Surgical Management and Outcomes in Pediatric Adrenocortical Carcinoma: A Pediatric Surgical Oncology Research Collaborative Study

Shachi Srivatsa¹, Jennifer H Aldrink², Md Rejuan Haque³, Andrew Murphy⁴, Daniel Gehle⁴, Huma Halepota⁴, Erika A Newman⁵, Keyonna Williams⁵, Peter Mattei⁶, William R Johnston⁶, Rosa Hwang⁶, Timothy B Lautz⁷, Samantha A Ayala⁷, Nelson Piché⁸, Caroline P Lemoine⁸, Julia Debertin⁹, Stephanie F Polites⁹, David H Rothstein¹⁰, Kimberly J Riehle¹⁰, Nzuekoh Nchinda¹⁰, Marcus M Malek¹¹, Hannah N Rinehardt¹¹, Catherine Gestrich¹², Shannon L Castle¹³, Adriana Lopez¹⁴, Matthew S Mayes¹⁴, Emily K Myers¹⁵, Jonathan P Roach¹⁵, Brian T Craig¹⁶, Dave R Lal¹⁶, Jennifer Schuh¹⁶, Barrett P Cromeens¹⁷, Sindhu V Mannava¹⁷, Mary T Austin¹⁸, Lauren K Mayon¹⁸, Zachary J Kastenberg¹⁹, Marshall Wallace¹⁹, Abigail Alexander¹⁹, Michael A Stellon²⁰, Hau D Le²⁰, Devashish S Joshi²⁰, Charbel Chidiac²¹, Daniel S Rhee²¹, Danielle Cameron²², Alyssa Stetson²², Barrie S Rich²³, Richard D Glick²³, Elizabeth A Fialkowski²⁴, Kathryn Fowler²⁴, Rukaya Fareh²⁴, Erin G Brown²⁵, Kathleen Doyle²⁶, Paige Abril²⁶, Christa Grant²⁷, Roshni Dasgupta²⁸, Chloé Boehmer²⁸, Sara A Mansfield²⁹

Affiliations

¹ Center for Surgical Outcomes and Research, Abigail Wexner Research Institute, Division of Pediatric Surgery, Department of Surgery, Nationwide Children's Hospital, The Ohio State University College of Medicine, Columbus, OH, USA.
² Division of Pediatric Surgery, Department of Surgery, Nationwide Children's Hospital, The Ohio State University College of Medicine, Columbus, OH, USA.
³ Department of Biomedical Informatics, The Ohio State University, Columbus, OH, USA.
⁴ Department of Surgery, St Jude Children's Research Hospital, Memphis, TN, USA.
⁵ C.S. Mott Children's Hospital, University of Michigan, Ann Arbor, MI, USA.
⁶ General, Thoracic and Fetal Surgery, Children's Hospital of Philadelphia, Philadelphia, PA, USA.
⁷ Department of Surgery, Division of Pediatric Surgery, Lurie Children's Hospital, Northwestern School of Medicine, Chicago, IL, USA.
⁸ Division of Pediatric Surgery, Centre Hospitalier Universitaire Ste-Justine, Université de Montréal, Montréal, Canada.
⁹ Department of Surgery, Mayo Clinic, Rochester, MN, USA.
¹⁰ Seattle Children's Hospital, University of Washington, Seattle, WA, USA.
¹¹ Division of Pediatric General and Thoracic Surgery, University of Pittsburgh Medical Center (UPMC) Children's Hospital of Pittsburgh, Pittsburgh, PA, USA.
¹² Division of Pathology, University of Pittsburgh Medical Center (UPMC) Children's Hospital of Pittsburgh, Pittsburgh, PA, USA.
¹³ Division of Pediatric Surgery, Valley Children's Hospital, Madera, CA, USA.
¹⁴ Valley Children's Hospital, Madera, CA, USA.
¹⁵ Division of Pediatric Surgery, Department of Surgery, University of Colorado School of Medicine, Children's Hospital of Colorado, Aurora, CO, USA.
¹⁶ Division of Pediatric Surgery, Medical College of Wisconsin, Children's Wisconsin, Milwaukee, WI, USA.
¹⁷ Division of Pediatric Surgery, Riley Hospital for Children, Indiana University School of Medicine, Indianapolis, IN, USA.
¹⁸ MD Anderson Cancer Center, University of Texas, Houston, TX, USA.
¹⁹ Department of Surgery, Division of Pediatric Surgery, University of Utah, Primary Children's Hospital, Salt Lake City, UT, USA.
²⁰ Division of Pediatric Surgery, American Family Children's Hospital, School of Medicine and Public Health, University of Wisconsin, Madison, WI, USA.
²¹ Division of Pediatric Surgery, Department of Surgery, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
²² Massachusetts General Hospital, Boston, MA, USA.
²³ Feinstein/Northwell, Cohen Children's Medical Center, Queens, NY, USA.
²⁴ Department of Surgery, Oregon Health & Science University, Portland, OR, USA.
²⁵ Division of Pediatric Surgery, Department of Surgery, University of California Davis Children's Hospital, Sacramento, CA, USA.
²⁶ Department of Surgery, University of California Davis, Sacramento, CA, USA.
²⁷ New York Medical College, Valhalla, NY, USA.
²⁸ Division of Pediatric Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
²⁹ Division of Pediatric Surgery, Department of Surgery, Nationwide Children's Hospital, The Ohio State University College of Medicine, Columbus, OH, USA. Sara.mansfield@nationwidechildrens.org.

PMID: 41266918
DOI: 10.1245/s10434-025-18731-6

Surgical Management and Outcomes in Pediatric Adrenocortical Carcinoma: A Pediatric Surgical Oncology Research Collaborative Study

Shachi Srivatsa et al. Ann Surg Oncol. 2025.

. 2025 Nov 20.

doi: 10.1245/s10434-025-18731-6. Online ahead of print.

Authors

Affiliations

¹ Center for Surgical Outcomes and Research, Abigail Wexner Research Institute, Division of Pediatric Surgery, Department of Surgery, Nationwide Children's Hospital, The Ohio State University College of Medicine, Columbus, OH, USA.
² Division of Pediatric Surgery, Department of Surgery, Nationwide Children's Hospital, The Ohio State University College of Medicine, Columbus, OH, USA.
³ Department of Biomedical Informatics, The Ohio State University, Columbus, OH, USA.
⁴ Department of Surgery, St Jude Children's Research Hospital, Memphis, TN, USA.
⁵ C.S. Mott Children's Hospital, University of Michigan, Ann Arbor, MI, USA.
⁶ General, Thoracic and Fetal Surgery, Children's Hospital of Philadelphia, Philadelphia, PA, USA.
⁷ Department of Surgery, Division of Pediatric Surgery, Lurie Children's Hospital, Northwestern School of Medicine, Chicago, IL, USA.
⁸ Division of Pediatric Surgery, Centre Hospitalier Universitaire Ste-Justine, Université de Montréal, Montréal, Canada.
⁹ Department of Surgery, Mayo Clinic, Rochester, MN, USA.
¹⁰ Seattle Children's Hospital, University of Washington, Seattle, WA, USA.
¹¹ Division of Pediatric General and Thoracic Surgery, University of Pittsburgh Medical Center (UPMC) Children's Hospital of Pittsburgh, Pittsburgh, PA, USA.
¹² Division of Pathology, University of Pittsburgh Medical Center (UPMC) Children's Hospital of Pittsburgh, Pittsburgh, PA, USA.
¹³ Division of Pediatric Surgery, Valley Children's Hospital, Madera, CA, USA.
¹⁴ Valley Children's Hospital, Madera, CA, USA.
¹⁵ Division of Pediatric Surgery, Department of Surgery, University of Colorado School of Medicine, Children's Hospital of Colorado, Aurora, CO, USA.
¹⁶ Division of Pediatric Surgery, Medical College of Wisconsin, Children's Wisconsin, Milwaukee, WI, USA.
¹⁷ Division of Pediatric Surgery, Riley Hospital for Children, Indiana University School of Medicine, Indianapolis, IN, USA.
¹⁸ MD Anderson Cancer Center, University of Texas, Houston, TX, USA.
¹⁹ Department of Surgery, Division of Pediatric Surgery, University of Utah, Primary Children's Hospital, Salt Lake City, UT, USA.
²⁰ Division of Pediatric Surgery, American Family Children's Hospital, School of Medicine and Public Health, University of Wisconsin, Madison, WI, USA.
²¹ Division of Pediatric Surgery, Department of Surgery, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
²² Massachusetts General Hospital, Boston, MA, USA.
²³ Feinstein/Northwell, Cohen Children's Medical Center, Queens, NY, USA.
²⁴ Department of Surgery, Oregon Health & Science University, Portland, OR, USA.
²⁵ Division of Pediatric Surgery, Department of Surgery, University of California Davis Children's Hospital, Sacramento, CA, USA.
²⁶ Department of Surgery, University of California Davis, Sacramento, CA, USA.
²⁷ New York Medical College, Valhalla, NY, USA.
²⁸ Division of Pediatric Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
²⁹ Division of Pediatric Surgery, Department of Surgery, Nationwide Children's Hospital, The Ohio State University College of Medicine, Columbus, OH, USA. Sara.mansfield@nationwidechildrens.org.

PMID: 41266918
DOI: 10.1245/s10434-025-18731-6

Abstract

Background: Pediatric adrenocortical carcinoma (ACC) is a rare and aggressive cancer. The appropriateness of minimally invasive surgery (MIS), role of nodal dissection, and aggressiveness of surgery in patients with metastatic disease remain poorly understood.

Patients and methods: We performed a retrospective review of patients < 18 years with ACC treated from 2012 to 2022 at 22 institutions participating in the Pediatric Surgical Oncology Research Collaborative. Data collected included demographics, clinical presentation, genetic predisposition, tumor characteristics, surgical approach, adjuvant treatment, and survival outcome. Survival probabilities were estimated with Kaplan-Meier methods.

Results: In all, 69 patients were included (median age: 8.4 years; 58% female), and 28% had Li-Fraumeni syndrome. At diagnosis, 36% had Stage IV disease and 28% had Stage I disease. A total of 55 (83%) patients underwent open adrenalectomy, and 11 (17%) underwent MIS. MIS approaches were employed for significantly smaller tumors (median: 4.4 cm, 38 g) compared with open cases (median: 9.6 cm, 246 g; p < 0.001), and none had tumor spillage (versus 22% in open cases; p = 0.087). Lymph node sampling was performed in 44% of open cases (mean yield: 2.96); no lymph nodes were sampled in MIS cases (p = 0.008). The 5 year overall survival (OS) for the entire cohort was 65.9%, and 5-year event-free survival (EFS) was 54.1%. Patients without metastases had significantly better survival than those with more than five metastatic lesions (5 year OS: 84.5% versus 11.9%; p < 0.001).

Conclusions: Complete surgical resection remains paramount for cure in children with ACC. Despite aggressive attempts at tumor clearance, outcomes in children with metastatic disease remain poor. Nodal dissection remains infrequently and incompletely performed, limiting conclusions on impact.

Keywords: Adrenal tumor; Adrenocortical carcinoma; Li–Fraumeni syndrome; Minimally invasive surgery; Outcomes; Pediatric.

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Conflict of interest statement

Disclosure: None.

References

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Surgical Management and Outcomes in Pediatric Adrenocortical Carcinoma: A Pediatric Surgical Oncology Research Collaborative Study

Affiliations

Surgical Management and Outcomes in Pediatric Adrenocortical Carcinoma: A Pediatric Surgical Oncology Research Collaborative Study

Authors

Affiliations

Abstract

Conflict of interest statement

References

LinkOut - more resources

Full Text Sources

Research Materials