Nutritional support in children treated for advanced adrenocortical carcinoma

Adrenocortical carcinoma (ACC) is a rare, aggressive pediatric malignancy. Advanced ACC requires multimodal treatment, including surgery and systemic chemotherapy including cisplatin, etoposide, doxorubicin, and mitotane. This is associated with significant gastrointestinal toxicity, resulting in many patients being unable to complete scheduled therapy. Often, supplemental nutrition is required if oral intake during treatment is poor. We assessed the frequency of nutritional supplement use in pediatric patients treated for advanced ACC.

This was a retrospective observational study of patients with ACC treated at St. Jude Children's Research Hospital over 10 years (2012-2022). Patient demographics, treatment received, and the need for supplemental enteral or parenteral nutrition were reviewed.

A total of 18 patients with ACC were treated from 2012 to 2022, with 11 having advanced ACC. 54.5% of patients required supplemental nutrition, both enteral and parenteral. All patients requiring supplemental nutrition were intolerant of oral intake, with a mean weight loss of 13.8% (range: 5.9-35%). Mean duration of nutritional support was 362 ± 337 days. Patients requiring supplemental nutrition tended to be younger than others (mean age: 4.45 ± 3.63 vs. 9.14 ± 4.59 years; median age: 3.35 vs. 8.40 years; range: 0.90-11.0 vs. 3.30-15.1 years) (p = 0.082).

Most patients with stage IV ACC require nutritional support during their treatment course, especially younger patients. Preemptive feeding tube placement should be considered to avoid delays in treatment.