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Case Reports in Oncological Medicine
Volume 2013, Article ID 627472, 4 pages
Case Report

Retroperitoneal Malignant Peripheral Nerve Sheath Tumor Replacing an Absent Kidney in a Child

1Pediatric Congenital Hematologic Disorders Research Center, Shahid Beheshti University of Medical Sciences, Tehran 15468-15514, Iran
2Mofid Children’s Hospital, Dr. Shariati Avenue, Tehran, Iran
3Pediatric Pathology Research Center, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran 15468-15514, Iran

Received 23 September 2013; Accepted 14 November 2013

Academic Editors: J. Ø. Keller, C. V. Reyes, and Y. Yokoyama

Copyright © 2013 Samin Alavi et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Malignant peripheral nerve sheath tumors (MPNSTs) are nonrhabdomyosarcoma soft tissue sarcomas with rare occurrence in children specially in the retroperitoneum. We describe a young child who presented with an abdominal mass. Both ultrasound and computed tomography revealed a large right-sided abdominal mass in the anatomic place of right kidney, while no kidney or ureter was observed at that side. He underwent surgical resection of the tumor with a primary impression of Wilms tumor. To the authors’ knowledge, this is the first case of retroperitoneal malignant peripheral nerve sheath tumor and absent kidney. This case suggests the very rare probability of association of MPNSTs in children with genitourinary tract anomalies such as renal agenesis.