Revisiting the role of the pathological grading in pediatric adrenal cortical tumors: results from a national cohort study with pathological review

Picard, Cécile; Orbach, Daniel; Carton, Matthieu; Brugières, Laurence; Renaudin, Karine; Aubert, Sébastien; Berrebi, Dominique; Galmiche, Louise; Dujardin, Fanny; Leblond, Pierre; Thomas‐Teinturier, Cécile; Dijoud, Frédérique

doi:10.1038/s41379-018-0174-8

Cited by 46 publications

(79 citation statements)

References 31 publications

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“…The stages were compared as follows. Stage I versus Stage II, III, and IV included eight studies 7,18,20,31,36,38,40,46 . The meta‐analysis showed a better outcome for Stage I ( P < .00001; RR 6.94; 95% CI 3.35‐14.4; I 2 12%).…”

Section: Resultsmentioning

confidence: 99%

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Clinical prognostic factors in pediatric adrenocortical tumors: A meta‐analysis

Zambaiti

Duci

Corti

et al. 2020

Pediatric Blood & Cancer

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Pediatric adrenocortical tumors (ACT) are rare and sometimes aggressive malignancies, but there is no consensus on the outcome predictors in children. A systematic search of MEDLINE, SCOPUS, Web of Science, and the Cochrane Library for studies from 1994 to 2020 about pediatric ACT was performed. In 42 studies, 1006 patients, aged 0-18 years, were included. The meta-analyses resulted in the following predictors of better outcome: age <4 years (P < .00001), nonsecreting tumors (P = .004), complete surgical resection (P < .00001), tumor volume (P < .0001), tumor weight (P < .00001), tumor maximum diameter (P = .0009), and Stage I disease (P < .00001). Moreover, patients affected by Cushing syndrome showed a worse outcome (P < .0001). International prospective studies should be implemented to standardize clinical prognostic factors evaluation, together with pathological scores, in the stratification of pediatric ACT.

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Section: Resultsmentioning

confidence: 99%

“…Age greater than or less than 4 years was considered according to previous reports, which showed that children under 4 years of age usually show a favorable outcome. Indeed, distant metastases are observed at a higher rate in adolescents 7,8 …”

Section: Methodsmentioning

confidence: 98%

Clinical prognostic factors in pediatric adrenocortical tumors: A meta‐analysis

Zambaiti

Duci

Corti

et al. 2020

Pediatric Blood & Cancer

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“…The Wieneke criteria, which include tumor size, local invasion, and histological features, have been reported useful in pediatric ACT malignancy definition and prognosis prediction ( 11 , 18 , 30 ). Recently, Picard and colleagues ( 31 ) proposed a pathological scoring system incorporating the Ki67 index ≥15% in a prognostication algorithm to guide adjuvant treatment in pediatric ACTs, mostly for those with incomplete resection. In our cohort, the Wieneke score could not predict clinical outcomes in patients who experienced metastatic disease.…”

Section: Discussionmentioning

confidence: 99%

Clinical, Genetic, and Prognostic Features of Adrenocortical Tumors in Children: A 10-Year Single-Center Experience

et al. 2020

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Background and Aims: Pediatric adrenocortical tumors (ACTs) are very rare endocrine neoplasms in childhood. In this study, we performed a retrospective analysis of children with ACT treated at our institution by examining clinical and genetic disease features, treatment strategies, and outcomes. Methods: We retrospectively analyzed a cohort of 13 children treated at the Bambino Gesù Children's Hospital from November 2010 to March 2020. Results: The median age at diagnosis was 17 months (range = 0-82 months). The female: male ratio was 3.3/1. Mixed symptomatology (>1 hormone abnormality) was the most common presentation (46.1%). In three cases, the tumor was detected during prenatal or perinatal echographic screening. All patients presented with localized disease at diagnosis and underwent total adrenalectomy. Six patients were identified as having malignancies according to the Wieneke scoring system, five benign, and two undetermined. Seven patients underwent mitotane adjuvant therapy for 12 months. There was metastatic disease in three patients, with no correlation with age or Wieneke score. The most common sites of metastases were the liver and lungs. Metastatic patients were treated with surgery (n = 2), mitotane (n = 1), chemotherapy (n = 2) associated with anti-EGFR (n = 1), or immunotherapy with anti-PD1 (pembrolizumab) (n = 1); two patients achieved complete disease remission. Overall 2-and 5-year survival rates were 100%, with a median follow-up of 5 years (range = 2-9.5 years). Two-and 5-year disease free survival was 76.9 and 84.6%, respectively (95% confidence interval = −66.78-114.76 months). All patients are alive, 12 without disease, and one with stable disease. Genetic analyses showed TP53 germline mutations in six of eight patients analyzed (five inherited, one de novo). One patient had Beckwith-Wiedemann syndrome, with mosaic paternal uniparental disomy of chromosome 11, in both neoplastic and healthy adrenal tissue. Miele et al. Adrenocortical Tumors in Children Conclusion: We report the cases of 13 patients treated for ACT, including 12 aged <4 years at diagnosis, with a relative short time from symptoms onset. Our cohort experienced an excellent prognosis. TP53 mutation was found in 75% of tested patients (6/8) confirming the need to perform genetic tests and familial counseling in this disease.

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“…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 some Wieneke criteria with tumor staging features artificially upgrades malignancy . We believe that, after tumor staging assessment, the use of a simple pathological score that includes the most predictive and discriminant Wieneke microscopic 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 .…”

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confidence: 66%

“…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 . Contemporary to Jehangir et al, we reported a comprehensive series of 95 cases of pediatric ACTs in the French population . 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 = 24 vs. 32%, n = 7) neoplasms were similar to the present series, illustrating comparable sampling of patients.…”

mentioning

confidence: 87%