Weineke criteria, Ki-67 index and p53 status to study pediatric adrenocortical tumors: Is there a correlation?

Das, Satadal; Sengupta, Moumita; Islam, Nelofar; Roy, Paromita; Datta, Chhanda; Mishra, Prafulla Kumar; Banerjee, Sugato; Chaudhuri, Manoj Kumar; Chatterjee, Uttara

doi:10.1016/j.jpedsurg.2016.07.014

Cited by 48 publications

(45 citation statements)

References 32 publications

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“…Validity of the Wieneke index was further demonstrated by Chatterjee et al [1, 41], in a group of 13 children with ACT (mean age 2.9 years). Applying the Wieneke index, 6 patients were assigned to benign, 1 to the intermediate, and 6 to the malignant pathology group, which was eventually consistent with the clinical behavior of these tumors.…”

Section: Discussionmentioning

confidence: 85%

Adrenocortical Carcinoma in Children: A Clinicopathological Analysis of 41 Patients at the Mayo Clinic from 1950 to 2017

et al. 2018

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Background/Aims: Adrenocortical carcinoma (ACC) is an aggressive childhood cancer. Limited evidence exists on a definite histopathological criterion to differentiate ACC from adrenocortical adenoma. The aim of this study was to investigate the clinicopathological data of children with ACC, identify prognostic factors, and validate a histopathological criterion to differentiate ACC from adrenocortical adenoma. Methods: This retrospective cohort included 41 children, followed at the Mayo Clinic from 1950 to 2017 (onset of symptoms ≤21 years). Outcomes of interest were: alive with no evidence of disease, alive with evidence of disease, and dead of disease. Results: Median age at onset of symptoms was 15.7 years (n = 41; range, 0.2–21 years). Female:male ratio was 3.6: 1. Mixed symptomatology (> 1 hormone abnormality) was the most common presentation (54%, n = 22). Sixty-six percent of patients (n = 27 out of 41) underwent total adrenalectomy. Metastatic disease was more common in children aged > 12 years (p = 0.002 compared to < 4 years). The most common sites of metastases were the liver and lungs. Overall 2-year and 5-year survival rates were 61% (95% CI 45–77) and 46% (95% CI 30–62), respectively. Metastasis at the time of diagnosis was independently associated with poor prognosis (risk ratio 13.7%; 95% CI 3.9–87.7). Weiss criteria (29%) and modified Weiss criteria (33%) were less accurate in younger patients (< 12 years), compared to the Wieneke index (100%). Conclusion: The presence of metastases was an independent prognostic factor. The Wieneke index was the most accurate in predicting clinical outcomes in younger children.

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Section: Discussionmentioning

confidence: 85%

Adrenocortical Carcinoma in Children: A Clinicopathological Analysis of 41 Patients at the Mayo Clinic from 1950 to 2017

et al. 2018

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“…in 26 children, and Giovannoni et al. in nine children (Table , including 22 children from the present cohort, total n = 175) . Of these, 29 (16.5%) children were in the intermediate group (not included in the table); nine (30%) had a malignant evolution and 20 (70%) benign.…”

Section: Resultsmentioning

confidence: 59%

“…A cumulative analysis showed Wieneke criteria to have a sensitivity of 100%, specificity of 87%, positive predictive value for malignancy of 80% and negative predictive value for malignancy of 100%. Four studies (including ours) applied and reported separate components of the Wieneke criteria . Less than 5% of children with a benign progression had IVC involvement, periadrenal extension and more than 15 mitotic figures per 20 high power fields, making them the most specific components of the criteria.…”

Section: Resultsmentioning

confidence: 93%

Pathological prognostication of paediatric adrenocortical tumours: Is a gold standard emerging?

Jehangir

Nanjundaiah

Sigamani

et al. 2018

Pediatric Blood & Cancer

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Background Criteria for the pathological classification of adult adrenocortical tumours (ACTs) have been found to overestimate the malignant potential of childhood ACTs. We sought to evaluate the accuracy and utility of criteria developed for paediatric ACT compared to current criteria for adults. Methods ACTs treated between January 2006 and December 2016 in two paediatric institutions were evaluated. Patients classified clinically as malignant (CM) had locally invasive disease at surgery requiring extensive en bloc resection to achieve clear margins, had local recurrence or distant metastasis. Slides were reviewed by pathologists blinded to the clinical outcome. A grade was assigned to each tumor according to the Weiss, Aubert, Wieneke and Dehner‐Hill criteria. The pathological grade was compared to the clinical outcome. Results The median follow‐up was 60 months (interquartile range 25‐80 months). Based on clinical criteria, of 22 patients 14 (64%) had a benign course and eight (34%) behaved malignant. The malignant potential was overestimated by Weiss criteria in 23% and Aubert criteria in 27%. Wieneke and Dehner‐Hill criteria showed good clinicopathological correlation; no child who had a benign course was classified as malignant. The Dehner‐Hill criteria, however, classified five (23%) children as intermediate risk of which three had a clinically benign and two a CM course. Conclusion The Wieneke criteria accurately predicts the clinical course in childhood ACTs and could be considered the gold standard in their pathological characterization.

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“…In the comprehensive study from the International Pediatric Adrenocortical Tumor Registry based on the analysis of 254 patients, the major parameters influencing the risk of recurrence were age, stage at diagnosis and tumor weight (Michalkiewicz et al 2004). After analysis of a series of 83 patients, Wieneke and coworkers proposed a set of 9 macroscopic and microscopic criteria for the diagnosis of malignancy in pediatric ACT (Wieneke et al 2003), further validated in other series (Magro et al 2012, Das et al 2016. Transcriptome analysis of pediatric ACT revealed that HLA class II gene expression was downregulated in tumors classified histologically as carcinomas (West et al 2007).…”

Section: Markers Of Malignancy Are Different In Pediatric Act Comparementioning

confidence: 99%

The next step: mechanisms driving adrenocortical carcinoma metastasis

Lalli

Luconi²

2018

Endocrine-Related Cancer

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Endocrine tumors have the peculiarity to become clinically evident not only due to symptoms related to space occupation by the growing lesion, similarly to most other tumors, but also, and most often, because of their specific hormonal secretion, which significantly contributes to their pathological burden. Malignant endocrine tumors, in addition, have the ability to produce distant metastases. Here, we critically review the current knowledge about mechanisms and biomarkers characterizing the metastatic process in adrenocortical carcinoma (ACC), a rare endocrine malignancy with a high risk of relapse and metastatization even when the primary tumor is diagnosed and surgically removed at an early stage. We highlight perspectives of future research in the domain and possible new therapeutic avenues based on targeting factors having an important role in the metastatic process of ACC.

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Weineke criteria, Ki-67 index and p53 status to study pediatric adrenocortical tumors: Is there a correlation?

Cited by 48 publications

References 32 publications

Adrenocortical Carcinoma in Children: A Clinicopathological Analysis of 41 Patients at the Mayo Clinic from 1950 to 2017

Adrenocortical Carcinoma in Children: A Clinicopathological Analysis of 41 Patients at the Mayo Clinic from 1950 to 2017

Pathological prognostication of paediatric adrenocortical tumours: Is a gold standard emerging?

The next step: mechanisms driving adrenocortical carcinoma metastasis

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