Faculty Collaboration Database

Surgical Management and Outcomes in Pediatric Adrenocortical Carcinoma: A Pediatric Surgical Oncology Research Collaborative Study. Ann Surg Oncol 2026 Mar;33(3):2712-2728

Date

11/21/2025

Pubmed ID

41266918

DOI

10.1245/s10434-025-18731-6

Scopus ID

2-s2.0-105022591275 (requires institutional sign-in at Scopus site)   1 Citation

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.

Author List

Srivatsa S, Aldrink JH, Haque MR, Murphy A, Gehle D, Halepota H, Newman EA, Williams K, Mattei P, Johnston WR, Hwang R, Lautz TB, Ayala SA, Piché N, Lemoine CP, Debertin J, Polites SF, Rothstein DH, Riehle KJ, Nchinda N, Malek MM, Rinehardt HN, Gestrich C, Castle SL, Lopez A, Mayes MS, Myers EK, Roach JP, Craig BT, Lal DR, Schuh J, Cromeens BP, Mannava SV, Austin MT, Mayon LK, Kastenberg ZJ, Wallace M, Alexander A, Stellon MA, Le HD, Joshi DS, Chidiac C, Rhee DS, Cameron D, Stetson A, Rich BS, Glick RD, Fialkowski EA, Fowler K, Fareh R, Brown EG, Doyle K, Abril P, Grant C, Dasgupta R, Boehmer C, Mansfield SA

Authors

Brian T. Craig MD Assistant Professor in the Surgery department at Medical College of Wisconsin
Dave Lal MPH, MD Chief, Professor in the Surgery department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Adolescent
Adrenal Cortex Neoplasms
Adrenalectomy
Adrenocortical Carcinoma
Child
Child, Preschool
Female
Follow-Up Studies
Humans
Infant
Lymph Node Excision
Male
Minimally Invasive Surgical Procedures
Prognosis
Retrospective Studies
Survival Rate