Akiko Sakoda1, Imran Mushtaq1, Gill Levitt2, Neil J Sebire3. 1. Department of Paediatric Urology, Great Ormond Street Hospital for Children and Institute of Child Health, London WC1N 3JH, UK. 2. Department of Oncology, Great Ormond Street Hospital for Children and Institute of Child Health, London WC1N 3JH, UK. 3. Department of Histopathology, Great Ormond Street Hospital for Children and Institute of Child Health, London WC1N 3JH, UK. Electronic address: Neil.Sebire@gosh.nhs.uk.
Abstract
BACKGROUND/ PURPOSE: Adrenocortical tumors (ACTs) are rare in children and the extent to which histopathological features can predict clinical behavior remains uncertain. The aim of this study was to investigate the relationship between histopathological features (Weiss score), surgical approach, tumor size, underlying genetic predisposition syndrome, and outcome. METHODS: Twenty-nine ACTs treated at our institution between 1987 and 2011 were identified from a histopathology database. The histological features were categorized using the Weiss scoring system. For tumor staging, the UKCCSG staging system was utilized. RESULTS: At a median follow-up of 25 months, 19 patients (65.5%) survived without evidence of disease and 10 patients (35.5%) had died. There was a strong association between high Weiss score and both large tumor size (P<0.01) and adverse outcome (P<0.01). Outcome for stage I and IIA disease was significantly better compared to higher stage disease and/or tumor rupture (P<0.01). CONCLUSION: There is an association between high Weiss score, large tumor size, underlying genetic predisposition syndrome and an adverse outcome for pediatric ACTs. Regardless of histopathological findings, complete surgical resection, without tumor spillage, is optimal for survival. Genetic evaluation is recommended in patients with ACTs, particularly those with a high Weiss score.
BACKGROUND/ PURPOSE:Adrenocortical tumors (ACTs) are rare in children and the extent to which histopathological features can predict clinical behavior remains uncertain. The aim of this study was to investigate the relationship between histopathological features (Weiss score), surgical approach, tumor size, underlying genetic predisposition syndrome, and outcome. METHODS: Twenty-nine ACTs treated at our institution between 1987 and 2011 were identified from a histopathology database. The histological features were categorized using the Weiss scoring system. For tumor staging, the UKCCSG staging system was utilized. RESULTS: At a median follow-up of 25 months, 19 patients (65.5%) survived without evidence of disease and 10 patients (35.5%) had died. There was a strong association between high Weiss score and both large tumor size (P<0.01) and adverse outcome (P<0.01). Outcome for stage I and IIA disease was significantly better compared to higher stage disease and/or tumor rupture (P<0.01). CONCLUSION: There is an association between high Weiss score, large tumor size, underlying genetic predisposition syndrome and an adverse outcome for pediatric ACTs. Regardless of histopathological findings, complete surgical resection, without tumor spillage, is optimal for survival. Genetic evaluation is recommended in patients with ACTs, particularly those with a high Weiss score.
Authors: Emilia Modolo Pinto; Carlos Rodriguez-Galindo; John Kim Choi; Stanley Pounds; Zhifa Liu; Geoffrey Neale; David Finkelstein; John M Hicks; Alberto S Pappo; Bonald C Figueiredo; Raul C Ribeiro; Gerard P Zambetti Journal: Clin Cancer Res Date: 2016-06-15 Impact factor: 12.531