Nidhi Gupta1, Michael Rivera2, Paul Novotny3, Vilmarie Rodriguez4, Irina Bancos5, Aida Lteif1. 1. Division of Pediatric Endocrinology and Metabolism, Mayo Clinic, Rochester, Minnesota, USA. 2. Department of Anatomic Pathology, Mayo Clinic, Rochester, Minnesota, USA. 3. Department of Health Sciences Research, Mayo Clinic, Rochester, Minnesota, USA. 4. Division of Pediatric Hematology-Oncology, Mayo Clinic, Rochester, Minnesota, USA. 5. Division of Endocrinology, Diabetes, Metabolism and Nutrition, Mayo Clinic, Rochester, Minnesota, USA.
Abstract
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.
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.
Authors: Jan Idkowiak; Yasir S Elhassan; Pascoe Mannion; Karen Smith; Rachel Webster; Vrinda Saraff; Timothy G Barrett; Nicholas J Shaw; Nils Krone; Renuka P Dias; Melanie Kershaw; Jeremy M Kirk; Wolfgang Högler; Ruth E Krone; Michael W O'Reilly; Wiebke Arlt Journal: Eur J Endocrinol Date: 2019-03-01 Impact factor: 6.664
Authors: Ozgur Mete; Lori A Erickson; C Christofer Juhlin; Ronald R de Krijger; Hironobu Sasano; Marco Volante; Mauro G Papotti Journal: Endocr Pathol Date: 2022-03-14 Impact factor: 4.056