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

Abstract

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.

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%).…”

“…A meta-analysis (Supporting Information S3) including 13 studies 5,20,23,36,27,32,33,37,38,42,51,53,54 compared the outcome between patients undergone complete resection and those undergone incomplete resection. The first ones showed a better outcome (P < .00001; RR 3.50; 95% CI 2.51-4.88; I 2 16%).…”

“…Two meta-analyses were performed for the two different cutoffs. Either tumors weighing more than 400 g [17][18][19][20][21]27,31,33,35,[37][38][39][40]42,52 or tumors weighing more than 100 g 17,18,20,21,31,[33][34][35][37][38][39][40]42,45,52 were considered as risk factor for a worse outcome (respectively, P < .00001; RR 4.29; 95% CI 3.19-5.78;…”

Reply to “Pathological prognostication of pediatric adrenocortical tumors: Is a gold standard emerging?”

“…To the Editor: In a recently published article in Pediatric Blood & Cancer , Jehangir et al studied 22 cases of pediatric adrenocortical tumors (ACTs) and found that Wieneke criteria accurately predicted clinical outcome . The authors demonstrated correctly how the Wieneke score was superior compared to adult scores to predict malignancy, which is now recognized in the literature .…”

“…Assessing the prognostic value of Wieneke score in small series of patients has been done few times but appears to us not sufficient enough to guide perioperative treatment recommandations . Pathologists have to primarily focus on how the pathological grading is integrated by clinicians to decide whether or not an adjuvant therapy is needed.…”