Abstract:Adrenocortical tumors are rare in children. This systematic review summarizes the published evidence on pediatric adrenocortical carcinoma (ACC) to provide a basis for a better understanding of the disease, investigate new molecular biomarkers and therapeutic targets, and define which patients may benefit from a more aggressive therapeutic approach. We included 137 studies with 3680 ACC patients (~65% female) in our analysis. We found no randomized controlled trials, so this review mainly reflects retrospectiv… Show more
“…In the pediatric setting, evidence for adjuvant RT is even more limited. In a recent systematic review on pACC, we demonstrated that the rate of local recurrences was higher than expected: 81% of reported relapses were due to local recurrences or lymph node metastases [10]. In contrast, local recurrences in the adult setting have been reported only in around 50-60% of patients [11].…”
Section: Introductionmentioning
confidence: 76%
“…Available information on RT from the literature is listed in Table 1. None of these were randomized controlled trials (RCTs) but retrospective analyses, with 76 patients managed with RT [10]. Additionally, we included data of 4 previous unreported patients upon receiving further information from the authors.…”
Section: Resultsmentioning
confidence: 99%
“…Therefore, in order to gain clear evidence on the efficacy of RT patients have to be enrolled in randomized controlled trials (RCTs) or at least in standardized prospective registry trials enabling research. Still, recently published pediatric data [2,10] showed improvements in local control and long-term outcomes in pACC patients managed with RT.…”
Section: Discussionmentioning
confidence: 99%
“…Excerpted data was double-reviewed for inclusion (MR, VW). The search strategy was performed according to Riedmeier et al [10].…”
Section: Introductionmentioning
confidence: 99%
“…On the remaining 50 patients, no additional information was available. Tumor stages were reported according to the international TNM classification system or the ENSAT criteria [10,23].…”
“…In the pediatric setting, evidence for adjuvant RT is even more limited. In a recent systematic review on pACC, we demonstrated that the rate of local recurrences was higher than expected: 81% of reported relapses were due to local recurrences or lymph node metastases [10]. In contrast, local recurrences in the adult setting have been reported only in around 50-60% of patients [11].…”
Section: Introductionmentioning
confidence: 76%
“…Available information on RT from the literature is listed in Table 1. None of these were randomized controlled trials (RCTs) but retrospective analyses, with 76 patients managed with RT [10]. Additionally, we included data of 4 previous unreported patients upon receiving further information from the authors.…”
Section: Resultsmentioning
confidence: 99%
“…Therefore, in order to gain clear evidence on the efficacy of RT patients have to be enrolled in randomized controlled trials (RCTs) or at least in standardized prospective registry trials enabling research. Still, recently published pediatric data [2,10] showed improvements in local control and long-term outcomes in pACC patients managed with RT.…”
Section: Discussionmentioning
confidence: 99%
“…Excerpted data was double-reviewed for inclusion (MR, VW). The search strategy was performed according to Riedmeier et al [10].…”
Section: Introductionmentioning
confidence: 99%
“…On the remaining 50 patients, no additional information was available. Tumor stages were reported according to the international TNM classification system or the ENSAT criteria [10,23].…”
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