Extrarenal Wilms Tumor in the Retroperitoneum: An Unusual Case Report and Review of Literature

Extrarenal Wilms tumor (ERWT) is an exceptionally rare neoplasm. The definition of an ERWT requires that it to be located extrarenally with histological evidence. ERWT is a unique tumor, with few reports in the literature. Till date, there have been only 51 reported cases of retroperitoneal ERWT in...

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Main Authors: Hemavathi Narayan, Sanghamitra Mukherjee, Jesmy Mary Varughese, Priya Dharmalingam, Geeta V. Patil Okaly
Format: Article
Language:English
Published: Thieme Medical and Scientific Publishers Pvt. Ltd.
Series:Indian Journal of Medical and Paediatric Oncology
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Online Access:http://www.thieme-connect.de/DOI/DOI?10.1055/s-0045-1804502
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author Hemavathi Narayan
Sanghamitra Mukherjee
Jesmy Mary Varughese
Priya Dharmalingam
Geeta V. Patil Okaly
author_facet Hemavathi Narayan
Sanghamitra Mukherjee
Jesmy Mary Varughese
Priya Dharmalingam
Geeta V. Patil Okaly
author_sort Hemavathi Narayan
collection DOAJ
description Extrarenal Wilms tumor (ERWT) is an exceptionally rare neoplasm. The definition of an ERWT requires that it to be located extrarenally with histological evidence. ERWT is a unique tumor, with few reports in the literature. Till date, there have been only 51 reported cases of retroperitoneal ERWT in the literature. ERWT is a challenging entity to diagnose, treat, and prognosticate. We present a case of a 4-year-old girl with complains of abdominal mass for past 1 month. Abdominal contrast-enhanced computed tomography scan revealed an extrarenal large solid mass in the retroperitoneum. Postsurgery histomorphology and immunohistochemistry confirmed the tumor as ERWT displaying favorable histology. This case highlights the importance of a precise histopathology diagnosis as well as clinical acumen regarding such rare cases. In atypical clinical presentation with inconclusive imaging findings, the diagnosis is based on histomorphology and immunohistochemistry. It also aids in giving this unusual tumor a clear perspective.
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publisher Thieme Medical and Scientific Publishers Pvt. Ltd.
record_format Article
series Indian Journal of Medical and Paediatric Oncology
spelling doaj-art-74c1daf148cb4f84a09e96a7bd57f0c72025-08-20T02:22:20ZengThieme Medical and Scientific Publishers Pvt. Ltd.Indian Journal of Medical and Paediatric Oncology0971-58510975-212910.1055/s-0045-1804502Extrarenal Wilms Tumor in the Retroperitoneum: An Unusual Case Report and Review of LiteratureHemavathi Narayan0Sanghamitra Mukherjee1Jesmy Mary Varughese2Priya Dharmalingam3Geeta V. Patil Okaly4Department of Pathology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, IndiaDepartment of Pathology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, IndiaDepartment of Pathology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, IndiaDepartment of Pathology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, IndiaDepartment of Pathology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, IndiaExtrarenal Wilms tumor (ERWT) is an exceptionally rare neoplasm. The definition of an ERWT requires that it to be located extrarenally with histological evidence. ERWT is a unique tumor, with few reports in the literature. Till date, there have been only 51 reported cases of retroperitoneal ERWT in the literature. ERWT is a challenging entity to diagnose, treat, and prognosticate. We present a case of a 4-year-old girl with complains of abdominal mass for past 1 month. Abdominal contrast-enhanced computed tomography scan revealed an extrarenal large solid mass in the retroperitoneum. Postsurgery histomorphology and immunohistochemistry confirmed the tumor as ERWT displaying favorable histology. This case highlights the importance of a precise histopathology diagnosis as well as clinical acumen regarding such rare cases. In atypical clinical presentation with inconclusive imaging findings, the diagnosis is based on histomorphology and immunohistochemistry. It also aids in giving this unusual tumor a clear perspective.http://www.thieme-connect.de/DOI/DOI?10.1055/s-0045-1804502extrarenal Wilms tumorretroperitoneumnonanaplasticchildhood malignancyintermediate risk
spellingShingle Hemavathi Narayan
Sanghamitra Mukherjee
Jesmy Mary Varughese
Priya Dharmalingam
Geeta V. Patil Okaly
Extrarenal Wilms Tumor in the Retroperitoneum: An Unusual Case Report and Review of Literature
Indian Journal of Medical and Paediatric Oncology
extrarenal Wilms tumor
retroperitoneum
nonanaplastic
childhood malignancy
intermediate risk
title Extrarenal Wilms Tumor in the Retroperitoneum: An Unusual Case Report and Review of Literature
title_full Extrarenal Wilms Tumor in the Retroperitoneum: An Unusual Case Report and Review of Literature
title_fullStr Extrarenal Wilms Tumor in the Retroperitoneum: An Unusual Case Report and Review of Literature
title_full_unstemmed Extrarenal Wilms Tumor in the Retroperitoneum: An Unusual Case Report and Review of Literature
title_short Extrarenal Wilms Tumor in the Retroperitoneum: An Unusual Case Report and Review of Literature
title_sort extrarenal wilms tumor in the retroperitoneum an unusual case report and review of literature
topic extrarenal Wilms tumor
retroperitoneum
nonanaplastic
childhood malignancy
intermediate risk
url http://www.thieme-connect.de/DOI/DOI?10.1055/s-0045-1804502
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AT jesmymaryvarughese extrarenalwilmstumorintheretroperitoneumanunusualcasereportandreviewofliterature
AT priyadharmalingam extrarenalwilmstumorintheretroperitoneumanunusualcasereportandreviewofliterature
AT geetavpatilokaly extrarenalwilmstumorintheretroperitoneumanunusualcasereportandreviewofliterature