&nbsp;Wilms’ tumor is one of the most common abdominal tumor of childhood, primarily a malignant renal tumor. &nbsp;Extrarenal wilms’ tumor, however, is a rare disease, more commonly reported in Indian subcontinent. The locations of an extrarenal wilms’ tumor include retroperitoneum, uterus, skin and thorax

Liaghat, Naeem

Rasul, Laraib

Salman, Zainab

Shahzad, Iqra

English

Journals Portal, Shahid Beheshti University of Medical Sciences

112 Case Report  Extra-renal Wilms’ Tumor in a child: A Case Report  Naeem Liaqat1 , Laraib Rasul2, Zainab Salman3, Iqra Shahzad4  1 Pediatric Surgeon, Ibn Sina Hospital, Kuwait 2 PostGraduate Resident Department of Pediatric Surgery, Holy Family Hospital, Rawalpindi Pakistan 3 house officer Department of Pediatric Surgery, Holy Family Hospital, Rawalpindi Pakistan  4 House officer Department of Pediatric Surgery, Holy Family Hospital, Rawalpindi Pakistan *Address for Corresponder: Dr Naeem Liaqat, Assistant professor Pediatric surgery, Holy Family Hospital, Rawalpindi Pakistan. (Email:  simsonian.chaudary@gmail.com) How to cite this article: Liaqat N, Rasul L, Salman Z, Shahzad I. Extra-renal Wilms’ Tumor in a child: A Case Report. Iranian Journal of Pediatric Surgery 2023; 9(1):112-119.  DOI: https://doi.org/10.22037/irjps.v9i1.32755  Abstract  Keywords • Wilms’ tumor • Pediatric • extrarenal  Wilms’ tumor is one of the commonest abdominal tumors of childhood, primarily a malignant renal tumor. However, extrarenal Wilms’ tumor (ERWT) is a rare disease, and only a few cases have been reported in the pediatric age group. Here we report a child with retroperitoneal mass, who had ERWT.  Introduction Renal tumors account for 6% of all the malignancies in children and Wilma's tumor is the commonest. Extra-renal    Wilms’ tumor (ERWT) or Extra-renal Nephroblastomatosis is a rare condition  and accounts for 0.5-1% of all Wilms’ tumor cases. ERWT has been reported in received: 2 November 2020accepted: 19 June 2021Published online:May 2023This open-access article is distributed under the terms of the Cre-ative Commons Attribution Non Commercial 3.0 License (CC BY-NC 3.0). Downloaded from: http://journals.sbmu.ac.ir/irjps113Extra-Renal Wilms’ Tumor in A Child: A Case Report                 Liaqat et alIranian Journal of Pediatric Surgery    Vol.9    No.1/2023     This open-access article is distributed under the terms of the Cre-ative Commons Attribution Non Commercial 3.0 License (CC BY-NC 3.0). Downloaded from: http://journals.sbmu.ac.ir/irjpsthe retroperitoneum, scrotum, inguinal region, and pelvis.1-2 Due to rarity of ERWT, it’s prognostic markers and specific pre-operative diagnostic tools can’t be determined. To date, less than 100 cases of ERWT in pediatric age groups have been reported.4 Mostly it presents as a mass and general symptom related to the mass. Here we present a case of ERWT in a male child having a retroperitoneal mass.   Case Report A twoyears old male child presented with an abdominal mass on the left side for two months. This mass was gradually increasing in size with no associated pain. However, there was an associated history of weight loss and anorexia. A 12 x 8 cm mass was palpable on abdominal examination, which was occupying the left hypochondrium and left lumbar region. It was a non-tender, firm, smooth, and mobile mass. Its upper limit was not reachable; however lower limit was approachable. His baseline investigations were obtained, which were within normal ranges. Ultrasound revealed it arising from left     kidney and havingboth solid and cystic  components within it, however, right kidney was not visualized. CT scan of the abdomen showed this mass attached to the retroperitoneum, closely abutting the left kidney's upper pole and having foci of calcifications. There was hydronephrosis on the left side due to mass compression, and the right kidney was absent. His renal Diethylenetriamine pentaacetate (DTPA) scan revealed absent/non-functioning right kidney and reduced left kidney function. His serum Alfa fetoprotein level was in normal range. The patient was optimized and explored. Per-operatively, mass was lying close to the left kidney and had both solid and cystic components. There was a hydroureter, and both the left kidney and ureter were pushed by mass. Mass was completely separated from surrounding structures and was excised in toto (figure 1A & figure 1B). His postoperative recovery remained uneventful. A biopsy report of the mass showed it to be ERWT (figure 2). The patient is in our two years follow-up and doing well to date.     Iranian Journal of Pediatric Surgery    Vol.9    No.1/2023  This open-access article is distributed under the terms of the Cre-ative Commons Attribution Non Commercial 3.0 License (CC BY-NC 3.0). Downloaded from: http://journals.sbmu.ac.ir/irjps114 Extra-Renal Wilms’ Tumor in A Child: A Case Report                 Liaqat et al  Figure 1:  A) Intra-operative picture of the mass B) Mass excised in toto   Figure 2: Histopathological picture of the mass Iranian Journal of Pediatric Surgery    Vol.9    No.1/2023     This open-access article is distributed under the terms of the Cre-ative Commons Attribution Non Commercial 3.0 License (CC BY-NC 3.0). Downloaded from: http://journals.sbmu.ac.ir/irjps115Extra-Renal Wilms’ Tumor in A Child: A Case Report                 Liaqat et al  Discussion Wilms' tumor is one of the most common abdominal malignancies of childhood; ERWT, on the other hand, is a rarity. The first case of ERWT was reported by Moyson in 1961.1  Most cases that have been reported in literature, are predominately present in the retroperitoneum. Other reported sites for ERWT include sacrococcygeal region, paravertebral region, inguinal canal, ovary, uterus, paratesticular region, mediastinum and urinary bladder.3-4  Although this tumor's exact origin is still hazy, the most plausible hypothesis includes that of origin from ectopic metanephric blastema.5  These ectopic cells may reside at any site and lead to ERWT. There is also a theory of romance of persistent embryoic potential cells (Connheims’ cell rest theory). There are other situations in which ERWT may be found like as a metastatic disease or a nephroblastoma originating in a teratoma.1 ERWT are more common in females, with a female:male ratio of 56:44 and occurs within the first decade of life. Patients with ERWT present usually in range of 2 months to 10 years; contrary to the age of Wilms’ tumor being 44 months.1-6 The oldest case reported in literature of ERWT   was in a 77 years old female who had uterine ERWT.1 ERWT usually presents as an asymptomatic abdominal mass with insidious growth. There is no specific presenting feature to it and usually they depend upon the site of origin.5-7-6 In the indexed case, child had painless abdominal mass, causing hydronephrosis due to compression of the ureter and kidney by mass. Investigating this tumor may reveal elevated tumor markers like alpha-fetoprotein and beta-HCG. As for imaging modalities, the initial and the most commonly used radiological intervention is ultrasonography. Sonograms usually demonstrate well defined, smooth mass of renal origin with uniform echogenicity. Contrast-enhanced CT scans provide a better and clearer picture of the tumor's quality and extension, highlighting the usual picture of a heterogeneous infiltrative mass with vessel encasement and calcification. Bony metastasis is common, for which bone scans can be useful.2-9-11  The differential diagnosis for ERWT (of cystic variety) includes cystic extragonadal germ cell tumors, neurogenic tumors, and, rarely, myxoid liposarcoma.2-10 Iranian Journal of Pediatric Surgery    Vol.9    No.1/2023  This open-access article is distributed under the terms of the Cre-ative Commons Attribution Non Commercial 3.0 License (CC BY-NC 3.0). Downloaded from: http://journals.sbmu.ac.ir/irjps116Unfortunately, being an aggressive malignant tumor, it carries the potential of distant metastasis, and local recurrences. The cystic variety of this tumor has a better prognosis than solid ones.3-4  Ultimately, renal and extra-renal Wilms tumors can only be confirmed on histopathological evaluation, as in our case. The diagnosis of ERWT is usually difficult. As per National Wilms’ Tumor Study Groups, three components are necessary inorder to make a diagnosis of ERWT.  These include: 1) tumor should not be present in both kidney, 2) there must be a    Table 1: triphasic pattern in histopathology including undifferentiated blastemal, epithelial and mesenchymal components, and 3) no teratoma or renal cell carcinoma detected on histopathology.1-4 Approximately 200 cases of ERWT in both adults and children have been reported.1 Shojaeian et al. reported 80 childhood ERWT cases from 1961-2015.4 We did a literature review from 2015 onwards and retrieved 18 more cases. We also found four case12-14 in the literature which were reported before 2015, but were missed by Shojaeian. Their summary has been given in Table 1.   Sr. No. Author Year Age Gender Location 1.  Lakhkar et al (13) 2004 2 years Male Right iliac fossa 2.  Dabus et al (14) 2004 2 years Female Retroperitoneal right supra-renal 3.  C Ramachandra et al (12) 2007 4 years Male Retroperitoneal right lumbar 4.  C Ramachandra et al (12) 2007 3 years Female Retroperitoneal right supra-renal 5.  Thakkar et al (15) 2015 5 years Female Right ovary 6.  Park et al (16) 2016 4 years Female Inguinal canal 7.  Igbaseimokumo U et al (17) 2017 Newborn Female Lumbar Spine 8.  Ozkisacik et al  (8) 2017 3 years Male Retro-vesical 9.  Wabada S et al (5) 2017 2 years Male Retroperitoneal mass 10.  Itoshima R et al (3) 2017 4 years Male Retroperitoneal Extra-Renal Wilms’ Tumor in A Child: A Case Report                 Liaqat et alIranian Journal of Pediatric Surgery    Vol.9    No.1/2023     This open-access article is distributed under the terms of the Cre-ative Commons Attribution Non Commercial 3.0 License (CC BY-NC 3.0). Downloaded from: http://journals.sbmu.ac.ir/irjps117Unfortunately, being an aggressive malignant tumor, it carries the potential of distant metastasis, and local recurrences. The cystic variety of this tumor has a better prognosis than solid ones.3-4  Ultimately, renal and extra-renal Wilms tumors can only be confirmed on histopathological evaluation, as in our case. The diagnosis of ERWT is usually difficult. As per National Wilms’ Tumor Study Groups, three components are necessary inorder to make a diagnosis of ERWT.  These include: 1) tumor should not be present in both kidney, 2) there must be a    Table 1: triphasic pattern in histopathology including undifferentiated blastemal, epithelial and mesenchymal components, and 3) no teratoma or renal cell carcinoma detected on histopathology.1-4 Approximately 200 cases of ERWT in both adults and children have been reported.1 Shojaeian et al. reported 80 childhood ERWT cases from 1961-2015.4 We did a literature review from 2015 onwards and retrieved 18 more cases. We also found four case12-14 in the literature which were reported before 2015, but were missed by Shojaeian. Their summary has been given in Table 1.   Sr. No. Author Year Age Gender Location 1.  Lakhkar et al (13) 2004 2 years Male Right iliac fossa 2.  Dabus et al (14) 2004 2 years Female Retroperitoneal right supra-renal 3.  C Ramachandra et al (12) 2007 4 years Male Retroperitoneal right lumbar 4.  C Ramachandra et al (12) 2007 3 years Female Retroperitoneal right supra-renal 5.  Thakkar et al (15) 2015 5 years Female Right ovary 6.  Park et al (16) 2016 4 years Female Inguinal canal 7.  Igbaseimokumo U et al (17) 2017 Newborn Female Lumbar Spine 8.  Ozkisacik et al  (8) 2017 3 years Male Retro-vesical 9.  Wabada S et al (5) 2017 2 years Male Retroperitoneal mass 10.  Itoshima R et al (3) 2017 4 years Male Retroperitoneal 11.  Chin Tan et al (7) 2018 2 years Female Pelvic mass with skeletal and glial differentiation 12.  Tang H et al (18) 2018 2 years Male Retroperitoneal mass 13.  Tang H et al(18) 2018 2 years Female Mesentry 14.  Tan GC et al (7) 2018 2 years Female Pelvis 15.  Groth et al  (19) 2019 2 years Male Para-testicular mass 16.  Sindhu et al (1) 2019 6 years Male Urinary bladder 17.  Liang H et al (6) 2020 5 years Male Retroperitoneal mass 18.  Liang H et al (6) 2020 3.4 years Female Presacral 19.  Liang H et al (6) 2020 3.4 years Female Duplicate sigmoid colon 20.  Liang H et al (6) 2020 9.8 years Male Retroperitoneal mass 21.  Liang H et al (6) 2020 2.8 years Male Gubernaculum Testis 22.  Filizoglu N et al (20) 2021 3 years Female Lumbar mass Conclusion We conclude that ERWT is a rare pathology, but it must be considered as one of the differentials among the retroperitoneal tumors. The diagnosis can only be confirmed with histopathology.  Ethical Considerations This study is approved by the Institutional Ethics Committee of Yenepoya (Deemed to be University) with the Protocol No: YEC2/833.   Acknowledgment  Not Applicable   Funding/Support  Not Applicable   Conflict of interests  There are no conflicts of interest.      Extra-Renal Wilms’ Tumor in A Child: A Case Report                 Liaqat et alIranian Journal of Pediatric Surgery    Vol.9    No.1/2023  This open-access article is distributed under the terms of the Cre-ative Commons Attribution Non Commercial 3.0 License (CC BY-NC 3.0). Downloaded from: http://journals.sbmu.ac.ir/irjps118  References: 1.  Sindhu II, Saeed H, Wali R, et al; Primary extra-renal Wilms’ tumor in urinary bladder: rare presentation of a common pediatric malignancy. J Coll Physicians Surg Pak. 2019 Jun 1;29(6):S31-3. 2. Baskaran D; Extrarenal teratoid wilms' tumor in association with horseshoe kidney. Indian J Surg. 2013;75(2):128-32. 3. Itoshima R, Kobayashi R, Sano H, et al; Extrarenal nephroblastoma of the retroperitoneal space in children: A case report and review of the literature. J Pediatr Hematol Oncol. 2017;39(4):296-8. 4. Shojaeian R, Hiradfar M, Sharifabad PS, et al; Extrarenal Wilms’ Tumor: challenges in diagnosis, embryology, treatment and prognosis. Exon Publications. 2016 Mar 23:77-93. 5. Wabada S, Abubakar AS, Adamu AI, et al; A retroperitoneal extra-renal wilms’ tumour: A case report. Nigerian Journal of Clinical Practice. 2017 Mar 7;20(3):388-91. 6. Liang H, He Y, Fu L, et al; Extrarenal Wilms tumor in children: A retrospective observational case series. Journal of Pediatric Urology. 2020 Oct 1;16(5):664-e1. 7. Tan GC, Prasad V; Extrarenal wilms tumor with skeletal and glial differentiation. Int J Surg Pathol. 2018;26(1):34. 8. Ozkisacik S, Erdem A, Yazici M; A rare antitiy extrarenal Wilms tumor: A case report. Med J Clin Trials Case Stud. 2017;1(2):000108. 9. Goel V, Verma AK, Batra V,et al; ‘Primary extrarenal Wilms’ tumour’: rare presentation of a common paediatric tumour. Case Reports. 2014 Jun 6;2014:bcr2013202172. 10. Sastri J, Dedhia R, Laskar S, et al; Extra-renal Wilms' tumour--is it different? Pediatr Nephrol. 2006;21(4):591-6. 11. Ratnam GV, Abu-Eshy S, Morad N, et al; Adult extrarenal Wilms' tumour: A case report and review of literature. West Afr J Med. 2006;25(1):75-8. 12. Ramachandra C, Attili VSS, Dadhich HP, et al: Extrarenal Wilms' tumor; A report of two cases and review of literature. J Indian Assoc Pediatr Surg. 2007;12(3):145. Extra-Renal Wilms’ Tumor in A Child: A Case Report                 Liaqat et alIranian Journal of Pediatric Surgery    Vol.9    No.1/2023     This open-access article is distributed under the terms of the Cre-ative Commons Attribution Non Commercial 3.0 License (CC BY-NC 3.0). Downloaded from: http://journals.sbmu.ac.ir/irjps119  13. Lakhkar BN, Rajgopal K, Shetty C, et al; Extrarenal Wilms’ tumour: A case report. Indian J Radiol Imaging. 2004;14(2):147. 14. Dabus GD, Pereira IM; Tumor de Wilms extra-renal: relato de caso. Radiologia Brasileira. 2004;37:299-301. 15. Thakkar NC, Sarin YK: Extra-renal Wilms' tumor; A rare diagnosis. APSP journal of case reports. 2015;6(2). 16. Park J; Extrarenal retroperitoneal Wilms' tumor with subsequent pulmonary and peritoneal metastasis in a 4 year-old girl: A case report and review of literature. J Pediatr Surg Case Rep. 2016;8:19-21. 17. Igbaseimokumo U, Cartwright C, Lewing K, et al; The rare association of spina bifida and extrarenal Wilms tumor: A case report and review of the literature. World Neurosurg. 2017;104:1046 e1- e5. 18. Tang H, Lu M, Jiang S, et al; Two rare cases of abdominal tumor in children: Answers. Pediatr Nephrol. 2018;33(8):1343-5. 19. Groth TW, Southern J, Goetz JT, et al; A case of primary paratesticular wilms tumor in an undescended testis. Urology. 2019;129:197-9. 20. Filizoglu N, Oksuzoglu K, Bozkurt S, et al; FDG PET/CT findings of extrarenal Wilms tumor originating from the spinal cord. Clin Nucl Med. 2021;46(1):86-7.  Extra-Renal Wilms’ Tumor in A Child: A Case Report                 Liaqat et al

Extra-renal Wilms’ Tumor in a child: A Case Report

https://journals.sbmu.ac.ir/irjps/article/download/32755/31350

Extra-renal Wilms’ Tumor in a child: A Case Report

Abstract

Similar works

Full text

Available Versions

Journals Portal, Shahid Beheshti University of Medical Sciences