0026apos; tumors, three diagnostic criteria have been established to identify ERWTs. First, it is essential to rule out a primary intrarenal tumor with secondary extrarenal metastases or a supernumerary kidney (\u003cspan class=\"CitationRef\"\u003e9\u003c/span\u003e\u0026ndash;\u003cspan class=\"CitationRef\"\u003e11\u003c/span\u003e). Second, the pathologic assessment should reveal Wilms\u0026rsquo; tumor\u0026rsquo;s characteristic triphasic histologic pattern. Lastly, a comprehensive examination of the entire tumor should demonstrate the absence of teratoma or renal carcinoma (\u003cspan class=\"CitationRef\"\u003e12\u003c/span\u003e).\u003cstrong\u003e[\u003c/strong\u003eFigure\u003cspan class=\"InternalRef\"\u003e3\u003c/span\u003e\u003cstrong\u003e]\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eMicroscopic analysis typically reveals classical histological features characterized by a triphasic composition encompassing blastema, epithelial, and stromal areas. The blastema, which is the least differentiated component, comprises small, round blue cells with active mitotic activity and overlapping nuclei. This tends to be more prominent in adults and correlates with an unfavorable prognosis. The epithelial part shows variable differentiation, ranging from primitive tubular formations to structures resembling nephrogenesis at different stages. The stromal component may consist of densely packed mesenchymal cells or myxoid areas, which exhibit less aggressive behavior (\u003cspan class=\"CitationRef\"\u003e13\u003c/span\u003e, \u003cspan class=\"CitationRef\"\u003e14\u003c/span\u003e).\u003c/p\u003e\n\u003cp\u003eTo confirm the presence of ERWT, a thorough evaluation of the kidneys using multi-slice spiral CT images is essential to rule out any intrarenal tumors. A double-contrast CT scan is often recommended to ascertain the tumor\u0026apos;s location and assess its resectability. MRI can also be advantageous, particularly for paraspinal and thoracic tumors, when there are symptoms of spinal cord compression (\u003cspan class=\"CitationRef\"\u003e15\u003c/span\u003e). However, relying solely on these imaging modalities cannot provide a conclusive and definite diagnosis or accurately differentiate ERWT from other malignancies that may be considered in the differential diagnoses. These may include primary intrarenal tumors with metastasis to extrarenal sites, teratomas containing nephroblastoma elements, and various other primitive mesenchymal tumors, among others (\u003cspan class=\"CitationRef\"\u003e16\u003c/span\u003e, \u003cspan class=\"CitationRef\"\u003e17\u003c/span\u003e).\u003c/p\u003e\n\u003cp\u003eStaging ERWTs is challenging, especially when following the National Wilms\u0026apos; Tumor Study (NWTS) guidelines. These guidelines recommend categorizing all such tumors as stage II or higher due to their location beyond the renal capsule, which necessitates chemotherapy for all patients. While treatment strategies align with those for renal Wilms\u0026apos; tumors, the unique locations and proximity to adjacent organs introduce distinctive considerations when planning surgical and adjuvant approaches (\u003cspan class=\"CitationRef\"\u003e18\u003c/span\u003e). Managing these complex tumors requires a multidisciplinary approach, integrating diagnostic precision, surgical planning, intraoperative guidance, and postoperative care to address intricacies effectively (\u003cspan class=\"CitationRef\"\u003e19\u003c/span\u003e, \u003cspan class=\"CitationRef\"\u003e20\u003c/span\u003e, \u003cspan class=\"CitationRef\"\u003e18\u003c/span\u003e, \u003cspan class=\"CitationRef\"\u003e21\u003c/span\u003e).\u003c/p\u003e\n\u003cp\u003eNephrogenic rests (NR), originating from persistent nephrogenic blastema, are proposed as precursors to Wilms\u0026apos; tumor (\u003cspan class=\"CitationRef\"\u003e22\u003c/span\u003e). The exact embryological origins of ectopic NRs remain elusive. However, studies indicate that approximately 67% of NRs located in the lumbosacral region are linked with spinal dysraphism. (\u003cspan class=\"CitationRef\"\u003e3\u003c/span\u003e) It is hypothesized that these tumors may develop from NRs trapped between the dura and the developing spinal cord, possibly due to neural tube defects impeding renal tissue migration (\u003cspan class=\"CitationRef\"\u003e23\u003c/span\u003e). While the exact pathogenesis remains debatable, the embryonic rest theory emerges as a plausible explanation, elucidating its unusual anatomical location.\u003c/p\u003e\n\u003cp\u003eDue to the limited number of cases, standard management approaches for adults with this condition are lacking. In many specific cases, it is based on pediatric guidelines (\u003cspan class=\"CitationRef\"\u003e24\u003c/span\u003e). Management protocols for this condition typically adhere to guidelines established by the NWTS and the Society of Paediatric Oncology (SIOP) in Europe. The NWTS approach advocates for surgery as the initial step, followed by adjuvant chemotherapy and radiotherapy based on surgical staging. In contrast, SIOP\u0026apos;s approach involves universal neoadjuvant chemotherapy, followed by surgery and subsequent adjuvant therapies determined by staging. However, the effectiveness of these guidelines is still questioned due to the rarity of extrarenal adult Wilms\u0026apos; tumors (\u003cspan class=\"CitationRef\"\u003e25\u003c/span\u003e, \u003cspan class=\"CitationRef\"\u003e26\u003c/span\u003e).\u003c/p\u003e"},{"header":"Conclusion","content":"\u003cp\u003eIn conclusion, this report highlights a unique case of ERWT in an adult, a rare occurrence that challenges conventional diagnostic approaches due to the absence of hallmark imaging features. Given the distinct nature of this case, we strongly recommend timely surgical intervention upon encountering unusual masses in comparable anatomical locations. In addition, this pioneering case highlights the crucial necessity for additional research and increased clinical awareness regarding ERWT.\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003eEthics approval and Consent to participate\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThis study received approval from the Institutional Review Board at Guilan University of Medical Sciences, with the ethics approval code IR.GUMS.REC.1402.369.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConsent for publication\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eWritten informed consent was obtained from the patient to publish this case report and accompanying images. A copy of written consent is available for review by the editor-in-chief of this journal on request.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAcknowledgment\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe authors created the illustration using www.biorender.com\u003cbr\u003e\u0026nbsp;\u003cstrong\u003eAvailability of data and materials\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe data used to support the findings of this case report are available from the corresponding author upon request.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCompeting interests\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThere are no competing interests.\u003cbr\u003e\u003cstrong\u003eFunding\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eNone.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAuthors Contributions\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eB.A. and E.A. co-authored in writing the manuscript. E.A. and S.K. wrote the first draft and collected data; M.H. and Z.R. conceived the idea and revised the manuscript, aided by input from S.J., M.J., and N.Z., who provided valuable feedback and contributed to the manuscript\u0026apos;s refinement.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\u003cli\u003e\u003cspan\u003eMitry E, Ciccolallo L, Coleman MP, Gatta G, Pritchard-Jones K, EUROCARE Working Group. Incidence of and survival from Wilms\u0026rsquo; tumour in adults in Europe: data from the EUROCARE study. Eur J Cancer Oxf Engl 1990. 2006;42(14):2363\u0026ndash;8.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eArmanda V, Culić S, Pogorelić Z, Kuljiš D, Budimir D, Kuzmić-Prusac I. Rare localization of extrarenal nephroblastoma in 1-month-old female infant. J Pediatr Urol. 2012;8(4):e43\u0026ndash;45.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eSteelman CK, Bannister LL, Palmer M, Chang TS, Elawabdeh N, Shehata BM. 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AME Med J [Internet]. 2019 Nov 26 [cited 2023 Nov 19];4(0). \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://amj.amegroups.org/article/view/5250\u003c/span\u003e\u003cspan address=\"https://amj.amegroups.org/article/view/5250\" targettype=\"URL\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eKarim A, Shaikhyzada K, Abulkhanova N, Altyn A, Ibraimov B, Nurgaliyev D, et al. Pediatric Extra-Renal Nephroblastoma (Wilms\u0026rsquo; Tumor): A Systematic Case-Based Review. Cancers. 2023;15(9):2563.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eToku\u0026ccedil; AG, Eker N, Tufan Taş B, Uyar Bozkurt S, Sakar M, Aras S, et al. An Astonishing Extrarenal Wilms Localisation; Spinal Cord. J Pediatr Res. 2022;9(2):188\u0026ndash;91.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eSharma MC, Jain D, Sarkar C, Bhatnagar V, Rishi A, Suri V, et al. Lumbosacral Wilms\u0026rsquo; tumor as a component of immature teratoma associated with spinal dysraphism\u0026ndash;a rare case and short literature review. Fetal Pediatr Pathol. 2009;28(5):201\u0026ndash;8.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eDeshpande AV, Gawali JS, Sanghani HH, Shenoy AS, Patankar JZ, Borwankar SS. Extrarenal Wilm\u0026rsquo;s tumour - a rare entity. Pediatr Surg Int. 2002;18(5\u0026ndash;6):543\u0026ndash;4.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eGovender D, Hadley GP, Nadvi SS, Donnellan RB. Primary lumbosacral Wilms tumour associated with occult spinal dysraphism. Virchows Arch Int J Pathol. 2000;436(5):502\u0026ndash;5.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eFahner JB, Switzer R, Freyer DR, Mann JD, Mann RJ. Extrarenal Wilms\u0026rsquo; tumor. Unusual presentation in the lumbosacral region. Am J Pediatr Hematol Oncol. 1993;15(1):117\u0026ndash;9.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eMirkin LD, Azzarelli B, Seo IS. Extrarenal Wilms\u0026rsquo; Tumor with Cerebellar Metastasis in a Four-Year-Old Girl with Spina Bifida. Am J Clin Pathol. 1990;93(6):805\u0026ndash;9.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eFernbach SK, Naidich TP, McLone DG, Leestma JE. Computed tomography of primary intrathecal Wilms tumor with diastematomyelia. J Comput Assist Tomogr. 1984;8(3):523\u0026ndash;8.\u003c/span\u003e\u003c/li\u003e\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":false,"highlight":"","institution":"","isAcceptedByJournal":true,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"bmc-urology","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"buro","sideBox":"Learn more about [BMC Urology](http://bmcurol.biomedcentral.com/)","snPcode":"","submissionUrl":"https://www.editorialmanager.com/buro/default.aspx","title":"BMC Urology","twitterHandle":"BMC_series","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"em","reportingPortfolio":"BMC Series","inReviewEnabled":true,"inReviewRevisionsEnabled":true},"keywords":"Wilms tumor, Nephroblastoma, Extrarenal Wilms tumor, Extra-renal nephroblastoma, Spinal dysraphism, Spinal tumors","lastPublishedDoi":"10.21203/rs.3.rs-4242104/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-4242104/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003e\u003cstrong\u003eBackground: \u003c/strong\u003eWilms tumor (WT), also known as nephroblastoma, is a rare occurrence in adults, accounting for merely 3% of all nephroblastomas or 0.2 cases per million individuals. Extrarenal Wilms tumor (ERWT) emerges outside the renal confines and comprises 0.5 to 1% of all WT cases. Although ERWT is more prevalent in children, its incidence in adults signifies an even rarer incidence. 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.\u003cbr\u003e\nWe conducted a thorough search on 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.\u003cbr\u003e\n\u003cstrong\u003eCase presentation:\u003c/strong\u003e 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 6x5x3 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.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConclusion:\u003c/strong\u003e This report underscores a rare case of ERWT in an adult, challenging conventional diagnostics. Timely surgical intervention is recommended for similar masses, emphasizing the need for further research and clinical awareness.\u003c/p\u003e","manuscriptTitle":"First Incidence of Extrarenal Wilms Tumor within the Spinal Canal in the Adult Population: A Novel Case Report and Literature Review","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2024-04-18 17:38:40","doi":"10.21203/rs.3.rs-4242104/v1","editorialEvents":[{"type":"communityComments","content":0},{"type":"decision","content":"Revision requested","date":"2024-04-22T13:20:24+00:00","index":"","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2024-04-21T19:52:31+00:00","index":"hide","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2024-04-20T17:47:02+00:00","index":"hide","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2024-04-18T17:37:29+00:00","index":"hide","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2024-04-15T21:51:02+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"13344d93-a1bd-47ab-90b4-f0d7ff3349b3","date":"2024-04-13T13:00:36+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"56f931e1-ed1d-44f8-bde9-79a57f8f6806","date":"2024-04-12T16:19:04+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"91f9ae10-c695-4963-b020-9b053ff2de0a","date":"2024-04-12T14:10:19+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"44bd196e-d795-418f-b66a-116e48d1be1b","date":"2024-04-12T13:50:49+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"9579b196-f746-4b04-9e2f-31a0b1fb0b57","date":"2024-04-10T13:10:51+00:00","index":"hide","fulltext":""},{"type":"reviewersInvited","content":"","date":"2024-04-10T13:05:01+00:00","index":"","fulltext":""},{"type":"editorAssigned","content":"","date":"2024-04-10T05:44:13+00:00","index":"","fulltext":""},{"type":"checksComplete","content":"","date":"2024-04-10T05:44:13+00:00","index":"","fulltext":""},{"type":"submitted","content":"BMC Urology","date":"2024-04-09T13:03:07+00:00","index":"","fulltext":""}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"bmc-urology","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"buro","sideBox":"Learn more about [BMC Urology](http://bmcurol.biomedcentral.com/)","snPcode":"","submissionUrl":"https://www.editorialmanager.com/buro/default.aspx","title":"BMC Urology","twitterHandle":"BMC_series","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"em","reportingPortfolio":"BMC Series","inReviewEnabled":true,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"374f23ed-5b9f-4024-be45-72691b377473","owner":[],"postedDate":"April 18th, 2024","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"published-in-journal","subjectAreas":[],"tags":[],"updatedAt":"2024-06-21T14:49:21+00:00","versionOfRecord":{"articleIdentity":"rs-4242104","link":"https://doi.org/10.1186/s12894-024-01508-6","journal":{"identity":"bmc-urology","isVorOnly":false,"title":"BMC Urology"},"publishedOn":"2024-06-10 14:49:21","publishedOnDateReadable":"June 10th, 2024"},"versionCreatedAt":"2024-04-18 17:38:40","video":"","vorDoi":"10.1186/s12894-024-01508-6","vorDoiUrl":"https://doi.org/10.1186/s12894-024-01508-6","workflowStages":[]},"version":"v1","identity":"rs-4242104","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-4242104","identity":"rs-4242104","version":["v1"]},"buildId":"qtupq5eGEP_6zYnWcrvyt","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}