Outcome and prognostic factors in high‐risk childhood adrenocortical carcinomas: A report from the European Cooperative Study Group on Pediatric Rare Tumors (EXPeRT)

Abstract

Distant metastases and large tumor volume were the main unfavorable prognostic factors. Presence of two or more factors related to ACC was associated with an aggressive behavior of disease.

Adrenocortical Carcinoma in Children: A Clinicopathological Analysis of 41 Patients at the Mayo Clinic from 1950 to 2017

“…Overall 2-year and 5-year survival rates (61 and 46%, respectively) of our patients were comparable to those reported previously [4, 14, 15, 17]. In IPACTR [15], at a median follow-up of 2 years and 5 months, 61.8% patients remained alive.…”

“…We acknowledge that larger studies on pediatric ACT (adenomas plus carcinomas) have been published previously. These studies have reported cohorts of patients from multicenter registries over a period of 10–35 years [1, 3, 4, 14-17, 26, 42]. Our study is the largest single institution experience with longest duration of data on pediatric ACC, reported so far.…”

“…Even after complete resection, a high risk of recurrence of ACC remains. Age < 4 years, tumor size < 10 cm [14], virilization alone [15], tumor volume < 200 cm 3 [16, 17], tumor weight and stage [18], metastatic disease [14], and quality of surgery [14, 19] have been suggested as important prognostic markers. Despite multimodality approaches including mitotane [an adrenolytic drug: (o,p’-DDD: 1,1-dichloro-2-o-chlorophenyl)-2-(p-chlorophenyl) ethane], chemotherapy with cisplatin, etoposide and doxorubicin (CED), and radiotherapy, prognosis of pediatric ACC remains poor with an estimated 5-year survival rate ranging from 30 to 90% [2, 4, 15, 18, 20].…”

Pediatric patients with cutaneous melanoma: A European study

“…The EXPeRT project (founded in 2008 to pool the experience and strengths of various national cooperative groups) represents a model for promoting international research on such very rare neoplasms . This study on melanoma follows other retrospective reports on other specific histotypes, with the collection of cases deriving from prospective series seen in recent years by national cooperative groups taking part in the project.…”

Erythrocytosis as the first manifestation of adrenal carcinoma

“…ACC is extremely rare in children and teenagers. 6,7 In many cases, it is characterized by aggressive behavior and bad prognosis, and only 16% to 47% of patients are still alive after five years. 8 Cortisol or testosterone hypersecretion occurs in 50% and 15% of the cases, respectively.…”