Contemporary Biology, Management, and Outcomes of Renal Medullary Carcinoma in Children and Adults: A Pediatric Surgical Oncology Research Collaborative Study

Catherine B Beckhorn¹, Marcus M Malek², Harold N Lovvorn³, Harold J Leraas⁴, Katlyn G McKay⁵, Nelly-Ange T Kontchou⁵, Zachary J Kastenberg⁶, David W Hoyt⁶, Jonathan P Roach⁷, Emily K Myers⁷, Nicholas G Cost⁸, Bhargava Mullapudi⁹, Charles R Marchese⁹, Amanda R Jensen⁹, Timothy B Lautz¹⁰, Michela Carter¹⁰, Roshni Dasgupta¹¹, John Lundstedt¹¹, Joseph G Brungardt¹¹, Lindsay J Talbot¹², Andrew M Davidoff¹², Andrew J Murphy¹², Jennifer H Aldrink¹³, Sara A Mansfield¹³, Nelson Piché¹⁴, Dave R Lal¹⁵, Brian T Craig¹⁵, Jennifer M Schuh¹⁵, Barrett P Cromeens¹⁶, Sindhu V Mannava¹⁶, Shannon L Castle¹⁷, Adriana Lopez¹⁸, Kelsey Mello¹⁸, Joshua Short¹⁹, Robin T Petroze²⁰, Shay Rajavel²¹, Grace R Thompson²², Peter Mattei²³, David H Rothstein²⁴, Elizabeth Fialkowski²⁵, Kathryn L Fowler²⁵, Nathan Martchenke²⁵, Barrie S Rich²⁶, Richard D Glick²⁷, Erin G Brown²⁸, Kathleen Doyle²⁹, Paige Abril²⁹, Hannah N Rinehardt², Natashia M Seemann³⁰, Jacob Davidson³⁰, Claire A Wilson³⁰, Hau D Le³¹, Devashish Joshi³², Michael Stellon³², Tamer Ahmed³³, Alexandra M Dimmer³³, Erika A Newman³⁴, Maya Hammoud³⁴, Keyonna Williams³⁴, Christa N Grant³⁵, Merit Gorgy³⁶, Stephanie F Polites³⁷, Julia Debertin³⁷, Danielle B Cameron³⁸, Alyssa Stetson³⁸, Eugene S Kim³⁹, William G Lee³⁹, Aaron Barkhordar³⁹, Mary T Austin⁴⁰, Brian A Coakley⁴¹, Anastasia Kahan⁴², Joseph T Murphy⁴³, Michael Pitonak⁴³, Chloé Boehmer¹¹, Elisabeth T Tracy⁴⁴

Affiliations

¹ Duke University School of Medicine, Durham, North Carolina, USA.
² Division of Pediatric General and Thoracic Surgery, UPMC Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania, USA.
³ Department of Pediatric Surgery, Vanderbilt University Medical Center, Nashville, Tennessee, USA.
⁴ Department of Surgery, Duke University Medical Center, Durham, North Carolina, USA.
⁵ Department of Surgery, Vanderbilt University Medical Center, Nashville, Tennessee, USA.
⁶ Division of Pediatric Surgery, Department of Surgery, University of Utah, Primary Children's Hospital, Salt Lake City, Utah, USA.
⁷ Division of Pediatric Surgery, Department of Surgery, University of Colorado School of Medicine, Surgical Oncology Program Children's Hospital Colorado, Aurora, Colorado, USA.
⁸ Division of Urology, Department of Surgery, University of Colorado School of Medicine, Surgical Oncology Program Children's Hospital Colorado, Aurora, Colorado, USA.
⁹ Children's Mercy Research Institute, Kansas City, Kansas, USA.
¹⁰ Division of Pediatric Surgery, Department of Surgery, Ann and Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA.
¹¹ Division of Pediatric Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
¹² Department of Surgery, St Jude Children's Research Hospital, Memphis, Tennessee, USA.
¹³ Division of Pediatric Surgery, Department of Surgery, Nationwide Children's Hospital, The Ohio State University College of Medicine, Columbus, Ohio, USA.
¹⁴ Division of Pediatric Surgery, Centre Hospitalier Universitaire Ste-Justine, Université de Montréal, Montréal, Québec, Canada.
¹⁵ Division of Pediatric Surgery, Medical College of Wisconsin, Children's Wisconsin, Milwaukee, Wisconsin, USA.
¹⁶ Division of Pediatric Surgery, Riley Hospital for Children, Indiana University School of Medicine, Indianapolis, Indiana, USA.
¹⁷ Division of Pediatric Surgery, Valley Children's Hospital, Madera, California, USA.
¹⁸ Valley Children's Hospital, Madera, California, USA.
¹⁹ Pediatric Surgical Associates, Children's Minnesota, Minneapolis, Minnesota, USA.
²⁰ C.S. Mott Children's Hospital, University of Michigan, Ann Arbor, Michigan, USA.
²¹ College of Medicine, University of Florida, Gainesville, Florida, USA.
²² Department of Surgery, University of Florida, Gainesville, Florida, USA.
²³ Thoracic and Fetal Surgery, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.
²⁴ Seattle Children's Hospital, University of Washington, Seattle, Washington, USA.
²⁵ Department of Surgery, Oregon Health & Science University, Portland, Oregon, USA.
²⁶ Northwell Health, Cohen Children's Medical Center, New Hyde Park, New York, USA.
²⁷ Feinstein/Northwell, Cohen Children's Medical Center, Queens, New York, USA.
²⁸ Division of Pediatric Surgery, Department of Surgery, University of California Davis Children's Hospital, Sacramento, California, USA.
²⁹ Department of Surgery, University of California Davis, Sacramento, California, USA.
³⁰ Division of Pediatric Surgery, Children's Hospital, London Health Sciences Centre, London, Ontario, Canada.
³¹ Division of Pediatric Surgery, American Family Children's Hospital, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin, USA.
³² Department of Surgery, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin, USA.
³³ Division of Pediatric Surgery, SUNY Upstate Medical University, Syracuse, New York, USA.
³⁴ C.S. Mott Children's Hospital, Section of Pediatric Surgery, University of Michigan, Ann Arbor, Michigan, USA.
³⁵ Division of Pediatric Surgery, Westchester Medical Center, New York Medical College, Valhalla, New York, USA.
³⁶ New York Medical College, Valhalla, New York, USA.
³⁷ Department of Surgery, Mayo Clinic, Rochester, Minnesota, USA.
³⁸ Massachusetts General for Children, Boston, Massachusetts, USA.
³⁹ Division of Pediatric Surgery, Guerin Children's, Cedars-Sinai Medical Center, Los Angeles, California, USA.
⁴⁰ University of Texas, MD Anderson Cancer Center, Houston, Texas, USA.
⁴¹ Division of Pediatric Surgery, Department of Surgery, Icahn School of Medicine Mount Sinai, New York, New York, USA.
⁴² Department of Surgery, Icahn School of Medicine Mount Sinai, New York, New York, USA.
⁴³ Children's Health Children's Medical Center, University of Texas Southwestern, Dallas, Texas, USA.
⁴⁴ Division of Pediatric Surgery, UNC Children's Hospital, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, USA.

PMID: 40394815
DOI: 10.1002/pbc.31774

Multicenter Study

Contemporary Biology, Management, and Outcomes of Renal Medullary Carcinoma in Children and Adults: A Pediatric Surgical Oncology Research Collaborative Study

Catherine B Beckhorn et al. Pediatr Blood Cancer. 2025 Aug.

. 2025 Aug;72(8):e31774.

doi: 10.1002/pbc.31774. Epub 2025 May 20.

Authors

Affiliations

¹ Duke University School of Medicine, Durham, North Carolina, USA.
² Division of Pediatric General and Thoracic Surgery, UPMC Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania, USA.
³ Department of Pediatric Surgery, Vanderbilt University Medical Center, Nashville, Tennessee, USA.
⁴ Department of Surgery, Duke University Medical Center, Durham, North Carolina, USA.
⁵ Department of Surgery, Vanderbilt University Medical Center, Nashville, Tennessee, USA.
⁶ Division of Pediatric Surgery, Department of Surgery, University of Utah, Primary Children's Hospital, Salt Lake City, Utah, USA.
⁷ Division of Pediatric Surgery, Department of Surgery, University of Colorado School of Medicine, Surgical Oncology Program Children's Hospital Colorado, Aurora, Colorado, USA.
⁸ Division of Urology, Department of Surgery, University of Colorado School of Medicine, Surgical Oncology Program Children's Hospital Colorado, Aurora, Colorado, USA.
⁹ Children's Mercy Research Institute, Kansas City, Kansas, USA.
¹⁰ Division of Pediatric Surgery, Department of Surgery, Ann and Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA.
¹¹ Division of Pediatric Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
¹² Department of Surgery, St Jude Children's Research Hospital, Memphis, Tennessee, USA.
¹³ Division of Pediatric Surgery, Department of Surgery, Nationwide Children's Hospital, The Ohio State University College of Medicine, Columbus, Ohio, USA.
¹⁴ Division of Pediatric Surgery, Centre Hospitalier Universitaire Ste-Justine, Université de Montréal, Montréal, Québec, Canada.
¹⁵ Division of Pediatric Surgery, Medical College of Wisconsin, Children's Wisconsin, Milwaukee, Wisconsin, USA.
¹⁶ Division of Pediatric Surgery, Riley Hospital for Children, Indiana University School of Medicine, Indianapolis, Indiana, USA.
¹⁷ Division of Pediatric Surgery, Valley Children's Hospital, Madera, California, USA.
¹⁸ Valley Children's Hospital, Madera, California, USA.
¹⁹ Pediatric Surgical Associates, Children's Minnesota, Minneapolis, Minnesota, USA.
²⁰ C.S. Mott Children's Hospital, University of Michigan, Ann Arbor, Michigan, USA.
²¹ College of Medicine, University of Florida, Gainesville, Florida, USA.
²² Department of Surgery, University of Florida, Gainesville, Florida, USA.
²³ Thoracic and Fetal Surgery, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.
²⁴ Seattle Children's Hospital, University of Washington, Seattle, Washington, USA.
²⁵ Department of Surgery, Oregon Health & Science University, Portland, Oregon, USA.
²⁶ Northwell Health, Cohen Children's Medical Center, New Hyde Park, New York, USA.
²⁷ Feinstein/Northwell, Cohen Children's Medical Center, Queens, New York, USA.
²⁸ Division of Pediatric Surgery, Department of Surgery, University of California Davis Children's Hospital, Sacramento, California, USA.
²⁹ Department of Surgery, University of California Davis, Sacramento, California, USA.
³⁰ Division of Pediatric Surgery, Children's Hospital, London Health Sciences Centre, London, Ontario, Canada.
³¹ Division of Pediatric Surgery, American Family Children's Hospital, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin, USA.
³² Department of Surgery, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin, USA.
³³ Division of Pediatric Surgery, SUNY Upstate Medical University, Syracuse, New York, USA.
³⁴ C.S. Mott Children's Hospital, Section of Pediatric Surgery, University of Michigan, Ann Arbor, Michigan, USA.
³⁵ Division of Pediatric Surgery, Westchester Medical Center, New York Medical College, Valhalla, New York, USA.
³⁶ New York Medical College, Valhalla, New York, USA.
³⁷ Department of Surgery, Mayo Clinic, Rochester, Minnesota, USA.
³⁸ Massachusetts General for Children, Boston, Massachusetts, USA.
³⁹ Division of Pediatric Surgery, Guerin Children's, Cedars-Sinai Medical Center, Los Angeles, California, USA.
⁴⁰ University of Texas, MD Anderson Cancer Center, Houston, Texas, USA.
⁴¹ Division of Pediatric Surgery, Department of Surgery, Icahn School of Medicine Mount Sinai, New York, New York, USA.
⁴² Department of Surgery, Icahn School of Medicine Mount Sinai, New York, New York, USA.
⁴³ Children's Health Children's Medical Center, University of Texas Southwestern, Dallas, Texas, USA.
⁴⁴ Division of Pediatric Surgery, UNC Children's Hospital, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, USA.

PMID: 40394815
DOI: 10.1002/pbc.31774

Abstract

Background: Renal medullary carcinoma (RMC) is an aggressive tumor associated with sickle cell trait. Despite treatment advances for other rare renal tumors, RMC survival remains poor. We aimed to describe the contemporary management and survival of children and adults with RMC.

Procedure: In this multicenter retrospective cohort study, Pediatric Surgical Oncology Research Collaborative sites searched their databases for patients diagnosed with RMC (2000-2022). Descriptive statistics were calculated and survival analyses performed using Kaplan-Meier and Cox regression.

Results: Thirty-four patients with RMC were identified. Median age was 19 years (IQR: 15-28; range: 7-52). Most were male (24/34; 71%), Black (27/32; 84%), had sickle cell trait or disease (30/33; 91%), presented with metastatic disease (27/34; 79%), and were symptomatic at presentation (32/34; 94%). Median overall survival (OS) was 24 months from diagnosis (16 months for children, 28 months for adults, p = 0.6). Receipt of platinum-based chemotherapy (23/34; 68%) was associated with significantly higher OS than other regimens (35 vs. 5 months, p < 0.001). Nephrectomy (24/34; 71%) was associated with significantly improved OS compared with non-operative management (34 vs. 7 months, p = 0.001). Immunotherapy, targeted therapy, or radiation therapy were not associated with significant differences in OS, nor were age, sex, race, sickle cell status, SMARCB1/INI-1, stage, nephrectomy approach, retroperitoneal lymph node dissection, gross residual disease, margins, or tumor size.

Conclusions: RMC survival remains poor despite newer therapies. Nephrectomy and platinum-based chemotherapy should be considered in locally advanced and metastatic disease. Coordinated international cooperative group studies are needed to meaningfully improve RMC survival.

Keywords: pediatric cancer; pediatric surgical oncology; renal cancer; renal medullary carcinoma; sickle cell.

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References

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Contemporary Biology, Management, and Outcomes of Renal Medullary Carcinoma in Children and Adults: A Pediatric Surgical Oncology Research Collaborative Study

Affiliations

Contemporary Biology, Management, and Outcomes of Renal Medullary Carcinoma in Children and Adults: A Pediatric Surgical Oncology Research Collaborative Study

Authors

Affiliations

Abstract

References

Publication types

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LinkOut - more resources

Full Text Sources

Medical