TriangleHEALTH PROFESSIONAL · SOURCE READING
Chemotherapy
Source: Childhood Adrenocortical Carcinoma Treatment (PDQ®)–Health Professional Version, National Cancer Institute.
Source updated: April 3, 2025 · Captured 2026-09-09.
Selected source text with whitespace normalised. This Triangle page is not an NCI PDQ summary.
Context: Treatment of Childhood Adrenocortical Carcinoma
Little information is available about the use of mitotane in children, although response rates appear to be similar to those seen in adults.[4,14] In adults, mitotane is commonly used as a single agent in the adjuvant setting after complete resection.[4]
Evidence (surgery and chemotherapy):
One review included 11 children with advanced adrenocortical tumors who were treated with a mitotane and cisplatin-based chemotherapeutic regimen.[4]
Source links and citations
One review included 11 children with advanced adrenocortical tumors who were treated with a mitotane and cisplatin-based chemotherapeutic regimen.[4]
Measurable responses were seen in seven patients.
One review included 11 children with advanced adrenocortical tumors who were treated with a mitotane and cisplatin-based chemotherapeutic regimen.[4]
The mitotane daily dose required for therapeutic levels was approximately 4 g/m2, and therapeutic levels were achieved after 4 to 6 months of therapy.
In the GPOH-MET 97 trial, mitotane levels greater than 14 mg/L correlated with better survival in children.[6,7]
A retrospective analysis identified 177 Italian and German adult patients with completely resected adrenocortical carcinoma.[15] For more information, see Adrenocortical Carcinoma Treatment.
Source links and citations
A retrospective analysis identified 177 Italian and German adult patients with completely resected adrenocortical carcinoma.[15] For more information, see Adrenocortical Carcinoma Treatment.
Recurrence-free survival was significantly prolonged by the use of adjuvant mitotane.
A retrospective analysis identified 177 Italian and German adult patients with completely resected adrenocortical carcinoma.[15] For more information, see Adrenocortical Carcinoma Treatment.
The benefit was present with 1 to 3 g per day of mitotane, and this dose was associated with fewer toxic side effects than doses of 3 to 5 g per day.
Adrenocortical tumors are generally considered to be radioresistant. The use of radiation therapy in pediatric patients with adrenocortical tumors has not been consistently investigated. Furthermore, because many children with adrenocortical tumors carry germline TP53 pathogenic variants that predispose them to cancer, radiation may increase the incidence of secondary tumors. One study reported that three of five long-term survivors of pediatric adrenocortical tumors died of secondary sarcomas that arose within the radiation field.[6,16]
For more information, see Adrenocortical Carcinoma Treatment.
Source links and citations
Publication references
Read the original reference and check its publication notices.
Preserved source evidence · Not medical advice