Abstract
OBJECTIVE: This study aimed to summarize the clinical characteristics, comprehensive treatment, and prognosis of adrenocortical carcinoma (ACC) in children. METHODS: The clinical data of eight children with definite diagnoses of ACC were retrospectively analyzed, and statistical methods were used to analyze the clinical characteristics, comprehensive treatment mode, and prognosis of these patients. RESULTS: (1) Clinical characteristics: two were males and six were females with the median age of onset was six-years old were involved. Four patients had a rash and precocious puberty as the symptoms of onset. European Network for the Study of Adrenal Tumors (ENSAT) staging: stage II, two patients; stage IV, six patients. (2) Comprehensive treatment: all eight patients underwent surgical treatment and received six cycles of chemotherapy: the regimen was "etoposide + pirarubicin + cisplatin + mitotane." (3) Prognosis analysis: among these eight patients, two patients died, two patients achieved complete remission, the disease was stable in four patients, and the overall five-year survival rate was 75%. Prognosis analyzed according to ENSAT staging (stage II versus stage IV) revealed that two-year survival rates of the two groups were 100% versus 65%, respectively, without statistical significant (χ (2) = 1.066, P = 0.302). Prognosis analyzed according to Weiss score (Weiss score was <6, five patients;≥6, three patients) revealed That survival time of the two groups was 50±9.52 months versus 6±1.70 months, the two-year survival rates of the two groups were 100% versus 35%, and the difference in survival rates between these two groups was statistically significant (χ(2) = 4.091, P = 0.043). CONCLUSION: The Weiss score is an important prognostic factor for ACC. The chemotherapy regimen "mitotane + etoposide + adriamycin + cisplatin" is recommended.