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.

In pheochromocytoma and paraganglioma (PPGL), mutations in the TCA cycle related genes may lead to the disturbance of the TCA cycle's intermediate metabolite levels, then cause pseudohypoxia by abnormal hypoxia-inducible factor ( HIF) activation, it also may lead to epigenetic changes, impede cell differentiation and apoptosis, and finally promote the occurrence and development of PPGL. This review focus on the research advances on the TCA cycle in the pathogenesis of PPGL, as well as the application of relevant detection methods.

Objective The aim of this study was to compare the efficacy and safety of spironolactone in mono-therapy versus low dose spironolactone in combined-therapy for the treatment of idiopathic hyperaldosteronism (IHA). Methods This was a prospective and randomized study. After 2 weeks wash out period, 48 patients confirmed IHA were assigned to either spironolactone mono-therapy or low dose spironolactone combined-therapy groups. All patients underwent adrenal venous sampling and showed no lateralization. The primary outcome was the percentage of patients with blood pressure<140/90 mmHg(1 mmHg＝0.133 kPa) at 16 weeks. The other observatory parameters were: the occurrence of gynecomastia, renal insufficiency, and hyperkalemia. Results At the end of 16 weeks, the patients′blood pressure were normalized in 17 out of 24 patients(70.8%) in mono-therapy group and in 23 out of 24 patients ( 95. 8%) in combined-therapy group ( P<0. 05). There was no hyperkalemia in both groups. 2 patients and 5 patients in mono-therapy group were observed renal insufficiency and gynecomastia respectively, while no patients in combined-therapy group presented with those side effects. Conclusion Low dose spironolactone combined-therapy group is more effective to control blood pressure and hypokalemia without increase the occurrence of renal insufficiency or gynecomastia.

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 investigate the clinical characteristics in patients with bilateral macronodular adrenal hyperplasia ( BMAH). Methods We enrolled 357 patients with adrenal tumors including 42 BMAH, 167 cortisol-producing adrenal adenoma (CPA), and 148 non-functional adenomas (NFA). The clinical manifestations, hormonal explorations and complications were compared among groups. Results (1) Age at diagnosis of BMAH group was significantly higher than that of CPA group [(53.7±8.3 vs 41.1±13.1)years], female proportion of BMAH group was lower that that of CPA group(54.8%vs 88.0%, P＜0.01). Both were similar to NFA group. (2) Compared to NFA group, metabolic abnormalities were severer in BMAH and CPA groups. Prevalence of hypertension, diabetes mellitus, and hypokalemia were the highest in BMAH group. ( 3) BMAH patients had lower midnight serum cortisol, 24 hour urine free cortisol, and adrenocorticotrophic hormone levels than CPA patients (P＝0.008, 0.010, and P＝0.001, respectively). The suppression rate of serum cortisol after low-dose dexamethasone suppression test was significantly higher in BMAH group ( P＝ 0. 005). Conclusion BMAH patients were at higher age at diagnosis. Although hypercortisolism in BMAH patients is relatively slight, metabolic abnormalities are more severe. Besides functional diagnosis, physicians should pay more attention to the assessment of complications related to hypercortisolism.

Clinical data of 116 cases diagnosed as primary empty sella (PES) from 2012 to 2016 in the Department of Endocrine and Metabolic Diseases, Ruijin Hospital were analyzed in this study. Insulin-induced hypoglycemia test and magnetic resonance imaging (MRI) were applied to explore the predictive factors of hypopituitarism in patients with PES. The results showed that 43 (37. 07%) patients with PES were with hypopituitarism. Pituitary height<2. 35 mm and pituitary sagittal area<23. 5 mm2indicated a high risk of hypopituitarism in patients with PES.

A 57-year-old female patient was admitted to our hospital because of sustained hyponatremia within stress dose of hydrocortisone replacement after transsphenoidal partial pituitary tumor surgery. Multiple clinical parameters investigation for 2 weeks was shown increasing serum Na+and uric acid level and decreasing blood pressure level under water restriction about 500 ml/d, and even without hydrocortisone replacement, the serum Na+ still maintain normal level. After surgeries and radiotherapy, the tumor was undetectable and serum Na+ level was within the normal range without any water restriction.

[Summary] All patients with cholesterol side-chain cleavage enzyme ( P450scc) deficiency that have been reported presented with early adrenal failure. Here we described a 35-year-old male presented with infertility as the only initial presenting complaint. He had received two separate surgeries to remove bilateral testicular masses. We reevaluated the resected tumors and found testicular adrenal rest tumor ( TART) pathology in the resected tumor. We profiled steroid hormones and found significantly elevated ACTH. CT scan revealed bilateral adrenal hyperplasia. Mutation screening identified compound heterozygous mutations (R353W and P432L) in the P450scc encoding gene (CYP11A1). The patient was finally diagnosed as congenital adrenal hyperplasia.