Diagnosis and treatment of adrenal cortical carcinoma in children

Abstract

Objective To analyze the clinical features and prognosis of adrenalcortical carcinoma in children and improve its diagnosis and treatment. Methods Medical records were retrospectively analyzed for 16 patients hospitalized from January 2002 to March 2012. And their clinical symptoms, endocrine function, imaging findings, pathological diagnosis and treatment were discussed. And follow-ups were conducted. Results All cases were pathologically diagnosed as adrenal cortical carcinoma. There were functional (n=10) and non-functional (n=6) tumors. Complete macroscopic resection of tumor was performed for 12 cases while another, 4 cases had gross residue. The postoperative period was uneventful. Outpatient or telephone follow-ups were conducted for 6-12 years.. All 4 cases of total resection survived with a normal development. One case with severe kidney adhesions underwent nephrectomy with a postoperative survival of 6 months. Three cases with tumor thrombus survived 1, 5 and 12 months respectively. One case on a 4-week chemotherapy died from pulmonary metastasis while another 2 cases died 6 months later from bone marrow suppression and infection due to chemotherapy. One case received a 2-year chemotherapy and died from sudden brain metastases. One case died in a car accident While another 3 cases were lost. The average survival time was 35 months. Conclusions Adrenal cortical carcinoma is a rare tumor with a high degree of malignancy and a poor prognosis in children. Surgical excision remains main treatment for this disease. The perioperative application of corticosteroids is absolutely necessary. Laparotomy is preferred over laparoscopic operation. In fact, preoperative and postoperative chemotherapies fail to improve the prognosis. Key words: Adrenalcortical carcinoma; Diagnosis; Treatment