Outcomes and Surgical Management of Malignant Rhabdoid Tumor of the Kidney: A Report From the Pediatric Surgical Oncology Research Collaborative
Year: 2026
Type: article
Study design: Observational Study
Abstract: PURPOSE: Malignant rhabdoid tumor of the kidney (MRTK) is a rare, aggressive tumor seen in young children. The optimal timing of resection for locally advanced tumors is not well-defined. The purpose of this study is to evaluate modern oncologic outcomes and the impact of surgical timing. METHODS: A multicenter retrospective review was performed by institutions participating in the Pediatric Surgical Oncology Research Collaborative. Children younger than 21 years old with MRTK diagnosed between 2000 and 2022 were included. RESULTS: Sixty-nine patients were identified with MRTK and met the inclusion criteria. Median age of diagnosis was 10.1 months. Overall survival (OS) at 1, 5, and 10 years was 49%, 32%, and 19%, respectively. Patients with local Stage III disease who underwent upfront resection (n = 18) compared to those who had delayed resection after NAT (n = 15) had a similar OS, median OS greater than 60 months versus 14.6 months, respectively; p = 0.396. The surgical timing groups were balanced in terms of the presence of metastasis, length of follow-up, and tumor characteristics. There was one occurrence of primary intraoperative tumor spill and two occurrences of organ injury in the upfront resection group compared to none in the delayed resection group. CONCLUSION: MRTK carries a poor prognosis despite multimodal treatment. Histologic diagnosis may not be confirmed at presentation, and MRTK cannot be reliably distinguished from Wilms tumor on imaging alone. As overall survival is similar, the decision regarding surgical timing in locally advanced tumors should be individualized based on perceived resectability, balancing the risk of intraoperative complications against the possibility of tumor progression during neoadjuvant therapy.
Source: Pediatric Blood & Cancer
Authors: Hannah N. Rinehardt, Elisabeth T. Tracy, Anghela Paredes, Catherine Beckhorn, Harold J. Leraas, Joseph Fusco, Katlyn G. McKay, Nelly‐Ange T. Kontchou, Zachary J. Kastenberg, David W. Hoyt, Jonathan Roach, Emily K. Myers, Nicholas G. Cost, Bhargava Mullapudi, Charles R. Marchese, Amanda R. Jensen, Timothy B. Lautz, Michela Carter, Roshni Dasgupta, John Lundstedt, Joseph G. Brungardt, Lindsay Talbot, Andrew M. Davidoff, Andrew J. Murphy, Jennifer H. Aldrink, Sara Mansfield, Nelson Piche, Annie Le‐Nguyen, Dave R. Lal, Brian T. Craig, Jennifer M. Schuh, Barrett P. Cromeens, Sindhu Mannava, Shannon Castle, Adriana Lopez, Kelsey Mello, Joshua Short, Robin T. Petroze, Shay Rajavel, Grace R. Thompson, Peter Mattei, David H. Rothstein, Elizabeth Fialkowski, Kathryn Fowler, Nathan Martchenke, Barrie S. Rich, Richard D. Glick, Erin G. Brown, Kathleen Doyle, Paige Abril, Natashia Seemann, Jacob Davidson, Claire A. Wilson, Hau D. Le, Devashish Joshi, Michael Stellon, Tamer Ahmed, Alexandra Dimmer, Peter F. Ehrlich, Maya Hammoud, Keyonna Williams, Christa N. Grant, Merit Gorgy, Stephanie F. Polites, Julia Debertin, Danielle B. Cameron, Alyssa Stetson, Eugene S. Kim, William G. Lee, Aaron Barkhordar, Mary Austin, Brian A. Coakley, Anastasia Kahan, Joseph T. Murphy, Michael Pitonak, Chloé Boehmer, Marcus M. Malek
Institutions: Children's Hospital of Pittsburgh, University of Pittsburgh Medical Center, Duke Medical Center, Vanderbilt University Medical Center, Primary Children's Hospital, University of Utah, Children's Mercy Hospital, Northwestern University, Lurie Children's Hospital, Northwestern Medicine, Cincinnati Children's Hospital Medical Center, St. Jude Children's Research Hospital, Nationwide Children's Hospital, The Ohio State University, Centre Hospitalier Universitaire Sainte-Justine, Université de Montréal, Medical College of Wisconsin, Riley Hospital for Children, Indiana University School of Medicine, Children's Hospital Central California, Children's Minnesota, University of Florida Health, University of Florida, Seattle Children's Hospital, University of Washington, Oregon Health & Science University, Cohen Children's Medical Center, UC Davis Children's Hospital, University of California, Davis, London Health Sciences Centre, University of Wisconsin–Madison, University of Wisconsin American Family Children's Hospital, SUNY Upstate Medical University, University of Michigan, C. S. Mott Children's Hospital, Westchester Medical Center, New York Medical College, Mayo Clinic, Massachusetts General Hospital, Cedars-Sinai Medical Center, The University of Texas MD Anderson Cancer Center, Icahn School of Medicine at Mount Sinai, Children's Medical Center, The University of Texas Southwestern Medical Center
Language: English
Topic: Renal and related cancers
Subfield: Molecular Biology
Domain: Life Sciences
Open Access status: hybrid
Estimated APC paid: $4,640
OpenAlex ID: W7141468652