Clinical Study Of Rare Types Of ACTH-Independent Cushing's Syndrome

Objective:1.Primary Bilateral Macronodular Adrenal Hyperplasia(PBMAH)and Bilateral Adrenal Adenoma(BAA)are rare.Doctors have limited knowledge of these two diseases.This article compares the similarities and differences between the two diseases to help clinical differential diagnosis.2.To analyze how to determine the dominant side in patientswith Cushing's syndrome caused by Bilateral Adrenal Adenoma(BAA),and to explore therole of tumor size and Adrenal Venous Sampling(AVS)results in determining the dominant side before operation.Methods:1.The clinical data of 54 PBMAH patients and 12 BAA patients were retrospectively reviewed.The general condition,clinical manifestation,metabolic index,endocrine hormone level,imaging characteristics and treatment prognosis of the two groups were compared and analyzed.2.The clinical data of 11 patients with CS caused by BAA were retrospectively analyzed.The patients with successful AVS examination were operatedon according to the results of the dominant side.The other patients preferred to resect thelarger side of the tumor.The therapeutic effect and prognosis were compared.Results:1.The ratio of male to female in PBMAH group was more than 1(29:25),andthat in BAA group was less than 1(1:11).The average age of first visit in PBMAH group!was older than that in BAA group[(52.2±9.6)vs.(44.4± 12.3),P=0.019].There was significant difference between the two groups;The ratio of CS to Subclinical Cushing's Syndrome(SCS)in PBMAH group was 22:32,and that of CS to SCS in BAA group was 7:5.There was no significant difference between the two groups.There was significantdifference in the incidence of lower limb edema between the two groups,but no significantdifference in other clinical manifestations.Among the first imaging examinations of lesions,CT examination was the highest in both groups,accounting for 72.2%in PBMAH group and 83.3%in BAA group,respectively;The mean or median of cortisol and 24 hour Urinary Free Cortisol(24-hour UFC)in PBMAH group were lower than those in BAA group in the basic state or after Low Dose Dexamethasone Suppression Test(LDDST)and High Dose Dexamethasone Suppression Test(HDDST);The main CT features of PBMAH patients were bilateral adrenal enlargement and nodular hyperplasia with low or equal density.The pathological findings were adrenal cortical adenoma or cortical nodular(adenomatous)hyperplasia;Among 54 PBMAH patients,32 patients(16 CS patients and 16 SCS patients)underwent surgical treatment.25 cases(25/32)underwent unilateral adrenalectomy,7 cases(7/32)underwent bilateral adrenalectomy;17 cases(17/25)underwent unilateral adrenalectomy,and 3 cases(3/7)underwent regular follow-up evaluation.Among them,1 case(1/3)underwent residual adrenal tissue enlargement and reoperation;The remaining 22 patients(6 CS and 16 SCS)were treated with drugs only for complications or complications.2.6 patients underwent AVS and 3 cases was failed;Among 3 patients with successful AVS,the larger side of the tumor was the dominant side in 2 patients and the lesser side of the tumor was the dominant side in 1 patient;Symptoms and signs of 1 patient with clinical CS were improved than before after the operation.The clinical manifestations of 2 patients with SCS did not change.All the patients were followed up,during which their endocrine indicators were better than those before the operation.Blood pressure and blood glucose in patients with hypertension and diabetes were easier to control than before;The typical symptoms and signs of CS patients(6/8)were improved after resection of the larger side of the tumors.The clinical manifestations of SCS patients(2/8)remained unchanged.The endocrine indexes of 5(5/8)patients with long-term follow-up were improved compared with those before operation.Conclusions:1.Male patients with PBMAH are more than females,and the diagnosis age is older than BAA patients;SCS was more common in PBMAH patients than in CS patients,and cortisol secretion in adrenal lesions was lower than that in BAA patients.Adrenal CT was characterized by bilateral enlargement of adrenal glands and macronodule hyperplasia.Individualized treatment should be selected according to the severity of the patient's condition.2.Successful AVS can play an important role in preoperative preparation and evaluation in patients with BAA induced CS,but it has high technical requirements and low success rate.If AVS is unconditional,the larger side of the tumor can be excised first and followed up.