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.

Outcome and prognostic factors in high‐risk childhood adrenocortical carcinomas: A report from the European Cooperative Study Group on Pediatric Rare Tumors (EXPeRT)

“…observed that large size and heterogeneous structure often coincide with the malignant nature of the tumors. Although tumor size is assessed with different units of measures by different authors (volume, diameter, or weight), the concept that huge ACCs have a worse prognosis is shared by most (Supplementary Table S1) …”

“…13 The Interna- Supplementary Table S1). 1,14,19,20,27,29,30 At diagnosis, most ACCs occurring in children are localized and can be completely resected. 14,20 An initial surgical excision in our series was attempted in most cases (66/82), but the complete resection was possible only in 37.8% of the whole population (31/82) and in 45.2% of the patients with localized disease (28/62), confirming the difficulty in excising these tumors.…”

Clinical prognostic factors in pediatric adrenocortical tumors: A meta‐analysis

“…Thirty‐six papers 2,5,16–18,20–47,49,50 allowed the evaluation of gender effect on outcome: females were found to have RR of 1.21, without reaching a significance ( P = .14; 95% CI 0.94‐1.55; I 2 0%).…”

Actual 10‐year survivors following resection of adrenocortical carcinoma

“…In fact, to date, only eight case reports and one small case series of six patients in the literature have reported on patients with ACC who have achieved long‐term survival of more than 10 years . Of note, various clinical and pathologic factors such as tumor size , lymphadenectomy , major venous involvement , margin status , and higher Weiss score have not been consistently associated with long‐term survival following resection of ACC. Therefore, the objective of the current study was to use a multi‐institutional collaborative database of ACC patients in the United States to identify characteristics of actual 5‐ and 10‐year survivors following resection of ACC.…”