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

Jun 05, 2023

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

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

Author

admin

View all posts

ABOUT THE EXPERTS

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

Bindi Naik-Mathuria

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

Alan F Utria

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

Peter F Ehrlich

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

Jennifer H Aldrink

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

Andrew J Murphy

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

Timothy Lautz

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

Roshni Dasgupta

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

Scott S Short

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

Harold N Lovvorn

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

Eugene S Kim

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

Erica Newman

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

Dave R Lal

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

Barrie S Rich

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

Nelson Piché

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

Zachary J Kastenberg

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

Marcus M Malek

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

Richard D Glick

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

Robin T Petroze

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

Stephanie F Polites

Department of Surgery, Mayo Clinic, Rochester, MN.

Richard Whitlock

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

Elizabeth Alore

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

Pattamon Sutthatarn

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

Stephanie Y Chen

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

Shannon Wong-Michalak

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

Rodrigo Lp Romao

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

Ameer Al-Hadidi

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

Nathan S Rubalcava

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

John P Marquart

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

Hailey Gainer

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

Mike Johnson

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

Chloe Boehmer

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

Hannah Rinehardt

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

Natashia M Seemann

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

Jacob Davidson

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

Valerie Polcz

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

Sarah B Lund

Department of Surgery, Mayo Clinic, Rochester, MN.

Katlyn G McKay

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

Hernan Correa

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

David H Rothstein

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