The impact of tumor stage on prognosis in children with adrenocortical carcinoma

Purpose: We evaluated treatment outcomes in children with adrenocortical carcinoma. Materials and Methods: We studied 34 children with a median age of 3 years. In 27 of 28 patients without intracaval extension complete surgical excision was accomplished, while tumor resection combined with thrombectomy was carried out in 5 of 6 children with vascular invasion. In 2 children with cavoatrial thrombus the thrombectomy required cardiopulmonary bypass with deep hypothermia and circulatory arrest. Children with incomplete excision of the tumor and/or stage IV disease received adjuvant chemotherapy. Results: Ultrasonography, computerized tomography and magnetic resonance imaging exhibited specificity of 100% in the diagnosis of vascular invasion, and sensitivity of 50%, 66% and 100%, respectively. Patient age, tumor stage or size and vascular invasion were associated with survival in univariate analysis. Tumor stage was the only independent factor associated with survival in multivariate analysis. The overall 5-year survival rates according to tumor stage were 100% in stage I, 85% in stage II, 40% in stage III and 0% in stage IV. Of 11 children with local recurrence only 2 were alive without disease at 96 and 204 months after reoperation with complete tumor excision. Only 2 of 6 patients with vascular invasion were disease-free at 17 and 50 months. A total of 10 children with stage IV disease treated with chemotherapy died within a median of 6 months. Conclusions: Tumor stage was the most relevant prognostic factor for children with adrenocortical carcinoma. Reoperation for local tumor recurrence and thrombectomy for inferior vena caval tumor invasion should be attempted whenever possible.