title: Factors associated with survival in pediatric adrenocortical carcinoma: An analysis of the National Cancer Data Base (NCDB).
abstract: OBJECTIVE
  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.
  
  
  CONCLUSIONS
  Age, tumor size, extension of tumor, metastatic disease, and margin status are significantly associated with long-term survival in children with adrenocortical carcinoma.
authors.affiliation: Department of Surgery, Duke University Medical Center, Durham, NC. Electronic address: brian.gulack@duke.edu.
authors.forename: Brian C
authors.initials: BC
authors.lastname: Gulack
authors.affiliation: Department of Surgery, Duke University Medical Center, Durham, NC. Electronic address: Kristy.rialon@duke.edu.
authors.forename: Kristy L
authors.initials: KL
authors.lastname: Rialon
authors.affiliation: Department of Surgery, Duke University Medical Center, Durham, NC.
authors.forename: Brian R
authors.initials: BR
authors.lastname: Englum
authors.affiliation: Department of Surgery, Duke University Medical Center, Durham, NC. Electronic address: jina.kim1@duke.edu.
authors.forename: Jina
authors.initials: J
authors.lastname: Kim
authors.affiliation: Department of Surgery, Duke University Medical Center, Durham, NC. Electronic address: Lindsay.jones@duke.edu.
authors.forename: Lindsay J
authors.initials: LJ
authors.lastname: Talbot
authors.affiliation: Department of Surgery, Duke University Medical Center, Durham, NC. Electronic address: Obinna.adibe@duke.edu.
authors.forename: Obinna O
authors.initials: OO
authors.lastname: Adibe
authors.affiliation: Department of Surgery, Duke University Medical Center, Durham, NC. Electronic address: henry.rice@duke.edu.
authors.forename: Henry E
authors.initials: HE
authors.lastname: Rice
authors.affiliation: Department of Surgery, Duke University Medical Center, Durham, NC. Electronic address: Elisabeth.tracy@duke.edu.
authors.forename: Elisabeth T
authors.initials: ET
authors.lastname: Tracy
mesh_terms: D000293:Adolescent; D000306:Adrenal Cortex Neoplasms / Q000401:mortality* / Q000473:pathology; D018268:Adrenocortical Carcinoma / Q000401:mortality* / Q000473:pathology; D000367:Age Factors; D002648:Child; D002675:Child, Preschool; D016208:Databases, Factual; D005260:Female; D006801:Humans; D007223:Infant; D053208:Kaplan-Meier Estimate; D008297:Male; D009361:Neoplasm Invasiveness; D009362:Neoplasm Metastasis; D009367:Neoplasm Staging; D011379:Prognosis; D015996:Survival Rate; D014481:United States / Q000453:epidemiology
keywords: Adrenocortical carcinoma; Pediatric adrenal tumors; Pediatric cancer staging; Prognostic factors