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CC BY 4.0 · Indian J Med Paediatr Oncol
DOI: 10.1055/s-0045-1804502
Case Report with Review of Literature

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

Hemavathi Narayan
1   Department of Pathology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
,
Sanghamitra Mukherjee
1   Department of Pathology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
,
Jesmy Mary Varughese
1   Department of Pathology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
,
Priya Dharmalingam
1   Department of Pathology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
,
Geeta V. Patil Okaly
1   Department of Pathology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
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Abstract

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.

Keywords

extrarenal Wilms tumor - retroperitoneum - nonanaplastic - childhood malignancy - intermediate risk

Note

Few of the terminology used such as names of drugs and abbreviations like COG and SIOP are standard words and cannot be modified.


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Publication History

Article published online:
11 March 2025

© 2025. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/)

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