Objective:To evaluate the efficacy and safety of unilateral adrenalectomy for treating primary bilateral macronodular adrenal hyperplasia (PBMAH) of different clinical types.Methods:The clinical and biochemical data of 54 patients with PBMAH who underwent unilateral adrenalectomy from May 2008 to March 2023 were retrospectively collected. Preoperative CT images of all patients showed enlarged bilateral adrenal glands with multiple nodules of " fused masses". Mean preoperative blood cortisol concentration at 8am was (21.5±7.7)μg/dl, urinary free cortisol concentration was (442.6±300.4)μg/24h, and mean 8am ACTH concentration was (6.4±2.3)pg/ml. Postoperative symptoms, BMI, blood pressure, mass diameter, cortisol and ACTH concentration were recorded and analyzed.Results:Compared with ordinary laparoscopic surgery, robot-assisted surgery showed shorter operation time [(115.4±22.1)min vs.(95.0±19.8)min, P=0.045]; less blood loss [(118.2±57.0)ml vs. (125.6±45.3)ml, P=0.441] and shorter hospitalization time [(5.2±0.9)day vs. (6.4±1.2)day, P=0.279]. Compared with laparoscopic surgery, open surgery showed longer operation time [(134 34.5) min vs. (104.3±20.1) min, P=0.035]; more blood loss [(305.5±85.2) ml vs. (122.5±44.3) ml, P=0.012] and longer hospitalization time[(10.4±3.2)day vs. (5.7±1.0) day, P=0.020]. The average follow-up time was (23.7±11.7) months. Sixteen cases biochemically relapsed, and the average relapse-free time was (25.4±13.4) month. Mean postoperative systolic blood pressure was (131.1±16.8)mmHg ( P=0.001) while diastolic blood pressure decreased to (82.2±11.1)mmHg ( P=0.002). Postsurgical average blood cortisol concentration decreased to (10.2±4.0)μg/dl ( P<0.01), while urine cortisol concentration decreased to (106.6±43.4)μg/24h( P<0.01). Average ACTH concentration increased to (12.6±4.1)pg/ml( P=0.005). Recurrent patients had higher preoperative blood and urine cortisol concentration(24.7±8.2)μg/dl( P=0.046), (522.8±234.2)μg/24h( P=0.028), and all of them underwent contralateral adrenalectomy. Conclusions:Unilateral adrenalectomy is safe and effective for treatment of PBMAH while part of patients biochemically relapsed. Subclinical patients were observed no recurrent cases after surgery. Recurrent patients have higher preoperative blood and urine cortisol levels and should undertake contralateral adrenalectomy and supplement corticosteroids for whole life.

Objective:To investigate the clinical profile of primary bilateral macronodular adrenal hyperplasia(PBMAH) and sex difference.Methods:One hundred and forty cases of PBMAH were recruited in our center from 2014, and all patients were evaluated for hormone secretion, adrenal imaging, and metabolic parameters.Results:Overt Cushing′s syndrome accounted for 76.4% of PBMAH cohort and 47.9% were female. The overt group had higher serum cortisol and 24 h urinary free cortisol levels, lower adrenocorticotropic hormone, higher serum cortisol after low-dose dexamethasone suppression tests, larger total adrenal size, and a higher percentage of obesity, hypertension, diabetes mellitus, and hypokalemia than the subclinical group(all P<0.05). When compared with the male group, the female group had smaller adrenal size( P<0.001), lower HbA 1C( P=0.003), higher total cholesterol( P=0.005), and lower density lipoprotein-cholesterol levels( P=0.035). Further, 24 h urinary free cortisol in the male group was found to be positively correlated with diastolic blood pressure, fasting glucose, 2 h postprandial glucose after oral glucose tolerance test(OGTT), and HbA 1C after adjusted for age, body mass index, and onset duration, and was negatively correlated with body mass index and potassium levels. While 24 h urinary free cortisol in the female group was positively correlated only with diastolic blood pressure, fasting glucose, and 2 h postprandial glucose after OGTT(all P<0.05). During follow-up, 80.0% of patients achieved remission after unilateral adrenalectomy, with a recurrence rate of 17.9%. Conclusion:PBMAH related metabolic disorder is more pronounced in overt Cushing′s syndrome and males. Unilateral adrenalectomy as an effective treatment can benefit the majority of patients.

Objective To discuss the clinical characteristics of adrenal metastases,and summarize the experience of diagnosis and treatment.Methods From January 2008 to June 2018,the clinical data of 55 patients with adrenal metastases treated in our hospital were analyzed retrospectively.This study included 34 male patients and 21 female patients and the median age was 60 years old (ranged 55 to 84 years old).The median value of maximum diameter of adrenal metastases was 3 cm (ranged 1.9 to 10.3 cm);with 35 cases on the left side,13 cases right and 7 cases bilateral.The primary sites of malignant tumors were pancreas (18 cases,32.7％),lung (12 cases,21.8％),liver (6 cases,10.9％) and colorectum (6 cases,10.9％),respectively.Thirty-four cases were confirmed by pathology after adrenalectomy and 21 cases were confirmed by needle biopsy.Thirty cases were diagnosed synchronously with the primary tumor and 25 cases were metachronous.The median time from diagnosis of primary tumors was 13.3 months (ranged 2.0 to 97.4 months).42 cases of these 55 cases were diagnosed within one year.Treatment options for adrenal metastatic lesions included single adrenalectomy in 18 cases,adrenalectomy combined with radiotherapy 16 cases,single intravenous chemotherapy 10 cases,intravenous chemotherapy combined with non-operative treatment 10 cases and single radiotherapy 1 case.Results The main pathological types were adenocarcinoma (19 cases,34.5％),ductal adenocarcinoma (10 cases,18.2％),hepatocellular carcinoma (6 cases,10.9％) and clear cell carcinoma (4 cases,7.3％).Two cases were lost follow-up and the follow-up rate was 96％,the median follow-up time was 8 months (ranged 1 to 135 months).The median overall survival (OS) time of 55 patients was 5.3 months (ranged 1 to 134 months).The one-year survival rate was 23.6％ (13/55),the two-year survival rate was 12.7％ (7/55),the three-year survival rate was 9.1％ (5/55) and the five-year survival rate was 1.8％ (1/55).Conclusions Pancreatic cancer was the most common type of malignant tumor for adrenal metastases in our hospital.Most primary tumors and adrenal metastases were diagnosed synchronously or within one year.Comprehensive treatment with retroperitoneal adrenalectomy may improve the OS,however the overall prognosis is poor.

Objective To explore the expressions of hypoxia inducible factor ( HIF), vascular endothelial growth factor receptor 2 ( VEGFR 2), and microvessel density ( MVD) in adrenocortical adenoma ( ACA) and adrenocortical carcinoma ( ACC), in order to discuss their potential role in the development of adrenal tumours. Methods Fifty-five adrenal tumour specimens resected in the hospital with complete clinical data (including 30 ACA cases and 25 ACC cases) were examined by immunohistochemistry for the expressions of HIF-2α, HIF-1α, VEGFR 2, and MVD. Results VEGFR 2 and MVD up-regulated were found in the ACC group (P＜0.05). The expression of HIF-2α and HIF-1α correlated with VEGFR 2 (P＜0.05). The expressions of VEGFR 2 and MVD were related to some clinicopathological features ( P＜0. 05 ). Additionally, tumour size, expression of VEGFR 2 and MVD were independently associated with ACC (P＜0.05). Conclusions The high expression of HIF-2α, VEGFR 2, and MVD in adrenal tumours suggested their roles in tumour angiogenesis, which indicated that anti-angiogenesis therapies deserve intensive studies for malignant adrenocortical tumours.

Objective To investigate the methods and efficacy of treatment on severe adrenal Cushing′s syndrome. Methods The clinical data of 22 cases with severe adrenal Cushing′s syndrome ( severe group) , and 136 cases with mild or moderate adrenal Cushing′s syndrome ( non-severe group) were reviewed. The clinical features were analyzed by comparing the differences between these two groups when patients were admitted to hospital. We discussed the clinical managements of patients with severe adrenal Cushing′s syndrome by comparing the differences with non-severe group after preoperative preparation, and with themselves before and after preoperative preparation. The effects of surgery were evaluated by comparing the differences between pre-operation and post-operation on patients with severe adrenal Cushing′s syndrome. Results At admission, serum/urine cortisol, disease course, and blood pressure were significantly higher in the severe group than those in non-severe group ( P＜0.05 or P＜0.01) , serum potassium and ACTH level were decreased significantly in the severe group than those innon-severegroup[(3.01±0.75vs3.62±0.48)mmol/L,P＜0.01;(6.47±2.91vs8.21±3.22)pg/ml,P＜0.01] . However, no significant difference was observed in diastolic blood pressure, serum potassium, and fasting plasma glucose between these two groups after preoperative preparation (all P＞0.05). And then, we performed adrenalectomy. The symptoms of 22 cases with severe adrenal Cushing′s syndrome were obviously alleviated after 3 months. During follow-up, 5 cases of primary bilateral macronodular adrenal hyperplasia ( BMAH) and 1 case of primary pigmented nodular adrenocortical disease ( PPNAD ) were treated with contralateral adrenalectomy. Conclusion Sufficient preoperative preparation is essential for patients with severe adrenal Cushing′s syndrome because of its high level serum cortisol with severe complications. If preparation fails before surgery, cortisol-lowering medication or emergency unilateral adrenalectomy is necessary. Severe patients with BMAH and PPNAD were firstly performed unilateral adrenalectomy and followed-up closely, and then, contralateral adrenalectomy is needed when the recurrence of hypercortisolism recognized.

Objective To explore the efficacy and safety of minimal invasive adrenal sparing surgery for the treatment of Cushing syndrome caused by adrenocortical adenoma.Methods Patients who underwent minimal invasive adrenal surgery for adrenocortical adenoma in our institution from January 2010 to December 2015 were retrospectively analyzed.Preoperative, intraoperative, and postoperative variables were reviewed from the database.The mean patient age at diagnosis was 39 years and male:female ratio was 10:111.Of the 121 adenomas, 50 were located in the right adrenal and 71 in the left.The mean tumor size was 2.6 cm.84 cases had hypertension (69.4％), 36 cases had diabetes mellitus (29.8％), and 45 cases had obesity (37.2％).Postoperative follow-up was performed by evaluating adrenal gland function and imaging.Results Mininal invasive partial adrenalectomy was performed in 121 cases and the success rate of operation was 99.2％ (120/121).Sixty-two cases received operation through retroperitoneal approach,and 59 cases were through transperitoneal approach.The median operative time was 50 min with a median blood loss of 50 ml.The mean postoperative hospital stay were (5.0 ± 3.2) days.The vascular injury occurred in 2 cases (1 case in each surgical approach), while the abdominal organ injury occurred in 2 cases with 1 case of spleen injury and 1 case of liver injury (both in transperitoneal approach).Postoperative complications were observed in 6 cases:1 case of deep venous thrombosis, 1 case of wound hematoma, 4 cases of wound infection.Cortisol substitution was given in 2 to 12 months (mean 6.2 months)postoperatively.One year after operation, the remission rate of hypertension, diabetes and obesity was 58.3％ (49/84), 30.6％ (11/36) and 60.0％ (30/45), respectively.Conclusions Minimal invasive adrenal surgery using retroperitoneal and transperitoneal laparoscopic technique can be performed with low morbidity and achieve an excellent outcome.The perioperative hormone therapy may also play an important role.

_ Objective_ To evaluate the efficacy of unilateral subtotal adrenalectomy in the treatment of bilateral adrenal solitary neoplasma causing Cushing's syndrome and to elaborate the therapeutic principle. Methods From 2007 to 2013, a total of ten patients were diagnosed with Cushing's syndrome caused by bilateral solitary adrenal neoplasma. We compared patients'clinical symptoms, hormone profiles, biochemical and metabolic parameters, and imaging data before and after the surgery. Five of them chose the optimal neoplasma based on the lateralization ratio of adrenal venous sampling result and the other 5 patients chose the optimal neoplasma based on the diameter of the mass reflected by the computed tomography result and were then operated. Results After the unilateral subtotal adrenalectomy,the24-hour urinary free cortisol decreased significantly(P<0.05)and the midnight serum cortisol level also significantly reduced(P<0. 01). Plasma adrenocorticotropic hormone level increased significantly(P<0. 01). Nine patients of them did not need contralateral adrenalectomy and one patient received contralateral adrelectomy because of the remnant of Cushingnoid symptoms. Conclusion Unilateral subtotal adrenalectomy is an effective and safe way to treat Cushing's syndrome caused by bilateral solitary neoplasma.

Objective To review the experience with partial cystectomy combined with chemo-and radiation therapies in the treatment of muscle-invasive bladder cancer (MIBC) to assess the local control and survival rates,and to identify predictive factors for recurrence and survival.Methods From 2002 through 2007,a total of 100 patients with MIBC underwent partial cystectomy combined with adjuvant chemotherapy and radiation therapy (PC group).Meanwhile,36 patients with MIBC underwent radical cystectomy (RC group).The clinical and pathological data of these patients were retrospectively reviewed.Primary endpoints were cancer-specific survival (CSS),bladder-intact cancer-specific survival and bladder cancer recurrence.Results The 5-year CSS rate of the entire cohort was 65％,which was higher in PC group than in RC group (68％ vs 55％,P =0.033).In PC group,only 2 patients (2％) were confirmed to have residual tumor at the time of re-evaluation TUR 3 months after partial cystectomy.After a mean of 33.1 months,46 patients (46％) experienced superficial recurrence and 14 patients (14％) developed muscle-invasive recurrence.75％ of recurrence occurred within 16 months.8 patients underwent salvage cystectomy.The 5-year bladder-intact survival rate was 63％ in PC group.In multivariate analysis,the presence of tumor numbers more than 3 and tumors with infiltrating growth pattern were 2 predictive factors for cancer recurrence in PC group.In terms of survival,the presence of tumor numbers more than 3,lymphovascular invasion and partial cystectomy plus ureteral reimplantation (PC plus UR) were significantly associated with 5-y CSS in PC group and PC plus UR was indeed a protective factor for survival.By looking at the entire MIBC cohort,lymphovascular invasion,tumor numbers more than 3,history of superficial bladder cancer and age greater than 70 years old were identified as independent predictive factors for 5-y CSS.Conclusions Combined with adjuvant chemo-and radiation therapies,partial cystectomy might be a alternative to radical cystectomy for the treatment of MIBC,which provides adequate local control in selected patients,as well as acceptable survival rate.

Objective To improve the diagnosis and treatment of ACTH-independent macronodular adrenal hyperplasia(AIMAH).Methods A 51-year-old female patient with Cushing's syndrome caused by AIMAH was reported.Elevated early morning plasma cortisol levels,increased 24 h urinary free cortisol excretion,and loss of the normal circadian rhythm in cortisol secretion were presented.There was no suppression of cortisol secretion by administration of low-and high-dose overnight dexamethasone suppression test.Cardio-pulmonary function was very bad with the highest blood pressure reaching 300/120 mm Hg( 1 mm Hg=0.133 kPa).Initially,she was treated with mitotane(60 mg/d),but was not effective.After taking ketoconazole (800 mg/d)for 5 days,cardio-pulmonary function was not effectively improved with blood pressure only descending to 180/120 mm Hg.Orthopnoea appeared and Spo2fell once to 75％.The patient had to undergo right total adrenalectomy immediately.ResultsThe mass resected was 10 cm× 10 cm in size and weighted 67.5 g.Histological examination of the removed adrenal revealed nonpigmented macronodular cortical hyperplasia.The patient continued to take ketoconazole (400-800 mg/d)from the 6th day of the operation without steroid replacement therapy in that period.With normal cortisol levels( plasma cortisol at 8:00 was 18.65 μg/dl,24 h urinary free cortisol was 78.75 μmol),she left hospital after the general condition had been improved.During the following updated 10 months follow up,the indexes of her laboratory examination were maintained normal.ConclusionIndividualized therapy should be adopted for the patient with AIMAH.The medication is useful to suppress the adrenal gland cortisol production for those with progression of symptoms,very high blood pressure,hypokalemia and hypoproteinemia.Once the cardio-pulmonary function improves,the target organ should be resected as soon as possible.The supplement of cortisol is not appropriate during the perioperative period.The unilateral adrenalectomy is an effective treatment for AIMAH.

Objective To improve the diagnosis and treatment ot adrenocorticotropin-independent macornodular adrenal hyperplasia (AIMAH).Methods The clinical data of 17 cases with AIMAH from 2000 to 2011 were analyzed retrospectively,including 3 subclinical AIMAH,10 clinical AIMAH and 4 highrisk AIMAH patient,with common radiological characteristic of bilaterally enlarged adrenal glands with multiple nodules like ginger.The 3 cases of subclinical AIMAH patients presented with decreased serum ACTH,normal or slightly elevated plasma cortisol and urinary free cortisol level,no suppression following 1 mg overnight dexamethasone suppression test and absence of clinical signs of Cushing syndrome (CS).While clinical AIMAH and high-risk AIMAH presented with clinical signs of CS,elevated plasma cortisol and urinary free cortisol level,suppressed serum ACTH,loss of normal circadian rhythm in cortisol secretion and no suppression following the low-dose and high-dose overnight dexamethasone suppression test.Among the 4 cases of high-risk AIMAH,2 cases presented with osteoporosis,2 cases with hepatic dysfunction,3 cases with cardiopulmonary dysfunction,and 4 cases with severe hypertension.Three cases of subclinical AIMAH were treated with symptomatic treatment,10 cases of clinical AIMAH patients with surgical operation,4 cases of high-risk AIMAH patients with ketoconazole and surgical operation.Results Three subclinical AIMAH patients received symptomatic treatment and discharged from hospital with normal blood pressure and blood glucose.During the period of follow-up from 3 months to 3 years,endocrine results were normal.Seven clinical AIM AH patients underwent unilateral adrenal tumor resection plus ipsilateral partial adrenalectomy or total adrenalectomy.CS disappeared completely after 6 to 9 months.Two clinical AIMAH patients underwent simultaneous bilateral adrenalectomy.One case died of adrenal crisis after operation,and the other case presented with adrenal insufficiency but returned to normal after glucocorticoid replacement therapy,no Nelson's syndrome happened during the follow-up for 5 years.One clinical AIMAH patient undertook unilateral adrenalectomy twice by interval,followed by routine corticosteroid replacement therapy.Followed up for 10 years,no Nelson's syndrome happened.Four high-risk AIMAH patients received ketoconazole and then underwent right total adrenalectomy.Cortisol levels returned to normal after 1 to 2 months and during the follow-up for 1 to 3 years,the laboratory examinations maintained normal.Conclusions Different treatment methods should be adapted to different subtypes of AIMAH.For subclinical AIMAH,the principal treatment is symptomatic,and close follow-up with regular adrenal imaging and endocrine examination is required.Surgical operation should be performed when clinical symptoms of AIMAH appear.Medical management is essential for high-risk AIMAH to inhibit the production of cortisol at first.Once these patients could stand the stimulation caused by operation,the adrenal glands should be resected as soon as possible.The unilateral adrenalectomy is an effective treatment for clinical AIMAH.