NYMC Faculty Publications

Renal Sarcomas in Children and Adolescents: A Retrospective, Multicenter Cohort Study

Katlyn G. McKay, Department of Surgery, Vanderbilt University Medical Center, Nashville, TN, USA.
Catherine Beckhorn, Duke University School of Medicine, Durham, NC, USA.
Nelly-Ange T. Kontchou, Department of Surgery, Vanderbilt University Medical Center, Nashville, TN, USA.
Zachary J. Kastenberg, Department of Surgery, Division of Pediatric Surgery, University of Utah, Primary Children's Hospital, Salt Lake City, UT, USA.
Jonathan Roach, Department of Pediatric Surgery, Children's Hospital of Colorado, Denver, CO, USA.
Bhargava Mullapudi, Children's Mercy Hospital, Kansas City, MO, USA.
Timothy B. Lautz, Department of Surgery, Division of Pediatric Surgery, Lurie Children's Hospital, Northwestern School of Medicine, Chicago, IL, USA.
Roshni Dasgupta, Division of Pediatric Surgery, Cincinnati Children's Medical Center, Cincinnati, OH, USA.
Lindsay J. Talbot, Department of Surgery, St. Jude Children's Research Hospital, Memphis, TN, USA.
Jennifer H. Aldrink, Division of Pediatric Surgery, Department of Surgery, Nationwide Children's Hospital, The Ohio State University College of Medicine, Columbus, OH, USA.
Nelson Piché, Division of Pediatric Surgery, Centre Hospitalier Universitaire Ste-Justine, Université de Montréal, Montréal, Quebec, Canada.
Brian T. Craig, Division of Pediatric Surgery, Medical College of Wisconsin, Children's Wisconsin, Milwaukee, WI, USA.
Barrett Cromeens, Division of Pediatric Surgery, Riley Hospital for Children, Indiana University School of Medicine, Indianapolis, IN, USA.
Shannon L. Castle, Division of Pediatric Surgery, Valley Children's Hospital, Madera, CA, USA.
Joshua Short, Pediatric Surgical Associates, Children's Minnesota, Minneapolis, MN, USA.
Robin T. Petroze, Division of Pediatric Surgery, C.S. Mott Children's Hospital, University of Michigan, Ann Arbor, MI, USA.
Peter Mattei, General, Thoracic and Fetal Surgery, Children's Hospital of Philadelphia, Philadelphia, PA, USA.
David H. Rothstein, Division of Pediatric General and Thoracic Surgery, University of Washington, Seattle, WA, USA.
Elizabeth A. Fialkowski, Department of Surgery, Oregon Health & Science University, Portland, OR, USA.
Barrie S. Rich, Division of Pediatric Surgery, Feinstein/Northwell, Cohen Children's Medical Center, Queens, NY, USA.
Erin G. Brown, Division of Pediatric Surgery, Department of Surgery, University of California Davis Children's Hospital, Sacramento, CA, USA.
Natashia M. Seemann, Division of Pediatric Surgery, Children's Hospital, London Health Sciences Centre, London, ON, Canada.
Hau D. Le, Division of Pediatric Surgery, American Family Children's Hospital, University of Wisconsin School of Medicine and Public Health, Madison, WI, USA.
Tamer M. Ahmed, Division of Pediatric Surgery, SUNY Upstate Medical University, Syracuse, NY, USA.
Erika A. Newman, Division of Pediatric Surgery, C.S. Mott Children's Hospital, University of Michigan, Ann Arbor, MI, USA.
Christa N. Grant, Division of Pediatric Surgery, Westchester Medical Center, New York Medical College, Valhalla, NY, USA.
Stephanie F. Polites, Department of Surgery, Mayo Clinic, Rochester, MN, USA.
Danielle B. Cameron, Massachusetts General Hospital, Boston, MA, USA.
Eugene S. Kim, Division of Pediatric Surgery, Cedars-Sinai Medical Center, Los Angeles, CA, USA.
Mary T. Austin, Division of Pediatric Surgery, University of Texas MD Anderson Cancer Center, Houston, TX, USA.
Brian A. Coakley, Division of Pediatric Surgery, Department of Surgery, Icahn School of Medicine Mount Sinai, New York, NY, USA.
Joseph T. Murphy, Division of Pediatric Surgery, Children's Health Children's Medical Center, University of Texas Southwestern, Dallas, TX, USA.

Author Type(s)

Faculty

DOI

10.1016/j.eclinm.2025.103713

Journal Title

Eclinicalmedicine

First Page

103713

Document Type

Article

Publication Date

1-1-2026

Department

Surgery

Keywords

Anaplastic sarcoma of the kidney, Children, Clear cell sarcoma of the kidney, DICER1, Ewing sarcoma, Renal sarcoma

Disciplines

Medicine and Health Sciences

Abstract

BACKGROUND: Renal sarcomas arise rarely in children and adolescents and represent a histologically and biologically diverse disease category. Consequently, standardizing optimal therapies for pediatric renal sarcomas remains challenging. Leveraging a large North American research collaborative, the purposes of this study were to evaluate the current state of patient, disease, and survival characteristics among pediatric renal sarcomas and to expose knowledge gaps that will inform future discovery. METHODS: Patients 21 years or younger and treated for a primary renal sarcoma between January 1st, 2000 and November 30th, 2022 were identified through the Pediatric Surgical Oncology Research Collaborative. Patient (e.g., demographics) and disease (e.g., histology, stage, molecular alterations) characteristics were abstracted from contributing institutions. Descriptive statistics, Pearson-Chi square (categorical variables), Kruskal-Wallis (continuous variables), Cox regression (Hazard ratios), and Kaplan-Meier 4-year event-free and overall survival (OS) analyses were completed. FINDINGS: Among 158 patients, clear cell sarcoma of the kidney (CCSK; n = 94), Ewing sarcoma (EWS; n = 33), and undifferentiated sarcoma (n = 8) predominated. Sarcoma type correlated significantly with age at diagnosis (p < 0.0001), with infantile fibrosarcoma (IFS) and CCSK occurring in the youngest patients, whereas EWS and synovial sarcoma presented in the oldest. Predisposition syndromes were identified in 11/155 (7.1%) patients, most commonly DICER1 and Li-Fraumeni. Multimodal therapies varied significantly across sarcoma types (p = 0.0008), although nephrectomy was uniform. Tumor thrombectomy was performed in 9 patients (6 with EWS). When tested, somatic molecular alterations were observed principally in CCSK (17/38; 45%) and EWS (26/26; 100%; p = 0.001). At 4 years, OS differed significantly by sarcoma type, ranging from highest to lowest as follows: CCSK 0.927 (95% CI 0.845-0.967), EWS 0.901 (95% CI 0.723-0.967), undifferentiated sarcoma 0.833 (95% CI 0.273-0.975), IFS 0.667 (95% CI 0.054-0.945), and rhabdomyosarcoma 0.500 (95% CI 0.111-0.804; p = 0.036). Hematogenous metastases occurred most in the lungs (n = 19 total; 10 with EWS), followed by bone (n = 12), which occurred only with CCSK (n = 9) and EWS (n = 3). Two patients developed brain metastases (one each with CCSK and rhabdomyosarcoma). At 4 years, OS was 0.957 (95% CI 0.888-0.984) for patients presenting without metastases and 0.717 (95% CI 0.545-0.833) for those with metastases (p = 0.00015). INTERPRETATION: Renal sarcomas presenting in children and adolescents comprise a heterogeneous disease category with unique patient, clinical, and molecular characteristics that complicate standardizing therapeutic strategies beyond CCSK and EWS. FUNDING: None.

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