Adrenocortical Tumors in Children and Adolescents: The European PARTN-ER Project for Consensus Guidelines Development.

Background and Aims: Adrenocortical carcinomas (ACC) are rare diseases. Several collaborative studies performed over the last decades showed improved results compared to historical data, but standardized guidelines for diagnosis andmanagement of pediatric ACC are still unavailable. Methods: We present European consensus guidelines developed by the Cooperative Study Group for Pediatric Rare Tumors (EXPeRT) according to the ESMO scale, based on the evidence collected from published series, case reports and personal expertise. Results: ACT should be removed without rupture [Level V; Grade B]. Preferred option is open approach through a lateral transverse abdominal incision or a midline laparotomy in case of huge masses [Level IV; Grade B]. Mini-invasive surgery should be discouraged when volume exceeds 200cm3, suspicious regional nodal involvement and/or signs of local invasion are present [Level IV; Grade B]. All enlarged lymph nodes detected at radiology or intra-operatively should be removed [Level III; Grade A]. A systematic biopsy of the regional nodes should be performed, though they are not found suspicious pre- or intraoperatively [Level IV; Grade C]. Adjuvant therapy should be considered in advanced-stage ACT or in case of incomplete tumor resection [Level IV; Grade B]. First-line recommended regimen is CED (cisplatinum-etoposide-doxorubicin) plus mitotane [Level IV; Grade B]. For COG stage II tumors, mitotane is considered if several risk factors are associated: unfavorable histology (5-item score or Wieneke score >3), older age, hormonal secretion [Level IV; Grade C]. For unresectable tumors, gross/macroscopic residual disease, retroperitoneal lymph nodes involvement, CED with mitotane are considered [Level III; Grade B]. No data exist about the optimal duration of therapy. CED is usually scheduled in 6 to 8 cycles. Mitotane is continued for 1 to 2 years depending on patient’s tolerance and compliance [Level IV; Grade C]. Conclusions: This European PARTN-ER project leads to a consensus strategy regarding the treatment of children and adolescents with ACC.