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Treatment of Childhood Adrenocortical Carcinoma
Source: Childhood Adrenocortical Carcinoma Treatment (PDQ®)–Health Professional Version, National Cancer Institute.
Source updated: April 3, 2025 · Captured 2026-09-09.
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At the time of diagnosis, two-thirds of pediatric patients have limited disease (tumors that can be completely resected), and the remaining patients have either unresectable or metastatic disease.[1]
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The European Cooperative Study Group for Pediatric Rare Tumors within the PARTNER project (Paediatric Rare Tumours Network - European Registry) has published consensus guidelines for the diagnosis and treatment of childhood adrenocortical tumors.[2] Treatment of childhood adrenocortical tumors has evolved using the data from adult studies, and the same guidelines are used. Surgery is the most important mode of therapy, and mitotane and cisplatin-based regimens, usually incorporating doxorubicin and etoposide, are recommended for patients with advanced disease.[3-6]; [7][Level of evidence C1]
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Treatment options for childhood adrenocortical tumors include the following:
Surgery with or without adjuvant chemotherapy.
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Preserved source evidence · Not medical advice