Clinical and molecular aspects of a pediatric metachronous adrenocortical tumor

Lima, Lorena de Oliveira; Lerário, Antônio Marcondes; Alencar, Guilherme Asmar; Brito, Luciana Pinto; Almeida, Madson Q.; Domenice, Sorahia; Latrônico, Ana Claudia; Mendonca, Berenice B.; Fragoso, Maria Candida B. V.

doi:10.1590/s0004-27302011000100010

Cited by 9 publications

(7 citation statements)

References 19 publications

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“…Metastases were diagnosed by systematic imaging investigations mainly abdominal and chest computed tomography (CT) scans; bone scintigraphy in appropriated cases, magnetic resonance imaging, and more recently in selected cases with positron emission CT scan. All patients were submitted to unilateral adrenalectomy except one pediatric patient who presented a bilateral asynchronous ACT (15). Histopathological diagnosis, tumor weight, size, and Weiss scores were determined for each tumor specimen.…”

Section: Methodsmentioning

confidence: 99%

Combined expression of BUB1B, DLGAP5, and PINK1 as predictors of poor outcome in adrenocortical tumors: validation in a Brazilian cohort of adult and pediatric patients

Fragoso¹,

Almeida²,

Mazzuco³

et al. 2012

European Journal of Endocrinology

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Background: A recent microarray study identified a set of genes whose combined expression patterns were predictive of poor outcome in a cohort of adult adrenocortical tumors (ACTs). The difference between the expression values measured by qRT-PCR of DLGAP5 and PINK1 genes was the best molecular predictor of recurrence and malignancy. Among the adrenocortical carcinomas, the combined expression of BUB1B and PINK1 genes was the most reliable predictor of overall survival. The prognostic and molecular heterogeneity of ACTs raises the need to study the applicability of these molecular markers in other cohorts. Objective: To validate the combined expression of BUB1B, DLGAP5, and PINK1 as outcome predictor in ACTs from a Brazilian cohort of adult and pediatric patients. Patients and methods: BUB1B, DLGAP5, and PINK1 expression was assessed by quantitative PCR in 53 ACTs from 52 patients -24 pediatric and 28 adults (one pediatric patient presented a bilateral asynchronous ACT). Results: DLGAP5-PINK1 and BUB1B-PINK1 were strong predictors of disease-free survival and overall survival, respectively, among adult patients with ACT. In the pediatric cohort, these molecular predictors were only marginally associated with disease-free survival but not with overall survival. Conclusion: This study confirms the prognostic value of the combined expression of BUB1B, DLGAP5, and PINK1 genes in a Brazilian group of adult ACTs. Among pediatric ACTs, other molecular predictors of outcome are required.

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Section: Methodsmentioning

confidence: 99%

Combined expression of BUB1B, DLGAP5, and PINK1 as predictors of poor outcome in adrenocortical tumors: validation in a Brazilian cohort of adult and pediatric patients

Fragoso¹,

Almeida²,

Mazzuco³

et al. 2012

European Journal of Endocrinology

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“…The occurrence of ACC in multiple family members has also been well recognized, especially in LFS families. Bilateral or multiple primary ACCs occur in roughly 1% of ACC patients and have been described in the setting of hereditary syndromes (Bilimoria et al 2008; Griniatsos et al 2011; Lima Lde et al 2011). Other primary cancers were observed in 44 of 423 patients (10.4%) in the German registry, including four patients with two other primary malignancies; in the study of Nader et al 6 of 77 (9.1%) patients had at least one other primary malignancy and one patient had two other primary tumors (Fassnacht et al 2010; Nader et al 1983).…”

Section: Hereditary Aspects Of Adrenocortical Carcinomamentioning

confidence: 99%

Association of adrenocortical carcinoma with familial cancer susceptibility syndromes

Else

2012

Molecular and Cellular Endocrinology

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Our knowledge about inherited susceptibility to adrenocortical carcinoma (ACC) almost exclusively stems from experiences with familial cancer susceptibility syndromes, which are caused by single gene mutations (e.g. Li-Fraumeni syndrome (LFS)). Population-based studies are largely unavailable. ACC diagnosed during childhood is known to be commonly part of hereditary cancer syndromes. Childhood ACC is part of the classical tumor spectrum of LFS and Beckwith-Wiedemann syndrome (BWS). In adults ACC has been reported in patients with multiple endocrine neoplasia (MEN1), familial adenomatous polyposis coli (FAP) and neurofibromatosis type 1 (NF1). However, the evidence associating ACC with these syndromes is less well substantiated. Here, we will review the evidence for genetic predisposition in general and the association with known familial cancer susceptibility syndromes in particular. We will also review current recommendations regarding screening and surveillance of these patients as they apply to a specialized ACC or endocrine cancer clinic.

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“…Weiss's study also analyzed the expression of several cell cycle proteins, including Ki-67, bcl-2, p53, mdm-s, p21 and p27, but a significant predictive value for metastatic disease could not be identified. To overcome the limitations of the Weiss score, many researchers have recently searched for immunohistochemical markers of malignancy, prognostic factors and therapeutic targets (5, 17–19). In the present study, we investigated the diagnostic role of PSMA in a large series of ACCs.…”

Section: Discussionmentioning

confidence: 99%

Diagnostic Role of Prostate-Specific Membrane Antigen in Adrenocortical Carcinoma

Dou

et al. 2019

Front. Endocrinol.

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Objective: To investigate the role of PSMA in the differential diagnosis of adrenocortical carcinoma samples (ACCs) and adrenocortical adenoma samples (ACAs), to validate the prognostic role of PSMA in patients with ACCs, and to explore the possibility that this marker can differentiate localized ACCs from adrenal metastases from other sites. Methods: PSMA protein expression in tissue samples from 50 ACCs, 90 ACAs (including 20 from patients who presented with Cushing's syndrome, 20 aldosterone-producing adenomas and 50 non-functional tumors) and 10 tissues that were metastases from other primary sites was assessed by immunohistochemistry. The clinical and pathological characteristics were compared, the intensity and density were analyzed, and the prognostic role was evaluated. Results: The analysis of clinical and pathological features revealed that the size of ACCs was greater than that of benign tissues and the ACC patients were older than the ACA patients ( p < 0.01). The percentage of PSMA-positive vessels, the mean intensity and the degree of staining density were found to be significantly lower in ACAs than in ACCs ( p < 0.01). In these 140 samples, 60% of the ACCs were grouped in the positive category. The samples were negative for metastases that were from other primary sites. The ENSAT stage and Ki-67 were correlated with PSMA expression. The survival distribution revealed that high PSMA expression did not show any prognostic relevance in the current ACCs series. Those samples with a score of > 3.5 were 75 times more likely to be malignant (OR = 75). We established a cut-off score of 3.5 ( p < 0.05), which had 46% sensitivity and 99% specificity. Paralleling PSMA and Ki-67 maximized the area under the curve, with 72% sensitivity and 100% specificity. Conclusions: Our results strongly confirm that PSMA is helpful for distinguishing benign from malignant tumors and that its high expression levels correlate with a high ENSAT stage and high proliferation. The combination of PSMA and Ki-67 can be particularly useful. Furthermore, PSMA might be a useful tool for the identification of localized adrenal carcinoma and metastatic carcinoma.

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Clinical and molecular aspects of a pediatric metachronous adrenocortical tumor

Cited by 9 publications

References 19 publications

Combined expression of BUB1B, DLGAP5, and PINK1 as predictors of poor outcome in adrenocortical tumors: validation in a Brazilian cohort of adult and pediatric patients

Combined expression of BUB1B, DLGAP5, and PINK1 as predictors of poor outcome in adrenocortical tumors: validation in a Brazilian cohort of adult and pediatric patients

Association of adrenocortical carcinoma with familial cancer susceptibility syndromes

Diagnostic Role of Prostate-Specific Membrane Antigen in Adrenocortical Carcinoma

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