Diagnosis And Management Of Primary Adrenocortical Carcinoma (23 Cases Report)

Objective To improve the diagnosis and treatment of adrenocortical carcinoma (ACC) by analyzing the clinical data of ACC.Methods Retrospectively analyzed the clinical data of 23 pathological confirmed cases of primary adrenocortical carcinoma admitted to Shandong Provincial Hospital from August 1994 to August 2008.Retrospectively analyzed and summarized the clinical presentation,imaging character,laboratory examination,pathological character,treatment and prognosis showed by follow-up data of these cases.Results1.There were 11 male patients and 12 female patients,aged from 24 to 78 years old(average 49.3).And 1 case was a child patient of two-and-a-half-year-old and the other 22 cases were all adult patients.The tumors were located on the left in 13,right in 7,and bilaterally in 3 patients.The maximal diameter of the tumors ranged from 2.0cm to 24cm(average 9.45cm).In the 26 tumors,5 tumors were 5cm or smaller. The other 21 were large than 5cm in size.2.The child female patient and 1 adult female patient presented virilization and Cushing's syndrome,3 cases presented Cushing's syndrome,2 cases with increased aldosterone secretion,1 case with increased both aldosterone and cortisol,15 cases were nonfunctional tumors.3.All the patients were given B ultrasonography and the locating accuracy was 88%.17 patients were given computed tomography(CT) with a localization accuracy of 94.7%and a qualitation accuracy of 63.2%.6 patients were given magnetic resonance imaging(MRI) and the localization and qualitation accuracy were 100% and 16.7%,respectively.4.According to the Sullivan staging system,3 cases were stageⅠ,4 cases were stageⅡ,6 cases were stageⅢ,10 cases were stageⅣ.Among the 23 cases,20 were given surgery management first.Complete surgical resection was performed in 16 cases,and among the 16 cases postoperative radiotherapy was given in 2 eases and postoperative chemotherapy was given in another 2 cases.4 cases were treated by palliative resection.3 cases lost the opportunity of surgical therapy were given biopsy and confirmed pathologically.Among the rest 3 cases,1 case underwent interventional therapy,1 case underwent chemotherapy and 1 left without any treatment.5.The cases were followed up 2 to 70 months;median survival time was 20 months.6 patients are still alive till now.Postoperative recurrence occurred in 6 cases, and the recurrence time was 12-34 months(average 22.5 months).A repeat resection was performed in 5 cases which were reported adrenocortical adenoma in the first pathological report while they were confirmed to be adrenocortical carcinoma finally, one patient revealed hepatic metastasis and invasion of surrounding tissues.The other one recurrent case underwent conservative treatment only.Five-year survival by the Conclusions1.Primary adrenocortical carcinoma is rare.The rate of preoperatively diagnostic accuracy is very low.Without the malignant signs such as invasion of surrounding tissues,tumor thrombus,lymph node and distant metastasis,CT or MRI alone can't make an accurate diagnosis.But we should choose one of them to help us make surgical plan,because they can clearly show tumor thrombus,lymph node and distant metastasis and the relationship between the lesions and surrounding tissues.2.Pathological diagnosis is the diagnostic standard of ACC.If pathological diagnosis is unclear,immunohistochemical markers such as Ki-67,melan-A and inhibin-αcan provide important reference.3.Early diagnosis and treatment is the key to improve the prognosis of ACC and radical resection is the most effective treatment for ACC.Adjuvant therapy has a low efficiency.4.Patients should be followed up closely after operation so that local recurrence and metastasis can be found early.Patients with recurrence will have improved survival if the recurrence can be completely removed.