Management and Outcomes of Wilms Tumor with Suprarenal Intravascular Extension: A Pediatric Surgical Oncology Research Collaborative Study : Annals of Surgery

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Management and Outcomes of Wilms Tumor with Suprarenal Intravascular Extension

A Pediatric Surgical Oncology Research Collaborative Study

Naik-Mathuria, Bindi*; Utria, Alan F.†; Ehrlich, Peter F‡; Aldrink, Jennifer H.§; Murphy, Andrew J.∥; Lautz, Timothy; Dasgupta, Roshni#; Short, Scott S.**; Lovvorn, Harold N. III††; Kim, Eugene S.‡‡; Newman, Erica‡; Lal, Dave R.§§; Rich, Barrie S.∥∥; Piché, Nelson¶¶; Kastenberg, Zachary J.##; Malek, Marcus M.***; Glick, Richard D.∥∥; Petroze, Robin T.†††; Polites, Stephanie F.‡‡‡; Whitlock, Richard§§§; Alore, Elizabeth§§§; Sutthatarn, Pattamon∥; Chen, Stephanie Y.‡‡; Wong-Michalak, Shannon*; Romao, Rodrigo LP†; Al-Hadidi, Ameer§; Rubalcava, Nathan S.‡; Marquart, John P.§§; Gainer, Hailey§§; Johnson, Mike#; Boehmer, Chloe#; Rinehardt, Hannah***; Seemann, Natashia M.§§§; Davidson, Jacob§§§; Polcz, Valerie†††; Lund, Sarah B.‡‡‡; McKay, Katlyn G.††; Correa, Hernan††; Rothstein, David H.†

Author Information

*Department of Surgery, Division of Pediatric Surgery, University of Texas Medical Branch, Galveston, TX

†Division of General and Thoracic Surgery, Seattle Children’s Hospital; Department of Surgery, University of Washington School of Medicine, Seattle, WA

‡Section of Pediatric Surgery, Department of Surgery, C.S. Mott Children’s Hospital, The University of Michigan, Ann Arbor, MI

§Division of Pediatric Surgery, Department of Surgery, Nationwide Children’s Hospital, The Ohio State University College of Medicine, Columbus, OH

∥Department of Surgery, St. Jude Children’s Research Hospital, Memphis, TN

Division of Pediatric Surgery, Lurie Children’s Hospital, Department of Surgery, Northwestern School of Medicine, Chicago, OH

#Division of Pediatric General and Thoracic Surgery, Cincinnati Children’s Medical Center, Cincinnati, OH

**Division of Pediatric Surgery, Department of Surgery, University of Utah, Primary Children’s Hospital, Salt Lake City, UT

††Department of Pediatric Surgery, Pediatric Pathology, and Section of Surgical Sciences, Vanderbilt University Medical Center, Nashville, TN

‡‡Division of Pediatric Surgery, Cedars-Sinai Medical Center, Los Angeles, CA

§§Division of Pediatric Surgery, Medical College of Wisconsin, Children’s Wisconsin, Milwaukee, WI

∥∥Division of Pediatric Surgery, Zucker School of Medicine at Hofstra/Northwell, Cohen Children’s Medical Center, New Hyde Park, NY

¶¶Division of Pediatric Surgery, Centre Hospitalier Universitaire Ste-Justine, Université de Montréal, Montréal, Québec, Canada

##Division of Pediatric Surgery, Department of Surgery, University of Utah, Primary Children’s Hospital, Salt Lake City, Utah

***Division of Pediatric General and Thoracic Surgery, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA

†††Division of Pediatric Surgery, University of Florida, Gainesville, FL

‡‡‡Department of Surgery, Mayo Clinic, Rochester, MN

§§§Department of Surgery, Baylor College of Medicine, Texas Children’s Hospital, Houston, TX

∥∥∥Division of Pediatric Surgery, Department of Surgery, Western University, London, ON

¶¶¶Division of Pediatric Surgery, Department of Surgery, Children’s Hospital Los Angeles, Keck School of Medicine, University of Southern California, Los Angeles, CA

###Division of Pediatric Surgery and Pediatric Urology, IWK Health, Dalhousie University, Halifax NS, Canada

Research support: none

The authors report no conflicts of interest.

[email protected]

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Annals of Surgery ():10.1097/SLA.0000000000005921, June 2, 2023. | DOI: 10.1097/SLA.0000000000005921
  • Open
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Abstract

Objective: 

The purpose of this study was to describe management and outcomes from a contemporary cohort of children with Wilms Tumor (WT) complicated by inferior vena caval thrombus.

Summary Background Data: 

The largest series of these patients was published almost two decades ago. Since then, neoadjuvant chemotherapy has been commonly used to manage these patients and outcomes have not been reported.

Methods: 

Retrospective review from 19 North American centers between 2009-2019. Patient and disease characteristics, management, and outcomes were investigated and analyzed.

Results: 

Of 124 patients, 81% had favorable histology (FH), and 52% were stage IV. IVC thrombus level was infrahepatic in 53 (43%), intrahepatic in 32 (26%), suprahepatic in 14 (11%), and cardiac in 24 (19%). Neoadjuvant chemotherapy using a 3-drug regimen was administered in 82% and post-resection radiation in 90%. Thrombus level regression was 45% overall, with suprahepatic level showing the best response (62%). Cardiopulmonary bypass (CPB) was potentially avoided in 67%. Peri-operative complication rate was significantly lower following neoadjuvant chemotherapy ([25%] vs. Upfront surgery [55%]; P=0.005). CPB was not associated with higher complications (CPB [50%] vs. no CPB [27%]; P=0.08). Two-year event-free survival (EFS) was 93% and overall survival (OS) was 96%, higher in FH cases (FH 98% vs. unfavorable histology/anaplastic 82%; P=0.73). Neither incomplete resection nor viable thrombus cells affected EFS or OS.

Conclusions: 

Multimodal therapy resulted in excellent outcomes, even with advanced stage disease and cardiac extension. Neoadjuvant chemotherapy decreased the need for CPB to facilitate resection. Complete thrombectomy may not always be necessary.

Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc.