Adrenal cortical tumors in children: factors associated with poor outcome

作者：Klein Justin D; Turner Christopher G; Gray Fabienne L; Yu David C; Kozakewich Harry P; Perez Atayde Antonio R; Voss Stephan D; Zurakowski David; Shamberger Robert C; Weldon Christopher B^*

来源：Journal of Pediatric Surgery, 2011, 46(6): 1201-1207.

DOI：10.1016/j.jpedsurg.2011.03.052

• Adrenal cortical carcinoma
• Treatment
• Pathology
• Outcome
• Prognosis
• Pediatric

Purpose: The purpose of this study was to evaluate recurrence and survival outcomes in pediatric adrenal cortical neoplasms.
Methods: A 90-year retrospective review of children with adrenal cortical neoplasms was performed using multivariate Cox regression analysis to identify factors associated with recurrence and tumor-related mortality.
Results: The evaluable cohort included 29 patients. Twenty-seven underwent resection. Twenty-two (81%) had localized disease, and 5 (19%) had locally advanced disease (all received chemotherapy and 2 of 5 were cured). Two patients presenting with metastatic disease died despite treatment. There were 4 recurrences; all patients died. Tumor-related mortality was 24% (7/29). Kaplan-Meier freedom from recurrence was 85% at 1 year (95% confidence interval, 75%-95%). Multivariate Cox regression revealed that older age (P = .01), higher mitotic rate (P = .005), and necrosis (P < .001) were independent predictors of tumor-related death. Higher mitotic rate (P = .007) and larger tumor size (P = .03) were significant predictors of tumor recurrence.
Conclusion: Risk factors for poor outcomes in patients with adrenocortical tumors include older age, higher mitotic rate, higher percent necrosis, and larger tumor size. Therefore, the presence of these factors may warrant consideration of adjuvant chemotherapy, even in the absence of advanced disease.

• 出版日期2011-6