Adrenal cortical tumors in children: factors associated with poor outcome

详细信息    查看全文

• 作者：Justin D. Klein^a ; Christopher G. Turner^a ; Fabienne L. Gray^a ; David C. Yu^a ; ^b ; Harry P. Kozakewich^c ; Antonio R. Perez-Atayde^c ; Stephan D. Voss^d ; David Zurakowski^a ; Robert C. Shamberger^a ; Christopher B. Weldon^a ; ^{christopher.weldon@childrens.harvard.edu"" rel=""nofollow}
• 关键词：Adrenal cortical carcinoma ; Treatment ; Pathology ; Outcome ; Prognosis ; Pediatric
• 刊名：Journal of Pediatric Surgery
• 出版年：2011
• 出版时间：June 2011
• 年：2011
• 卷：46
• 期：6
• 页码：1201-1207
• 全文大小：318 K

文摘

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.