Primary adrenocortical carcinoma: diagnosis and treatment / 中国医师进修杂志

ABSTRACT

Objective To improve the diagnosis and treatment of primary adrenocortical carcinoma. Methods Retrospective analysis was performed in clinical data of 16 patients with primary adrenocortical carcinoma. The diagnosis was based on clinical presentations,hormonal studies and imaging. Surgical treatment was underwent on 13 cases. All patients were followed up for 3 to 62 months. Results Of 16 cases,functional tumors were in 8,including Cushing′s syndrome in 5,combination of Cushing′s syndrome and virilization in 2,and hyperaldosteronism in 1. Imaging studies revealed the tumors were 4.8 to 19.5 cm in diameter,average in 7.8 cm. Distant metastasis occurred in 3 cases. Radical surgery was done in 13 cases without distant metastasis,including radical adrenalectomy in 8,adrenalectomy plus nephrectomy in 2,adrenalectomy plus surgical extirpation of intracaval tumor thrombus in 2,and partial resection of the wall of inferior vena cava in 1. Pathologic stages were stage Ⅰ in 2 cases,stage Ⅱ in 8,stage Ⅲ in 3,stage Ⅳ in 3. Among the 11 cases who were treated over 2 years after operation,6 cases were still survival,while 1 case had pulmonary and 1 case had bone metastasis. The other 5 cases survive in average of 26 months. Conclusions Prognosis of primary adrenocortical carcinoma is poor. The keys to early diagnosis are clinical manifestations plus imaging. Surgical treatment is the only effective therapy for the disease.