von Hippel Lindau syndrome is a rare genetic disease which may present with bilateral adrenal masses requiring surgical intervention. Previous practice at UP-PGH was to perform outright total adrenalectomy on pathologic adrenal glands and rely on lifelong steroid replacement for patients who had both adrenals removed. Presented here is a case of a patient diagnosed with von Hippel Lindau syndrome with bilateral adrenal masses, surgically managed initially with open adrenalectomy on the right side, followed by the first ever performed minimally invasive cortical sparing adrenalectomy at UP-PGH on the left side.
Adrenalectomy

Primary aldosteronism (PA) is the most common form of secondary hypertension, with its main manifestations including hypertension and hypokalemia. Early identification of PA is extremely important as PA patients can easily develop cardiovascular complications such as atrial fibrillation, stroke, and myocardial infarction. The past decade has witnessed the rapid advances in the genetics of PA, which has shed new light on PA treatment. While surgery is the first choice for unilateral diseases, bilateral lesions can be treated with mineralocorticoid receptor antagonists (MRAs). The next-generation non-steroidal MRAs are under investigations. New medications including calcium channel blockers, macrophage antibiotics, and aldosterone synthase inhibitors have provided a new perspective for the medical treatment of PA.
Humans ; Hyperaldosteronism/complications* ; Adrenalectomy/adverse effects* ; Aldosterone/therapeutic use* ; Hypertension/drug therapy* ; Mineralocorticoid Receptor Antagonists/therapeutic use*

Background: Unilateral adrenalectomy is indicated for patients with unilateral primary aldosteronism resulting in normalization of hypokalemia and resolution of hypertension. This study aims to determine the proportion of patients with cure of hypertension and improvement of hypokalemia after unilateral adrenalectomy among patients with aldosterone-producing adenoma and assess the association of preoperative factors with these outcomes. Methods: This is a retrospective cohort study among patients with aldosterone-producing adenoma who underwent unilateral adrenalectomy with at least one month follow-up after the operation. Patients were selected from admissions to the University of Santo Tomas Hospital from January 2008 to November 2018. The proportion of patients with cure of hypertension and resolution of hypokalemia were determined. Binary logistic regression was used to determine preoperative factors associated with these outcomes. Results: Twenty-one patients were included in this study. Cure of hypertension was noted in 47.62% and 42.86% of patients within 24 hours of adrenalectomy and on follow-up, respectively. Improvement of hypokalemia was noted in 61.90% of patients within 24 hours of operation while all patients had improvement of hypokalemia on follow-up. Patients without preoperative hypokalemia were more likely to have cure of hypertension within 24 hours of adrenalectomy (OR=0.0250, p=0.005) and on follow-up (OR=0.0571, p=0.010). Conclusion Unilateral adrenalectomy results in improvement of hypertension and hypokalemia in the majority of patients with aldosterone-producing adenoma. Shorter duration of hypertension and absence of preoperative hypokalemia were significantly associated with cure of hypertension after unilateral adrenalectomy.
Hyperaldosteronism ; Adrenalectomy

OBJECTIVE: To analyze the risk factors of persistent hypertension in patients who underwent adrenalectomy for primary aldosteronism and to evaluate the predictive value of the aldosteronoma resolution score (ARS) scoring system for surgical outcomes of adrenalectomy for primary aldosteronism. METHODS: We reviewed the clinical characteristics of patients who underwent adrenalectomy for primary aldosteronism from 2018 to 2021 at Peking University People' s Hospital to recognize risk factors of uncured hypertension after surgery. Based on the patient' s clinical outcomes, the patients were divided into complete success group and partial/absent success group. Risk factors for persistent hypertension were analyzed. The value of the ARS scoring system was assessed by the area under the curve (AUC). RESULTS: In this study, 112 patients were included. Most of the patients benefited from the surgery for 94.6% were a complete or partial clinical success after follow-up for at least 6 months. According to postoperative hypertension status, the patients were divided into complete success group (51 cases) and partial/absent success group (61 cases). There were statistical differences between the two groups in age, body mass index (BMI), waist circumference, duration of hypertension, number of preoperative antihypertension medications, preoperative systolic blood pressure, history of diabetes, history of cardiovascular and cerebrovascular diseases, serum creatinine, estimated glomerular filtration rate(eGFR), high-density lipoprotein cholesterol and triglyceride. Logistic regression analysis showed that age (OR=1.111, 95%CI: 1.029-1.199), waist circumference (OR=1.073, 95%CI: 1.013-1.137), pre-operative systolic blood pressure (OR=1.033, 95%CI: 1.008-1.060) and history of cardiovascular and cerebrovascular diseases (OR=16.061, 95%CI: 1.312-196.612) were the risk factors for uncured hypertension in primary aldosteronism patients after surgery, but female gender not. The median ARS in the complete success group was 4 and in the partial/absent success group, it was 2. Among the patients with ARS of 4-5, the cure rate of hypertension was 76.5%. The area under the curve of ARS was 0.743. CONCLUSION The history of cardiovascular and cerebrovascular diseases is a significant risk factor for persistent hypertension after surgery in primary aldosteronism patients. ARS scoring system has a certain value in predicting the postoperative hypertension status of primary aldosteronism patients. However, further research is still needed on a prediction model for surgical outcomes of primary aldosteronism which is more suitable for the Chinese population is still needed.
Adrenalectomy/adverse effects* ; Blood Pressure ; Female ; Humans ; Hyperaldosteronism/surgery* ; Hypertension/etiology* ; Retrospective Studies ; Risk Factors ; Treatment Outcome

Hyperkalemia was one of the complications after primary aldosteronism surgery. Hyperkalemia after primary aldosteronism surgery was uncommon in clinical practice, especially persistent and serious hyperkalemia was rare. This complication was not attached great importance in clinical work. A case about persistent and serious hyperkalemia after primary aldosteronism adrenal adenoma surgery was reported and the patient was followed-up for fourteen months in this study. This patient had a laparoscopic adrenalectomy due to primary aldosteronism. Hyperkalemia was detected one month after surgery of this patient, the highest level of plasma potassium was 7.0 mmol/L. The patient felt skin itchy, nausea, palpitation. Plasma aldosterone concentration fell to 2.12 ng/dL post-operation from 35.69 ng/dL pre-operation, zona glomerulosa insufficiency was confirmed by hormonal tests in this patient after surgery. And levels of 24 hours urinary potassium excretion declined. Decrease of aldosterone levels after surgery might be the cause of hyperkalemia. Hyperkalemia lasted for 14 months after surgery and kalemia-lowering drugs were needed. A systemic search with "primary aldosteronism", "hyperkalemia", "surgical treatment" was performed in PubMed and Wanfang Database for articles published between January 2009 and December 2019. Literature review indicated that the incidence of hyperkalemia after primary aldosteronism surgery was 6% to 29%. Most of them was mild to moderator hyperkalemia (plasma potassium 5.5 to 6.0 mmol/L) and transient. 19% to 33% in hyperkalemia patients was persistent hyperkalemia. Previous studies in the levels of plasma potassium reached the level as high as 7 mmol/L in our case were rare. Whether hypoaldosteronemia was the cause of hyperkalemia was not consistent in the published studies. Risk factors of hyperkalemia after primary aldosteronism surgery included kidney dysfunction, old age, long duration of hypertention. This paper aimed to improve doctors' aweareness of hyperkalemia complication after primary aldosteronism surgery. Plasma potassium should be monitored closely after primary aldosteronism surgery, especially in the patients with risk factors. Some patients could have persistent and serious hyperkalemia, and need medicine treatment.
Adrenalectomy/adverse effects* ; Aldosterone/therapeutic use* ; Humans ; Hyperaldosteronism/surgery* ; Hyperkalemia/surgery* ; Potassium/therapeutic use*

Primary aldosteronism is the most common cause of secondary hypertension.This review focuses on the procedures related to surgical treatment and summarizes the available evidence.We analyzed the impact of primary aldosteronism on the body,the advantages of surgical treatment,the choice of patients and surgical methods,perioperative management,and surgical efficacy evaluation.Finally,we put forward the prospect of scientific research in this field,with a view to providing reference for clinical work.
Adrenalectomy ; Humans ; Hyperaldosteronism/surgery* ; Hypertension

Cushing’s syndrome due to ectopic adrenocorticotrophic hormone (ACTH) secretion is uncommon, accounting for 9 to 18% of cases; approximately 10% of ACTH producing tumours are caused by thymic carcinomas.1 We describe a young lady who presented with Cushing’s syndrome secondary to a primary neuroendocrine tumour (NET) arising from the thymus. She had surgical resection of her primary tumour with remission of her Cushing’s syndrome however subsequently went on to have locoregional recurrence followed by distant metastases to her bilateral ovaries. She underwent 6 surgeries including bilateral adrenalectomy and had 3 cycles of chemotherapy over the course of the 8 years since her diagnosis. Due to the rarity and highly aggressive nature of this disease, we highlight the need for a multidisciplinary team approach and use of multiple modalities in the management of our patient. Timely use of bilateral adrenalectomy particularly in young patients is important to prevent further complications and facilitate other treatment modalities.
Adrenalectomy ; ACTH Syndrome, Ectopic

A 57-year-old male patient was referred to our department with complaints of his right adrenal gland occupancy and hypertension about 6 months. When admitted to the hospital, the blood pressure was about 160/100 mmHg, and the heart rate was 110 beats/min. He was no obvious obesity, acne, abnormal mood, without weakness of limbs, acral numbness, palpitation and headache. He presented with type 2 diabetes for more than 3 years, with oral administration of metformin enteric coated tablets and subcutaneous injection of insulin glargine to control blood glucose, and satisfied with blood glucose control. Enhanced CT showed that: the right adrenal gland showed a kind of oval isodense, slightly hypodense shadow, the edge was clear, lobular change, the size was about 5.8 cm×5.4 cm, uneven density, there were nodular and strip calcification, round lipid containing area and strip low density area, and the CT value of solid part was about 34 HU. Enhanced scan showed heterogeneous nodular enhancement in the solid part of the right adrenal gland, nodular enhancement could be seen inside. The CT values of solid part in arterial phase, venous phase and delayed phase were 45 HU, 50 HU and 81 HU, respectively. Considering from the right adrenal gland, cortical cancer was more likely. No obvious abnormality was found in his endocrine examination. After adequate preoperative preparation, retroperitoneal laparoscopic adrenalectomy was performed under general anesthesia. During the operation, the 6 cm adrenal tumor was closely related to the inferior vena cava and liver, and after careful separation, the tumor was completely removed and normal adrenal tissue was preserved. The operation lasted 180 min and the blood loss was 100 mL, and the blood pressure was stable during and after the operation. There was no obvious complication. The results of pathological examinations were as follows: the size of the tumor was 7.5 cm×6.0 cm×3.5 cm, soft, with intact capsule and grayish-red cystic in section. Pathological diagnosis: (right adrenal gland) cavernous hemangioma, secondary intravascular thrombosis, old hemorrhagic infarction with calcification and ossification. After 6 months of observation, no obvious complications and tumor recurrence were found. In summary, cavernous hemangioma of adrenal gland is a rare histopathological change. Its essence is a malformed vascular mass. Blood retention is the cause of thrombosis and calcification in malformed vessels. The imaging findings were inhomogeneous enhancement of soft tissue masses, and the adrenal function examination showed no obvious abnormalities. Retroperitoneal laparoscopic surgery is feasible after adequate preoperative preparation. It is difficult to diagnose the disease preoperatively and needs to be confirmed by postoperative pathology.
Adrenal Gland Neoplasms/surgery* ; Adrenalectomy ; Diabetes Mellitus, Type 2 ; Hemangioma, Cavernous/surgery* ; Humans ; Laparoscopy ; Male ; Middle Aged ; Neoplasm Recurrence, Local

We report one case of estimated glomerular filtration rate (eGFR) decline after taking unilateral adrenalectomy due to aldosterone adenoma. A 60-year-old male with 23-year history of hypertension was reported to the endocrinologist due to hypokalemia (serum potassium 3.01 mmol/L). Urine microalbumin/creatinine (ALB/CR) was 70.15 mg/g, serum creatinine was 82 μmol/L and eGFR was 89.79 mL/(min·1.73 m²). Random serum aldosterone was 172.2-203.5 ng/L, and random plasma rennin activity was 0-0.17 μg/(L·h). His captopril challenge test suggested that his aldosterone le-vels were suppressed by 8% (< 30%) and the adrenal enhanced computed tomography scan revealed a left adrenal tumor. The patient was diagnosed with primary hyperaldosteronism (PA), aldosterone adenoma and underwent left laparoscopic adrenalectomy. Histological examination confirmed adrenal cortical adenoma. One week after the operation, his serum creatinine was increased to 127 μmol/L compared with preoperative level; eGFR was 32.34 mL/(min·1.73 m²). His systolic blood pressure (SBP) was 110 mmHg and diastolic blood pressure (DBP) was 60 mmHg (hypotensive drugs discontinued), and serum potassium level was 5.22 mmol/L. At the end of the 2-year follow up, the serum creatinine of this patient remained at 109-158 μmol/L and eGFR fluctuated from 63.28-40.12 mL/(min·1.73 m²). PA is one of the most common causes of secondary hypertension. Several studies have reported renal function deterioration of PA patients after unilateral adrenalectomy, like the patient in this article. Age, preoperative plasma aldosterone concentration, albuminuria and preoperative potassium level might be significant predictors of a decrease in the eGFR. Growing evidence suggests that aldosterone could contribute to structural kidney damage, arterial injury and hemodynamic disorder. At the same time, patients with PA exhibit glomerular hyperfiltration and glomerular vascular hypertension, leading to the misinterpretation of renal function in PA patients as subtle kidney damage may be masked by the glomerular hyperfiltration before treatment. After a unilateral adrenalectomy, glomerular hyperfiltration by aldosterone excess is resolved and renal damage can be unmasked. In conclusion, kidney function deterioration after adrenalectomy can be detected in some patients with PA. Thus, accurate evaluation of kidney function in patients with PA may be essential, especially for those with preoperative risk factors for postoperative renal impairment. After unilateral adrenalectomy, close monitoring of renal function and adequate management are required for PA patients.
Adrenal Gland Neoplasms/surgery* ; Adrenalectomy ; Glomerular Filtration Rate ; Humans ; Hyperaldosteronism/surgery* ; Male ; Middle Aged ; Renal Insufficiency, Chronic

Adrenocortical carcinoma is a rare type of endocrine malignancy with an annual incidence of approximately 1–2 cases per million. The majority of these tumors secrete cortisol, and a few secrete aldosterone or androgen. Estrogen-secreting adrenocortical carcinomas are extremely rare, irrespective of the secretion status of other adrenocortical hormones. Here, we report the case of a 53-year-old man with a cortisol and estrogen-secreting adrenocortical carcinoma. The patient presented with gynecomastia and abdominal discomfort. Radiological assessment revealed a tumor measuring 21×15.3×12 cm localized to the retroperitoneum. A hormonal evaluation revealed increased levels of estradiol, dehydroepiandrosterone sulfate, and cortisol. The patient underwent a right adrenalectomy, and the pathological examination revealed an adrenocortical carcinoma with a Weiss' score of 6. After surgery, he was treated with adjuvant radiotherapy. Twenty-one months after treatment, the patient remains alive with no evidence of recurrence.
Adrenal Gland Neoplasms ; Adrenalectomy ; Adrenocortical Carcinoma ; Aldosterone ; Dehydroepiandrosterone Sulfate ; Estradiol ; Gynecomastia ; Humans ; Hydrocortisone ; Incidence ; Male ; Middle Aged ; Radiotherapy, Adjuvant ; Recurrence