Adrenalectomy for primary aldosteronism and its related surgical characteristics.

Primary aldosteronism (PA) is a common cause of secondary hypertension. Adrenalectomy is an effective treatment for unilateral PA, particularly aldosterone-producing adenoma (APA), resulting in improvements in biochemical parameters and blood pressure in the vast majority of patients. The article provides a comprehensive overview of PA, focusing on the outcomes of adrenalectomy for PA and the factors that may suggest prognostic implications. Analysis of the outcome of different PA patients undergoing adrenalectomy in terms of preoperative factors, vascular and adipose conditions, type of pathology, and somatic variants. In addition, it is recommended to use the histopathology of primary aldosteronism (HISTALDO) consensus to classify the patient's pathological type, with classical and nonclassical pathological types showing a different prognosis and possibly being associated with an unresected contralateral adrenal gland. The primary aldosteronism surgical outcome (PASO) consensus sets uniform standards for postoperative outcomes in unilateral PA, but its setting of thresholds remains controversial. Partial adrenalectomy shows similar surgical results and fewer postoperative complications than total adrenalectomy, but there is a risk of missing the true source of abnormal aldosterone secretion. Steroid profiling and functional imaging techniques offer alternative options to adrenal vein sampling (AVS) for unilateral and bilateral judgments in patients with PA. A combination of factors is needed to predict the prognosis of PA patients undergoing adrenalectomy in order to manage patient expectations of the outcome of the procedure and to closely monitor blood pressure and biochemical parameters in patients who suggest a poorer prognosis.

Adrenal cysts less than 10 cm can be safely observed.

BACKGROUND: Adrenal cysts are rare and appropriate management is unclear due to a lack of data on their natural history. Understanding adrenal cyst growth patterns would assist in clinical management. METHODS: This single-institution study included all adult patients diagnosed with simple adrenal cysts between 2004 and 2021. Baseline characteristics and outcomes of those who underwent resection (ADX) or observation (OBS) were compared using the chi-squared test, student's t-test, and Wilcoxon rank-sum test. Growth curves and sensitivity analysis were plotted for all patients who had follow-up imaging. RESULTS: We identified 77 patients with imaging-confirmed adrenal cysts. The majority were female (75.3%) and more than half were white (55.8%). One-third of patients underwent ADX, and the remaining were observed. ADX patients were younger (median age [IQR]: 55.5 y [45.0-68.2 y] vs. 44.2 y [38.7-55.0 y], p = 0.01) and more likely to be Hispanic (12% vs. 0%, p = 0.05). ADX patients presented with larger cysts (5.6 vs. 2.6 cm, p = 0.002). The median time from diagnosis to last follow-up was 1.1 y for ADX and 4.1 y for OBS. Average growth for OBS was 0.3 cm/y, while average growth for ADX was 3.9 cm/y. In ADX patients, cysts >10 cm grew significantly faster than cysts <10 cm (median growth rate 13.2 cm/y vs. 0.3 cm/y, p < 0.05). There was no adrenal malignancy diagnosis, hyperfunctionality, or observation-related complications (e.g., rupture). CONCLUSION: While size >4-6 cm has guided surgical referral for solid adrenal masses, this study demonstrates a size threshold of 10 cm, below which asymptomatic, simple adrenal cysts can safely be observed.

The effect of mass functionality on laparoscopic adrenalectomy outcomes.

PURPOSE: This study aimed to determine the effect of adrenal mass functionality and different hormone subtypes synthesized by the adrenal masses on laparoscopic adrenalectomy (LA) outcomes. MATERIALS AND METHODS: The study included 298 patients, 154 of whom were diagnosed with nonfunctional masses. In the functional group, 33, 62, and 59 patients had Conn syndrome, Cushing's syndrome, and pheochromocytoma, respectively. The variables were analyzed between the functional and nonfunctional groups and then compared among functional masses through subgroup analysis. RESULTS: The incidence of diabetes mellitus, hypertension, and obesity, blood loss, and length of hospital stay (LOH) were significantly higher in the functional group than in the nonfunctional group. In the subgroup analysis, patients with pheochromocytoma had significantly lower body mass index but significantly higher mass size, blood loss, and LOH than the other two groups. A positive correlation was found between mass size and blood loss in patients with pheochromocytoma (p ≤ 0.001, r = 0.761). However, no significant difference in complications was found among the groups. CONCLUSIONS: In this study, patients with functional adrenal masses had higher comorbidity rates and American Society of Anesthesiologists scores. Moreover, blood loss and LOH were longer on patients with functional adrenal masses who underwent LA. Mass size, blood loss, and LOH in patients with pheochromocytoma were significantly longer than those in patients with other functional adrenal masses. Thus, mass functionality did not increase the complications.

Side-specific factors for intraoperative hemodynamic instability in laparoscopic adrenalectomy for pheochromocytoma: a comparative study.

BACKGROUND: Adrenalectomy for pheochromocytoma (PHEO) is challenging because of the high risk of intraoperative hemodynamic instability (HDI). This study aimed to compare the incidence and risk factors of intraoperative HDI between laparoscopic left adrenalectomy (LLA) and laparoscopic right adrenalectomy (LRA). METHODS: We retrospectively analyzed two hundred and seventy-one patients aged > 18 years with unilateral benign PHEO of any size who underwent transperitoneal laparoscopic adrenalectomy at our hospitals between September 2016 and September 2023. Patients were divided into LRA (N = 122) and LLA (N = 149) groups. Univariate and multivariate logistic regression analyses were used to predict intraoperative HDI. In multivariate analysis for the prediction of HDI, right-sided PHEO, PHEO size, preoperative comorbidities, and preoperative systolic blood pressure were included. RESULTS: Intraoperative HDI was significantly higher in the LRA group than in the LLA (27% vs. 9.4%, p < 0.001). In the multivariate regression analysis, right-sided tumours showed a higher risk of intraoperative HDI (odds ratio [OR] 5.625, 95% confidence interval [CI], 1.147-27.577, p = 0.033). The tumor size (OR 11.019, 95% CI 3.996-30.38, p < 0.001), presence of preoperative comorbidities [diabetes mellitus, hypertension, and coronary heart disease] (OR 7.918, 95% CI 1.323-47.412, p = 0.023), and preoperative systolic blood pressure (OR 1.265, 95% CI 1.07-1.495, p = 0.006) were associated with a higher risk of HDI in both LRA and LLA, with no superiority of one side over the other. CONCLUSION: LRA was associated with a significantly higher intraoperative HDI than LLA. Right-sided PHEO was a risk factor for intraoperative HDI.

Long-term survival following adrenalectomy for secondary adrenal tumors: A systematic review and meta-analysis.

BACKGROUND: Secondary adrenal tumors (SATs) are uncommon, and the benefits of adrenalectomy for SATs have not been well-established. A systematic review and meta-analysis were conducted to assess the survival benefits of adrenalectomy for SATs. METHOD: ology: A systematic literature search was performed (1990-2022). The inclusion criteria included a known primary tumor with confirmed adrenal metastasis in patients who underwent adrenalectomy. The primary outcome was the overall survival (OS). RESULTS: A total of 26 studies were included, with 2279 patients. The average age at the time of diagnosis was 61.1 years. Lung cancer was the most common primary tumor. The average time from primary tumor diagnosis to identification of adrenal metastasis was 17 months. The median OS was 35.2 months. One, three, and five-year OS were 79.7 â€‹%, 49.1 â€‹%, and 37.9 â€‹%, respectively. CONCLUSION: The results of this review provide insight into the long-term survival of patients with SATs who underwent adrenalectomy. The study highlights the need for further research to identify the risk factors that play a role in the outcome of adrenalectomy in patients with SATs.

Endothelial cyst of the adrenal gland: A rare case report.

Adrenal gland cysts are rare and uncommon manifestations. Mostly asymptomatic, discovered incidentally during radiological studies or at autopsy, or without characteristic symptoms. The spectrum of these entities may include benign cysts or malignant cystic neoplasms. They are classified into four types: pseudocysts, endothelial cysts, epithelial cysts, and parasitic cysts. Though pseudocysts are reported to be the most frequently clinically recognized adrenal cysts in surgical series, endothelial cysts are more frequent in autopsy series. Even with advanced imaging modalities, it is still difficult to differentiate a benign adrenal neoplasm from a malignant one. As a result, getting a definitive diagnosis and starting treatment is challenging. In both symptomatic and asymptomatic cases with a large diameter or increasing sizes during follow-up, or with any abnormality of adrenal hormones, surgery is the treatment of choice in symptomatic. Herein, we present a 47-year-old female with a nonfunctional left adrenal endothelial cyst, who was incidentally found during a computerized tomography scan. The patient presented with left-sided nephritic colic due to renal lithiasis. The objective of this paper is to recall the clinical characteristics and to specify the diagnostic contribution of imaging as well as the therapeutic modalities of this entity.

A Rare Case of Adrenal Carcinoma With Isolated Hypercortisolism Mimicking Hyperaldosteronism.

We report a case of a 22-year-old female with pedal edema, hypokalemia, and hypertension. On suspicion of hyperaldosteronism, further workup was pursued, which only revealed a low serum adrenocorticotropic hormone (ACTH) and an inappropriately normal cortisol level after a 1-mg dexamethasone suppression test, suggestive of primary hypercortisolism. CT of the chest, abdomen, and pelvis revealed a left adrenal mass. Based on the clinical findings and biochemical abnormalities, we were expecting this tumor to be aldosterone-secreting, but both serum aldosterone and renin levels were normal in our patient. Eventual surgical resection confirmed initial suspicions of malignancy, as it was found to be adrenal cortical carcinoma. This case highlights the unusual presentation of this rare but aggressive endocrinologic neoplasm and the importance of its prompt diagnosis and treatment.

Solitary fibrous tumor of the adrenal gland: a case report and review of the literature.

Solitary fibrous tumor (SFT) is a rare mesenchymal tumor, probably of fibroblastic origin, mainly in the extremities and pleura. Primary SFT of the adrenal gland is clinically more rare. Here, we report the case of a 47-year-old woman who detected a left adrenal mass on physical examination, without any symptoms, and no laboratory abnormalities. A computed tomography (CT) examination of the adrenal gland suggested a round-like soft tissue density shadow in the left adrenal area. An unenhanced scan showed uneven density of the mass, with a scattered circular-like cystic low-density shadow inside, and an enhanced scan showed obvious uneven enhancement. We considered it to be adrenal pheochromocytoma. Ultimately, the patient was treated with laparoscopic left adrenalectomy. A pathological examination suggested an adrenal SFT. We reviewed previous case reports of adrenal SFTs and summarized the clinical characteristics of adrenal SFT combined with the relevant literature. For adrenal tumors with uneven low-density shadow and uneven CT enhancement features, we should consider the differential diagnosis of adrenal SFT.

The role of the adrenalectomy in the management of pheochromocytoma: the experience of a Portuguese referral center.

PURPOSE: Pheochromocytoma is a rare neuroendocrine tumor. Despite the low incidence, these tumors are of indisputable importance. This study aimed to analyze the management of pheochromocytoma in a referral center, with an emphasis on the minimally invasive adrenalectomy, which is the preferred therapeutic approach. METHODS: A retrospective analysis was performed on a cohort of patients diagnosed with pheochromocytoma who underwent adrenalectomy between January 2013 and December 2022. Clinical data including demographics, timelines, symptomatology, comorbidities, biochemical markers, genetic testing, surgical details, and follow-up outcomes, were collected and analyzed. RESULTS: The cohort included 44 patients, predominantly women (52.27%), with a median age of 53.39 years (range 13-83). Most of patients exhibited paroxysmal symptoms suggesting catecholamine excess. Documented hypertension was the most frequent (86.36%), along with glucose anomalies (40.01%) and anxiety disorder (31.82%). Genetic testing was performed in 36 (81.81%) patients and 14 (38.88%) revealed a positive result, predominantly RET pathogenic variant. Laparoscopic surgery was performed in 34 (79.07%) patients, showing significantly shorter operative time (2.5 h vs. 4.25 h, t-test p < 0,001) and fewer complications (23.53% vs 77.78%, p = 0.008). Postoperative complications occurred in 36.36% of the patients, mostly mild (grade I, 56.25%), with no mortality. SDHB pathogenic variant correlated with both recurrent and metastatic disease (p = 0.006). One-year follow-up reported 9.09% recurrence and 6.82% metastasis. CONCLUSIONS: Adrenalectomy demonstrated a high safety and effectiveness. This study exhibited a higher rate of genetic testing referral than other studies. Despite past advances, there is still a need for further studies to establish protocols and evaluate new techniques.

Evaluation of Adrenal Incidentaloma.

Adrenal incidentalomas have increased due to advanced and more sensitive imaging modalities, their increased utilization, and the aging population. Most adrenal incidentalomas are nonfunctional and benign, but a subset of patients has functional and/or malignant tumors. The evaluation of patients with an adrenal incidentaloma involves addressing 2 clinical questions: (1) Is the tumor functional? (2) Is the tumor malignant? A careful history and physical examination focused on signs and symptoms of adrenal functional tumors, biochemical testing, and imaging features are the cornerstone in the evaluation of patients with an adrenal incidentaloma.

Giant Cystic Pheochromocytoma Associated With Neurofibromatosis Type 1: A Case Report.

Pheochromocytomas are tumors that develop from the chromaffin cells of the adrenal medulla. More than 40% of cases of pheochromocytomas are associated with genetic conditions such as neurofibromatosis type 1 (NF1) or von Hippel-Lindau syndrome. Cystic pheochromocytomas are rare, generally asymptomatic, and thus of bigger size at the time of diagnosis. Surgical treatment is necessary to prevent cardiovascular morbidity and malignancy risk. We report the case of a 27-year-old patient admitted for further examination of a left adrenal mass that was discovered by an abdominal CT scan in the context of abdominal pain associated with hypertension evolving for three years. The clinical examination showed the presence of multiple café au lait spots, axillary and inguinal freckling with two dermal neurofibromas diagnosed clinically, as well as Lisch nodules on bilateral ophthalmic examination, thus meeting the clinical criteria for the diagnosis of NF1. The clinical laboratory investigation showed elevated urinary metanephrine and normetanephrine levels. CT scan examination showed a 10 cm left adrenal cystic mass on abdominal CT. This mass uptake of the radioligand in metaiodobenzylguanidine (MIBG) scintigraphy without secondary extra-adrenal localization allowed the diagnosis of a seemingly benign cystic pheochromocytoma to be made. The patient was put on presurgical drug preparation with volume expansion and then underwent left unilateral adrenalectomy. The histopathological study was in favor of a rather aggressive cystic pheochromocytoma with a pheochromocytoma of the adrenal gland scaled (PASS) score of 9. Blood pressure and urine catecholamines at seven days, three months, six months, and one year after surgery were normalized. Cystic pheochromocytoma is a rare tumor with a potentially poor prognosis. It is characterized by a more insidious evolution and a larger volume at diagnosis. It should be considered a diagnosis in patients with a cystic adrenal mass or an extra-adrenal mass with fluctuating blood pressure during surgery. This case illustrates the importance of both presurgical preparation and screening for pheochromocytoma in neurofibromatosis type 1.

Current Management Strategies and Outcomes in Children With Adrenocortical Carcinoma

INTRODUCTION: Adrenocortical carcinoma (ACC) is a rare but aggressive pediatric endocrine tumor. However, there is no recent US national report on the management or outcomes of pediatric ACC. We aimed to examine the clinical characteristics, current management strategies, and outcomes of pediatric ACC. METHODS: In this retrospective National Cancer Database study between 2004 and 2019, children (<18 y) with ACC were included. Overall survival was examined by means of Kaplan-Meier method, log-rank tests, and Cox regression modeling. RESULTS: Seventy-eight children with ACC were included. The median age was 10 y, the median tumor size was 10.2 cm, and 35.9% had metastasis at diagnosis. Most patients underwent surgical treatment (84.6%), 56.4% received chemotherapy, and 7.7% received radiation. The 1-, 3-, and 5-y overall survival rates were 87.0%, 62.0%, and 60.1%, respectively. In unadjusted analysis, surgical treatment was associated with improved overall survival (log-rank test, P < 0.001). In multivariable Cox regression, metastasis at diagnosis was associated with inferior overall survival (hazard ratio: 2.72, 95% confidence interval: 1.15-6.40, P = 0.02), when adjusting for age, tumor size, receipt of surgical treatment, and chemotherapy. In patients with nonmetastatic ACC, increasing age was associated with inferior overall survival (hazard ratio: 1.12, 95% confidence interval: 1.00-1.24, P = 0.04), when adjusting for tumor size, receipt of surgical treatment, and chemotherapy. CONCLUSIONS: Most children with ACC in the USA undergo surgical treatment with about half of these also receiving chemotherapy. Metastasis at diagnosis was independently associated with inferior overall survival; in patients with nonmetastatic ACC, increasing age was independently associated with inferior overall survival.

Cystic adrenal mass revealing a pheochromocytoma in the setting of multiple endocrine neoplasia: A case report.

Pheochromocytoma, a neuroendocrine tumor, represents a rare medical condition characterized by the excessive secretion of catecholamines. These tumors often exhibit distinctive features on imaging studies, notably appearing hypervascular. Furthermore, they may present as cystic masses with thin walls, a characteristic that becomes more evident following the administration of contrast medium. The cystic form of adrenal pheochromocytoma, as exemplified in our case, is particularly uncommon, thus underscoring the importance of recognizing its atypical presentation. Accurate diagnosis hinges on a thorough understanding of both the clinical manifestations and radiological findings suggestive of pheochromocytoma. However, definitive confirmation typically necessitates histological examination of the surgical specimen post-adrenalectomy. By shedding light on this rare variant, our case emphasizes the critical role of comprehensive diagnostic approaches in managing such complex medical conditions. Additionally, it underscores the significance of multidisciplinary collaboration among clinicians, radiologists, and pathologists to ensure timely and accurate diagnosis, ultimately guiding appropriate treatment strategies and optimizing patient outcomes.

Efficacy and safety of radiofrequency ablation and laparoscopic adrenalectomy for primary aldosteronism: a meta­analysis.

OBJECTIVE: To compare the efficacy (including blood pressure, medication reduction, serum potassium, and clinical success) and safety parameters (including operative time, length of hospital stay, blood loss, hypertension crisis rate, and complication rate) of radiofrequency ablation (RFA) and laparoscopic adrenalectomy (LA) in the treatment of primary aldosteronism (PA). METHODS: Literature search was performed on PubMed, EMBASE, The Cochrane Library (Issue 8, 2023), Web of Science, China National Knowledge Infrastructure, and Wanfang from inception to August 2023. Study selection, data extraction, and risk of bias assessment were performed by two independent reviewers. Quality assessment was conducted using the Newcastle-Ottawa scale. The Stata 12.0 software was used for statistical analyses. Pooled odds ratios (OR) with corresponding 95% confidence interval (CI) were calculated for categorical outcomes, while mean difference (MD) with corresponding 95% CI were calculated for continuous outcomes. RESULTS: A total of 5 studies involving 204 patients (LA, n = 127; and RAF, n = 77) were included. LA had better diastolic blood pressure control than RFA (WMD = 5.19; 95% CI 0.96-9.43); however, the RFA demonstrated better shorter operative time (WMD = - 57.99; 95% CI - 116.54 to 0.57), and shorter length of hospital stay (OR - 1.6; 95% CI - 2.37 to - 0.83) compared to LA. All remaining parameters were comparable between the interventions. CONCLUSION: While grossly comparable in efficacy as treatment options for PA, RFA may allow for shorter operative time and hospital stay, less intraoperative blood loss, and lower hospitalization costs. However, LA has better diastolic blood pressure control. Even so, we still need larger prospective studies, specifically with comparative hypertension response (short and long term) and number of post-procedural antihypertensive medication requirement.

The level of accordance between preoperative and postoperative diagnosis in patients undergoing adrenalectomy - a study of 230 consecutive cases.

Introduction: Patients are qualified for an adrenalectomy due to endocrine or oncologic reasons. Final histopathological diagnoses include a wide spectrum of more than a dozen entities. The aim of this study was to compare preoperative and postoperative diagnoses of patients undergoing adrenalectomy to determine the level of diagnostic accuracy, as well as sex and age of patients. Material and methods: A group of 214 patients (230 specimens in total) operated on in a single center was studied and their demographic and pathological data were investigated. Results: The majority of diagnoses were characterized by both high positive predictive value and sensitivity, excluding pheochromocytoma (60.0% and 67.7%, respectively) and adrenal cyst (100% and 37.5%, respectively). Patients operated on due to Cushing's syndrome were statistically significantly more often females (p = 0.009), while those with metastases (diagnosed both pre- and postoperatively) were more often males (both p = 0.001). Patients qualified due to non-functioning tumors were older than those with Cushing's or Conn's syndrome (p = 0.044 and p = 0.002, respectively). Conclusions: The lowest diagnostic accuracy is observed in cases of pheochromocytoma and adrenal cyst. Meticulous preparation of the patient for hormonal tests, including discontinuation of certain medications, is essential for obtaining accurate results. The diagnosis of Cushing's syndrome is more prevalent in females, while metastasis syndrome is more prevalent in males. Adrenocortical carcinoma may initially be diagnosed as a non-functioning tumor (1.6% of such cases) or a recurrence of a previously resected tumor, which should always raise a suspicion of a malignant neoplasm.

Safety and surgical outcomes of robotic adrenalectomy from a 15-year experience at a single institution.

Robotic adrenalectomy (RA) has gained significant popularity in the management of adrenal gland diseases. We report our experience at a single tertiary institution and evaluate the safety and surgical outcomes of RA. The data of 122 consecutive patients who underwent RA from October 2009 to December 2022 at Korea University Anam Hospital (Seoul, Korea) were reviewed. There were no perioperative complications. Clinicopathological features and surgical outcomes were retrospectively analyzed through complete chart reviews. Noteworthy findings include the influence of sex, tumor size, and body mass index on operation time, with the female and small tumor groups exhibiting shorter operation times (P = 0.018 and P = 0.009, respectively). Pheochromocytoma was identified as a significant independent risk factor for a longer operation time in the multivariate analysis [odds ratio (OR), 3.709; 95% confidence interval (CI), 1.127-12.205; P = 0.031]. A temporal analysis revealed a decreasing trend in mean operation times across consecutive groups, reflecting a learning curve associated with RA adoption. RA is a safe and effective operative technique alternative to laparoscopic adrenalectomy that has favorable surgical outcomes and enhances the convenience of the operation.

Comparison of adrenalectomy with conservative treatment on mild autonomous cortisol secretion: a systematic review and meta-analysis.

Objective: This systematic review and meta-analysis was conducted to compare the benefits of adrenalectomy and conservative treatment for comorbidities associated with mild autonomous cortisol secretion (MACS) in patients diagnosed with MACS. Background: MACS is the most common benign hormone-secreting functional adrenal incidentaloma. Overproduction of cortisol is observed in MACS patients, resulting in a variety of long-term health issues, including arterial hypertension (HTN), diabetes mellitus (DM), dyslipidemia, obesity, and osteoporosis; however, the classic clinical manifestations of Cushing's syndrome (CS) are not present. Methods: A systematic search was conducted using MEDLINE, Embase, Web of Sciences, and Scopus databases on December, 2023. Two reviewers independently extracted data and assessed the quality of the included articles. A meta-analysis was performed to compare the beneficial effects of adrenalectomy versus conservative management for MACS-related comorbidities. Results: Fifteen articles were included in this study, which evaluated 933 MACS patients (384 Adrenalectomy and 501 Conservative treatment, and 48 excluded due to incomplete follow-up duration). MACS diagnosis criteria were different among the included articles. All studies, however, stated that there must be no overt CS symptoms. Meta-analysis demonstrates the overall advantage of adrenalectomy over conservative treatment for MACS-related comorbidities (Cohen's d = -0.49, 95% CI [-0.64, -0.34], p = 0.00). Subgroup analysis indicated that the systolic blood pressure (pooled effect size = -0.81, 95% CI [-1.19, -0.42], p = 0.03), diastolic blood pressure (pooled effect size = -0.63, 95% CI [-1.05, -0.21], p = 0.01), and BMD (pooled effect size = -0.40, 95% CI [-0.73, -0.07], p = 0.02) were significantly in favor of adrenalectomy group rather than conservative treatment but no significant differences between the two treatment groups in other MACS-related comorbidities were reported. Conclusion: Despite the limited and diverse data, this study demonstrates the advantage of adrenalectomy over conservative treatment for MACS-related comorbidities.

Biochemical Control in Cushing's syndrome: Outcomes of the treatment in a large single center cohort.

BACKGROUND: Normalization of hypercortisolism is essential to reduce morbidity and mortality in patients with Cushing's syndrome (CS). The aim of this analysis was to assess biochemical control rates in patients with Cushing's disease (CD), ectopic Cushing's syndrome (ECS) and adrenal Cushing's syndrome (ACS). METHODS: Patients with confirmed CS (n= 296) treated in a single tertiary care center were retrospectively analysed (185 CD, 27 ECS, 84 uni- and bilateral ACS). RESULTS: Firstline treatment led to biochemical control in 82% of the patients. Time to biochemical control (median, IQR) was longer in CD (11.0 weeks, 5.6-29.8; p< 0.05) than in ACS (7.7 weeks, 4.1-17.1) and ECS (5.6 weeks, 4.1-23.3). Disease persistence or recurrence after first-line therapy was observed more often in CD (24% and 18%; p< 0.05) than in ECS (15% and 15%) and ACS (6% and 4%). Total time in hypercortisolism since diagnosis was significantly shorter in patients with CD diagnosed since 2013, after specialized patient care was implemented, compared to patients diagnosed before 2013 (13.5 weeks, vs. 26.1 weeks; p< 0.0070). Control of hypercortisolism at last follow up (76 months, 38-163) was achieved in 94% of patients with ACS, 100% of patients with ECS and 92% of patients with CD. CONCLUSIONS: Biochemical control can be achieved in most patients with different subtypes of CS within a reasonable time frame. Control of hypercortisolism has improved over time.

Abiraterone in Classic Congenital Adrenal Hyperplasia: Results of Medical Therapy Before Adrenalectomy.

We present the case of a 20-year-old woman with classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency, with uncontrolled hyperandrogenemia despite supraphysiological glucocorticoid therapy. We used abiraterone acetate, an inhibitor of the 17-hydroxylase/17,20-lyase enzyme, to suppress adrenal androgen synthesis and allow physiological glucocorticoid and mineralocorticoid therapy, as a proof-of-concept, before proceeding to bilateral adrenalectomy. We report the patient's clinical course, the changes in adrenal steroids, and the immunohistochemistry of the adrenals.