RESUMO
Adrenocortical carcinoma (ACC) is a rare and lethal disease with a poor prognosis. This study aims to share our 41-year experience as a referral center, focusing on identifying risk factors associated with ACC mortality. Our retrospective analysis included a cohort of 150 adult patients with ACC in all stage categories, treated between 1981 and 2022. Tumor hormonal hypersecretion was observed in 78.6% of the patients, and the median age of diagnosis was 40 years. The majority presented as European Network for the Study of Adrenal Tumors (ENSAT) III or IV (22.9% and 31.2%, respectively), and the overall mortality rate was 54.6%. Independent predictors of death were elevated secretion of cortisol (HR = 2.0), androstenedione (HR = 2.2), estradiol (HR = 2.8), 17-OH progesterone (HR = 2.0), and 11-deoxycortisol (HR = 5.1), higher Weiss (HR = 4.3), modified Weiss (HR = 4.4), and Helsinki scores (HR = 12.0), advanced ENSAT stage (HR = 27.1), larger tumor size (HR = 2.7), higher Ki-67 percentage (HR = 2.3), and incomplete surgical resection (HR = 2.5). Mitosis greater than 5/50 high-power field (HR = 5.6), atypical mitosis (HR = 2.3), confluent necrosis (HR = 15.4), venous invasion (HR = 2.8), and capsular invasion (HR = 2.4) were also identified as independent predictors of death. Knowing the risk factors for ACC's mortality may help determine the best treatment option.
RESUMO
Thirty-two adrenocortical neoplasms in children and adolescents were evaluated for prognostic factors including clinical and morphological parameters and DNA ploidy. The patients were segregated into two groups according to clinical outcome: group A, represented by patients with clinically benign neoplasms (n = 15), and group B, patients with clinically malignant tumors as evidenced by local recurrence, metastases, or fatal outcome (n = 17). Clinical and morphological parameters in these two groups were evaluated using appropriate statistical methods. Parameters with a significant predictive value in terms of prognosis were age [p = .04], tumor size (p = .0003), median tumor weight (p = .0001), mitotic count (p = 0.04), and 25% tumor necrosis or more (p = .03). Twenty-three cases were studied for DNA ploidy: 10 cases by image analysis and 13 by both image analysis and flow cytometry. By ploidy analysis, 17 of 23 cases-12 of 14 in group A and 5 of 9 in group B-were found to be aneuploid. Multiple aneuploid peaks were found in 5 of 23 cases-4 of 14 cases in group A and 1 of 9 cases in group B. In tumors studied by both image analysis and flow cytometry, there was no discrepancy between results of ploidy analysis. There was no statistically significant association demonstrated between clinical outcome and DNA ploidy pattern. DNA ploidy heterogeneity, characterized by multiple aneuploid populations of cells, was also detected in both benign and malignant neoplasms. Based on our results, aneuploidy is relatively frequent in pediatrie adrenocortical tumors and does not appear to have predictive value for biological behavior.
RESUMO
Os autores apresentam um caso de leiomiossarcoma de abdômen de grandes proporçöes. Pela localizaçäo e pelos achados ultra-sonográficos e tomográficos pensou-se, inicialmente, tratar-se de um tumor pancreático. O diagnóstico foi feito na cirurgia, que revelou ausência de relaçäo do tumor com o pâncreas, ao qual estava apenas aderido
