Factors associated with survival in pediatric adrenocortical carcinoma: An analysis of the National Cancer Data Base (NCDB).

Journal Article (Journal Article)

PURPOSE: Adrenocortical carcinoma (ACC) is a rare tumor in children with important distinctions from the adult disease. We reviewed the National Cancer Data Base (NCDB) to determine factors associated with long-term survival. METHODS: The NCDB was queried for patients less than 18 years of age who were diagnosed with ACC between 1998 and 2011. Kaplan-Meier analysis was utilized to determine factors significantly associated with overall survival. RESULTS: A total of 111 patients were included (median age: 4 years, 69% female). ACC was more common in the youngest cohort, with 48% of cases occurring in children younger than the age of 3. Median tumor size was 9.5 cm (IQR: 6.5-13.0), and 87% of patients underwent some form of surgical resection. Among children with available data, 19 of 62 presented with metastases. Overall 1- and 3-year survival was 70% and 64%, respectively. Age, tumor size, extension of tumor into surrounding tissue, and metastatic disease were all found to be significantly associated with survival. Among patients who underwent a surgical procedure, margin status was also found to be significantly associated with survival. CONCLUSION: Age, tumor size, extension of tumor, metastatic disease, and margin status are significantly associated with long-term survival in children with adrenocortical carcinoma.

Full Text

Duke Authors

Cited Authors

  • Gulack, BC; Rialon, KL; Englum, BR; Kim, J; Talbot, LJ; Adibe, OO; Rice, HE; Tracy, ET

Published Date

  • January 2016

Published In

Volume / Issue

  • 51 / 1

Start / End Page

  • 172 - 177

PubMed ID

  • 26572849

Pubmed Central ID

  • PMC5131646

Electronic International Standard Serial Number (EISSN)

  • 1531-5037

Digital Object Identifier (DOI)

  • 10.1016/j.jpedsurg.2015.10.039


  • eng

Conference Location

  • United States