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Surgical Management and Outcomes in Pediatric Adrenocortical Carcinoma: A Pediatric Surgical Oncology Research
Shachi Srivatsa1, Jennifer H Aldrink2, Md Rejuan Haque3
1Center 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.
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.
Insights
Complete surgical resection is key for treating pediatric adrenocortical carcinoma (ACC). Outcomes for metastatic ACC remain poor, and the role of lymph node dissection needs further study.
Area of Science:
- Pediatric Oncology
- Surgical Oncology
- Cancer Research
Background:
- Pediatric adrenocortical carcinoma (ACC) is a rare and aggressive malignancy.
- Optimal surgical strategies, including minimally invasive surgery (MIS) and the role of lymph node dissection, are not well-defined for pediatric ACC.
- The management of metastatic disease in pediatric ACC presents significant challenges.
Purpose of the Study:
- To evaluate surgical approaches for pediatric adrenocortical carcinoma (ACC).
- To assess the impact of minimally invasive surgery (MIS) versus open adrenalectomy.
- To investigate the role and outcomes associated with lymph node dissection in pediatric ACC.
Main Methods:
- Retrospective review of 69 pediatric patients (<18 years) with ACC treated between 2012 and 2022 across 22 institutions.
- Data included demographics, tumor characteristics, surgical approach (open vs. MIS), adjuvant therapy, and survival outcomes.
- Survival probabilities were analyzed using Kaplan-Meier methods.
Main Results:
- Minimally invasive surgery (MIS) was associated with smaller tumors and no tumor spillage compared to open adrenalectomy.
- Lymph node sampling was performed in 44% of open cases but not in MIS cases.
- Five-year overall survival was 65.9%, with significantly poorer outcomes for patients with metastatic disease (5-year OS: 11.9% vs. 84.5% for non-metastatic).
Conclusions:
- Complete surgical resection is crucial for achieving a cure in pediatric adrenocortical carcinoma (ACC).
- Outcomes for pediatric ACC patients with metastatic disease remain poor despite aggressive treatment.
- The limited performance and incomplete nature of lymph node dissection hinder definitive conclusions regarding its impact.
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