ABSTRACT: Introduction: Adrenocortical tumors (ACT) are rare tumors of childhood. The majority of these tumors is hormone-producing and cause virilization and Cushing syndrome or feminization. Methodology: The authors describe 6 cases of adrenal cortical tumors treated at the Kuwait Cancer Center which were presented over a period of 20 years (1989-2009). Results: The mean age was 5.5 years (range 15 months - 12 years). All had signs of virilization. One child had hypertension, while 2 had a metastatic disease at presentation. The diagnosis was made by clinical signs and symptoms, high levels of relevant adrenal hormones and imaging. Two children were not fitfor surgery; one was too sick for any treatment and died shortly after diagnosis, while the other died after receiving one cycle of palliative chemotherapy. Four patients underwent complete surgical resection and achieved complete remission, three of whom later had recurrence (distant in one and local in two patients) and succumbed due to progressive disease. Mitotane was used in two children. Only one patient is currently surviving and well nearly 13 years after her surgery. In our series, the long-term outcome of children with adrenocortical tumors was very poor. Conclusions: Virilization is an important clue to the diagnosis of ACT. Early diagnosis and complete surgical resection are important for survival. Metastasis at presentation or as recurrence carries very dismal prognosis. Keywords: Adrenocortical tumor, Adrenal neoplasm, Virilizing adrenal tumor, Pediatric hormone secreting tumors.
gulf journal of oncology, The 07/2012; 1(12):38-46.