The diagnosis and treatment of adrenocortical carcinoma:report of 39 cases

ZHAN Fa-ming; LIN Hao; PAN Jin-cheng; ZHUANG Jin-tao; CHEN Xu; HUANG Bin; WANG Dao-hu; QIU Shao-peng

Abstract：Objective To investigate the clinicopathological characteristics and treatment of patients with adreno-cortical carcinoma ( ACC) .Methods Thirty-nine patients diagnosed with ACC between January 1999 and march 2014 were evaluated, with 39 time-matched adrenocortical adenoma ( ACA) were randomly drawn as control group.The clini-cal features and outcomes were reviewed.Results In ACC group, median age was 51.72 years (19 to 76 years) , among whom Weiss score was between 3 and 5 in 13 cases and over 5 in 26 case, with the tumor diamer of(11.66 ±4.21)cm. In ACA group, Weiss score was lower than 3 in 27 cases and between 3 and 5 in 2 cases, with the tumor diamer of(2.34 ± 1.27)cm.Endocrinolgical evaluation was performed in all patients.En bloc resetion was performed in 34 cases in ACC group, and palliative resection or biopsy was performed in 5 patients whom detected metastases at initial diagnosis.Two deaths were reported during the 1st week after surgery.Sorafenib was given to 2 cases in ACC group.With a follow-up of 1~80 months, the median survival time was 12 months, with the 1 -year and 5 -year survival rates of 45.7% and 32.7%, respectively.All patients in ACA group underwent operation successfully with satisfactorily postoperative rehabili-tation.Thirty-three cases were followed up 2 to 176 months with no death.Conclusion ACC is a rare disease with a poor prognosis.Tumor sizes could be used as one of a valuable indicator for distinguishing.Patients with positive VEGF in tumor tissue could try Sunitinib for molecular targeted drug therapy.