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.1 The authors demonstrated correctly how the Wieneke score was superior compared to adult scores to predict malignancy, which is now recognized in the literature.2 They showed the high sensitivity of Wieneke score as no patient categorized as benign (n = 14) had a malignant outcome, which was also assessed with Dehner and Hill criteria.3 Contemporary to Jehangir et al, we reported a comprehensive series of 95 cases of pediatric ACTs in the French population.4 Respective incidences of histologically “benign” (63.1%, n = 60 vs. 63.6%, n= 14), “intermediate” (12%, n= 11 vs. 4.5%, n= 1), and “malignant” (25%,n=24vs. 32%,n=7) neoplasmswere similar to thepresent series, illustrating comparable sampling of patients. Results regarding the prognostic value of Wieneke's criteria were similar with two criteria being not statistically significant between benign and malignant tumors, namely vena cava invasion and presence of atypical mitosis. The authors argue also the high specificity of the Wieneke score, which allows a better distinction between histologically benign and malignant tumors compared to other systems, and therefore limits deleterious consequences of a “cancer”misdiagnosis for child and family. However, among seven patients with an ACT classified as malignant according to Wieneke score, three were alive with disease and two in complete remission at the end of follow-up. What first determinedmalignancy in pediatric ACT and therefore guide treatment recommendations is tumor stage (as described by the International Pediatric Adrenocortical Tumor Registry).5 Besides biological data, it is defined by tumor volume, initial disease extension, and integrity of tumor resection, which are known to be central factors in the risk assessment of these tumors. 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.1,6–9 Pathologists have to primarily focus on how the pathological grading is integrated by clinicians to decide whether or not an adjuvant therapy is needed. Although it has a minor value in treatment algorithms for stages I and IV disease, it is of great significance for stages II-III tumors, which are associated with the most unpredictable outcome. Given the significance of choosing an adequate monitoring of these patients, clinicians need precise and relevant criteria from the pathological prognostication. In our study, we showed how the redundancy of someWieneke criteriawith tumor staging features artificially upgrades malignancy.4 We believe that, after tumor staging assessment, the use of a simple pathological score that includes the most predictive anddiscriminantWienekemicroscopic criteria (adrenal capsular invasion, venous invasion, confluent necrosis, and mitotic count), in addition to the proliferation index Ki67, would guide more precisely the need of an adjuvant therapy in pediatric localized ACTs.4 With a cut-off of two items, we showed a significant correlation between “favorable” or “unfavorable” histology tumors with outcome. Further independent studies are necessary to validate these findings.
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