Author/Authors :
Yesil, Sule Dr. Sami Ulus Children s Hospital - Department of Pediatric Oncology, Turkey , Çetinkaya, Semra Dr. Sami Ulus Children s Hospital - Department of Pediatric Endocrinology, Turkey , Oren, Ayse Ceyda Dr. Sami Ulus Children s Hospital - Department of Pediatric Oncology, Turkey , Erdeve, Senay Savas Dr. Sami Ulus Children s Hospital - Department of Pediatric Endocrinology, Turkey , Tekgunduz, Sibel Akpinar Dr. Sami Ulus Children s Hospital - Department of Pediatric Oncology, Turkey , Candir, Mehmet Onur Dr. Sami Ulus Children s Hospital - Department of Pediatric Oncology, Turkey , Aycan, Zehra Dr. Sami Ulus Children s Hospital - Department of Pediatric Endocrinology, Turkey
Abstract :
Objective: Childhood adrenocortical tumors (ACTs) are rare neoplasms, about which etiopathogenesis and disease management are not yet clearly understood. We aimed to review the management of ACTs in our single medical center. Methods: We retrospectively reviewed findings in seven children, who were 15 years old or younger, and were diagnosed with ACTs in our institution over the past 10 years. Information recorded for each patient included age, sex, presenting symptoms, hormonal status, pathological findings, stage of disease, treatment and outcome. Results: Four girls and three boys were treated for ACTs. Five had adrenocortical carcinoma (ACC) and two had adrenocortical adenoma (ACA). All patients underwent laparotomy and complete excision. Two of ACCs had a stage I disease, one had a stage III, and the latter two ACCs had a stage IV disease. ACAs were treated successfully by total excision without any concomitant therapy. Adjuvant chemotherapy and mitotane were commenced postoperatively in ACC patients. Six patients are alive and doing well at a mean follow-up of 5 years and 8 months. Conclusion: The small number of ACTs and the short follow-up period limit the assessments of prognosis and management. As a result, patients with ACTs should be studied in multi-institutional trials to address the role of prognostic factors and cytotoxic drugs in the disease management.
