title: Recurrence and mortality prognostic factors in childhood adrenocortical tumors: Analysis from the Brazilian National Institute of Cancer experience.
abstract: Prognostic markers that can help identifying precocious risk of unfavorable outcomes in patients with childhood adrenocortical tumors (ACTs) are still unclear. This observational and retrospective study aimed to identify clinical and pathology prognostic factors of recurrence and death in a tertiary cancer center population. Clinical, pathology, demographic, staging, and therapy data from patients with childhood ACT (median age: 3.6 years) treated at the Brazilian National Institute of Cancer between 1997 and 2015 were assessed. Univariate and bivariate analyses were used to study the association of clinical and pathology characteristics with recurrence and mortality. Recurrence and disease-related mortality were the main outcomes. Twenty-seven patients were included. Complete tumor resection was performed in 21 cases. The median tumor size was 8.2 cm. Mitotane was the most common adjuvant/palliative therapy (n = 13). Recurrence occurred in 6 patients, after a median time of 7.2 months, and was more common among those with larger tumors (P =.008), higher Weiss score (P =.001), and microscopic tumoral necrosis (P =.002). Ten patients died from the disease. Older age (P =.04), larger tumor size (P =.002), metastatic disease (P =.003), previous recurrence (P =.003), incomplete resection (P =.002), intraoperative tumor spillage (P =.005), higher Weiss score (P =.03), microscopic necrosis (P =.005), and capsular invasion (P =.02) were all associated with increased death risk. Even though complete tumor resection was performed in most cases, a considerable number of cases of childhood ACT resulted in recurrence and death. Early identification of unfavorable outcomes is essential to determine ideal therapy and appropriate surveillance.
authors.affiliation: a Endocrine Oncology Unit, Brazilian National Institute of Cancer-INCA , Rio de Janeiro, Rio de Janeiro , Brazil.|c Endocrinology Section, Federal Hospital of Lagoa , Rio de Janeiro, Rio de Janeiro , Brazil.
authors.forename: Daniel
authors.initials: D
authors.lastname: Bulzico
authors.affiliation: b Division of Pathology, Brazilian National Institute of Cancer-INCA , Rio de Janeiro, Rio de Janeiro , Brazil.
authors.forename: Paulo Antônio Silvestre
authors.initials: PA
authors.lastname: de Faria
authors.affiliation: c Endocrinology Section, Federal Hospital of Lagoa , Rio de Janeiro, Rio de Janeiro , Brazil.
authors.forename: Marcela Pessoa
authors.initials: MP
authors.lastname: de Paula
authors.affiliation: a Endocrine Oncology Unit, Brazilian National Institute of Cancer-INCA , Rio de Janeiro, Rio de Janeiro , Brazil.
authors.forename: Maria Alice Neves
authors.initials: MA
authors.lastname: Bordallo
authors.affiliation: a Endocrine Oncology Unit, Brazilian National Institute of Cancer-INCA , Rio de Janeiro, Rio de Janeiro , Brazil.
authors.forename: Cencita H C N
authors.initials: CH
authors.lastname: Pessoa
authors.affiliation: a Endocrine Oncology Unit, Brazilian National Institute of Cancer-INCA , Rio de Janeiro, Rio de Janeiro , Brazil.|d Nuclear Medicine Section, Medical School and Clementino Fraga Filho University Hospital, Rio de Janeiro Federal University , Rio de Janeiro, Rio de Janeiro , Brazil.
authors.forename: Rossana
authors.initials: R
authors.lastname: Corbo
authors.affiliation: e Department of Pediatric Oncology , Brazilian National Institute of Cancer-INCA , Rio de Janeiro , Rio de Janeiro , Brazil.
authors.forename: Sima
authors.initials: S
authors.lastname: Ferman
authors.affiliation: f Department of Internal Medicine and Endocrinology Section , Medical School and Clementino Fraga Filho University Hospital, Rio de Janeiro Federal University , Rio de Janeiro, Rio de Janeiro , Brazil.
authors.forename: Mario
authors.initials: M
authors.lastname: Vaisman
authors.affiliation: c Endocrinology Section, Federal Hospital of Lagoa , Rio de Janeiro, Rio de Janeiro , Brazil.|f Department of Internal Medicine and Endocrinology Section , Medical School and Clementino Fraga Filho University Hospital, Rio de Janeiro Federal University , Rio de Janeiro, Rio de Janeiro , Brazil.
authors.forename: Leonardo Vieira
authors.initials: LV
authors.lastname: Neto
mesh_terms: D000293:Adolescent; D000306:Adrenal Cortex Neoplasms / Q000188:drug therapy / Q000401:mortality* / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000453:epidemiology*; D011379:Prognosis; D012189:Retrospective Studies
keywords: Adrenocortical carcinoma; cancer staging; pediatric adrenal tumors; prognosis