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Introduction: Objective of this study was to assess the diagnostic efficacy of the most commonly used radiological evaluation in form of contrast enhanced computed tomography of abdomen with adrenal protocol, basic functional evaluation and surgical outcomes of primary adrenal masses., Material and Methods: We have retrospectively analysed the institutional records of 108, patients admitted from August 2017 to September 2023, who had underwent surgical intervention for their adrenal mass after thorough evaluation and stabilization., Results: Flank pain was the most common symptoms in 44 (40.74%) patients. Non-functional adrenal adenoma was found in 36 (33.33%) patients. Pheochromocytoma was the most the common functional adrenal mass found in 24 (22.22%) patients. CECT abdomen had suggested features of malignancy in 16(14.81%) patients. In final histopathological report 26(24.07%) patients had pheochromocytoma and 12 (11.11%) patients had adrenocortical carcinoma. CECT abdomen had sensitivity of 75%, specificity of 95.6%. Plasma free metanephrines and normetanephrine analysis had shown sensitivity of 90%, specificity of 92.86%, aldosterone to renin ratio had sensitivity of 92%, specificity of 100%. Sensitivity and specificity of the low-dose dexamethasone suppression test and plasma dehydroepiandrosterone was 100% in our study. Eighty patients (74.07%) were operated with laparoscopic adrenalectomy, 20 (18.52%) patients with open adrenalectomy. Eight patients (7.41%) were converted from laparoscopic to open. Laparoscopic approach had significantly lesser mean operating time, lesser blood loss, lesser hospitalization and lesser post-operative complications., Conclusion: Radiological analysis and functional analysis has shown good sensitivity and high specificity. Laparoscopic approach has advantage of lesser operative time, lesser hospitalization and lesser post-operative complications., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship and/or publication of this article.

Objective: This study describes the effects of introducing a protocol omitting preoperative α-blockade dose-escalation (de-escalation) in a prospective patient group., Background: The decline of mortality and morbidity associated with pheochromocytoma resection is frequently attributed to the introduction of preoperative α-blockade. Current protocols require preoperative α-blockade dose-escalation and multiple-day hospital admissions. However, correlating evidence is lacking. Moreover, recent data suggest equal perioperative safety regardless of preoperative α-blockade escalation., Methods: Single-institution evaluation of protocol implementation, including patients who underwent adrenalectomy for pheochromocytoma between 2015 and 2023. Intraoperative hemodynamic control was regulated by active adjustment of blood pressure using vasoactive agents. The primary outcome was intraoperative hypertension, defined as the time-weighted average of systolic blood pressure (TWA-SBP) above 200 mm Hg. Secondary outcomes included perioperative hypotension, postoperative blood pressure support requirement, hospital stay duration, and complications., Results: Of 102 pheochromocytoma patients, 82 were included; 44 in the de-escalated preoperative α-adrenergic protocol and 38 following the previous dose-escalation protocol. Median [IQR] TWA-SBP above 200 mm Hg was 0.01 [0.0-0.4] mm Hg in the de-escalated group versus 0.0 [0.0-0.1] mm Hg in the dose-escalated group ( P =0.073). The median duration of postoperative continuous norepinephrine administration was 0.3 hours [0.0-5.5] versus 5.1 hours [0.0-14.3], respectively ( P =0.003). Postoperative symptomatic hypotension occurred in 34.2% versus 9.1% of patients ( P =0.005). Median hospital stay was 2.5 days [1.9-3.6] versus 7.1 days [6.0-11.9] ( P <0.001). No significant differences in complication rates were observed., Conclusion: Our data suggest that adrenalectomy for pheochromocytoma employing a de-escalated preoperative α-blockade protocol is safe and results in a shorter hospital stay., Competing Interests: The authors report no conflicts of interest., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Surgical treatment of pheochromocytomas is associated with circulatory dynamics instability, necessitating systemic management of the patient in the intensive care unit after surgery. Early mobilization after pheochromocytoma surgery is not described in the guidelines, and to our knowledge, no reports have specifically focused on circulatory dynamics during early mobilization after surgery., Case Presentation: A 31-year-old Japanese woman was diagnosed with bilateral pheochromocytoma and underwent a second-stage adrenalectomy for bilateral pheochromocytoma at our hospital. We evaluated circulatory dynamics during a passive upright posture on postoperative days 1 and 4. She was placed on a tilt bed in the supine position (0° head) for 15 min, passive upright posture (60°) for 15 min, and supine position again for 10 min. In the passive upright posture, both systolic and diastolic blood pressure decreased, and the heart rate increased by 10 bpm over approximately 10 minutes. After the postural change from the passive upright posture to the supine position, the heart rate decreased to a level lower than in the supine prior to the passive upright posture. The same measurements were taken on postoperative day 4. Systolic blood pressure decreased in the passive upright posture, but diastolic blood pressure remained unchanged. The heart rate also increased earlier after passive upright posture compared with that on the day after surgery. When the patient returned to the supine position from the passive upright posture, the heart rate decreased even further compared with its level prior to the passive upright posture, as observed on the day after surgery., Conclusion: Circulatory dynamics may fluctuate after surgery for pheochromocytoma due to a relative decrease in epinephrine and norepinephrine secretion. In this case, the patient's response may be close to normal by the fourth postoperative day, but circulatory dynamics fluctuations were noted on both the first and fourth postoperative days when the patient was supine after passive upright posture. Although the substantial variability of perioperative circulatory dynamics in patients with pheochromocytoma has improved considerably with the development of perioperative management guidelines, circulatory dynamics should be carefully monitored during early mobilization and after its completion., (© 2024. The Author(s).)

This study aimed to compare the safety and clinical efficacy of Da Vinci robotic surgery (control group) versus traditional laparoscopic surgery in the treatment of large (tumor size >6 cm) pheochromocytomas/paragangliomas. The evaluation was based on intraoperative metrics such as operative time, blood loss, conversion to open surgery rates, postoperative drain duration, and length of postoperative hospital stay. A retrospective analysis was conducted on 29 patients (14 males and 15 females) who underwent Da Vinci robotic surgery for large (greater than 6 cm) pheochromocytomas/paragangliomas from October 2019 to September 2023. The average maximum tumor diameter was (7.2±1.6) cm. During the same period, 32 patients underwent laparoscopic resection of challenging pheochromocytomas/paragangliomas.Data showed no significant differences between the two groups, except for the norepinephrine levels. Operative time, blood loss, conversion rate, postoperative drainage duration, and length of hospital stay were recorded and compared between the two groups. The differences between the groups were analyzed using the t -test, and the conversion rates were compared using the chi-square (χ²) test. All surgeries were successfully performed. One patient underwent a one-stage bilateral tumor resection. In the Da Vinci robotic surgery group, the average operative time was (76±32) minutes, compared to (106±45) minutes in the control group ( P =0.003). The average intraoperative blood loss was (95±75) ml, compared to (160±90) ml in the control group ( P =0.019). The conversion rate to open surgery was 2 cases (6.9%) in the robotic group compared to 5 cases (15.6%) in the control group ( P =0.031). The average postoperative drainage duration was (3.0±1.0) days, compared to (3.5±1.5) days in the control group ( P =0.128). The average length of hospital stay was (4.5±1.2) days, compared to (4.7±2.2) days in the control group ( P =0.657). Postoperative pathological diagnosis confirmed pheochromocytoma or paraganglioma. Blood pressure normalized within 1 to 3 months postoperatively, with an average systolic blood pressure of (116±13) mmHg(1 mmHg=0.133 kPa）and diastolic blood pressure of (73±5) mmHg in the robotic surgery group. Follow-up for 3 to 40 months showed normal levels of blood catecholamines and no recurrence or metastasis on follow-up imaging studies, including chest, abdominal, and pelvic CT scans. The average levels of 3-methoxy-norepinephrine, 3-methoxy-epinephrine, and 3-methoxy-tyramine were (0.42±0.21) nmol/L (normal value≤1.05 nmol/L), (0.11±0.07) nmol/L (normal value≤0.32 nmol/L), and (0.017±0.006) nmol/L (normal value≤0.036 nmol/L), respectively. This study demonstrates that for the treatment of large pheochromocytomas/paragangliomas, the Da Vinci robotic technique offers advantages such as high-definition visualization, precise operation, and flexibility. These advantages enable the safe and efficient completion of surgeries, as evidenced by shorter operative times, less blood loss, and lower conversion rates.

Pheochromocytoma is a rare catecholamine-secreting mass which can lead to life-threatening cardiovascular complications and should be resected if confirmed. A 21- year-old male without any history of hypertension presented with congestive heart failure (HF) caused by pheochromocytoma-related cardiomyopathy; he was discovered to have hypertension at follow-up. A welldefined and uniform density mass was observed in his right adrenal region and confirmed to be pheochromocytoma. When the dilated left ventricle had become absolutely normal in size and hypertension was well controlled by medications, the patient needed no antihypertensive therapy at all after laparoscopic adrenalectomy at follow-up. Pheochromocytoma should be considered if HF occurred in patients, including young patients, with left ventricular enlargement and unknown medical history who initially presented with normal blood pressure. Even if the dilated left ventricle was restored and hypertension confirmed at follow-up, it could be well controlled by medications.

Objective: To evaluate the value of preoperative 3D planning and intraoperative navigation in robot-assisted laparoscopic surgery for complex pheochromocytoma., Study Design: Observational study. Place and Duration of the Study: Department of Urology, The Affiliated Hospital of Hebei University, from July 2022 to July 2023., Methodology: Sixty patients with complex pheochromocytoma were divided into the study group (n = 30) and the control group (n = 30) according to the treatment method. The study group was treated with robot-assisted laparoscopic surgery, with preoperative 3D printing-based planning, and intraoperative 3D navigation, and the control group underwent conventional retroperitoneal robot-assisted laparoscopic surgery. Surgical-related indicators and intraoperative and postoperative complication rates between the two groups were compared., Results: The operation time, postoperative hospital stay, and drainage tube retention time in the study group were significantly shorter than in the control group (p <0.001), and the intraoperative blood loss in the study group was significantly less than in the control group (p <0.001). The study group exhibited a significantly lower incidence of intraoperative haemodynamic instability (HI), use of vasoactive drugs, and postoperative ICU transfer rate than the control group (p <0.05). The incidence of surgical complications in the study group was significantly lower than in the control group (p = 0.04). The non-recurrence rate in the study and control groups after 1-year follow-up was 96.7% and 90%, respectively (χ2 = 2.77, p = 0.10)., Conclusion: Robot-assisted surgery, performed through renal surface, has the advantages of less bleeding, shorter surgical time, faster recovery, fewer complications related to chromaffin cell inflammation and postoperative complications, and is safe and effective in treating complex pheochromocytoma., Key Words: 3D planning and navigation, Robot-assisted surgery, Complex pheochromocytoma.

Context: Adrenal incidentalomas (AIs) are relatively uncommon neoplasms in 2% of apparently healthy individuals requiring evaluation for functionality and malignancy., Aim: We aimed to study the clinical, biochemical, and radiological profiles of patients presenting with AI and histopathological outcomes of those undergoing adrenalectomy. Materials and Methods: This retrospective study enrolled 62 AI patients attending a tertiary care center in South India between January 2016 and October 2023. Demographic details, radiological features, functionality, and histopathological data were analyzed., Results: Out of 62 patients, total masses evaluated were 65 indicating bilaterality in 3 patients. The female: male ratio was 1.69, with a median age of 55 years (interquartile range: 44-64 years). 45.1% of individuals were >60 years. The most common indication for imaging was pain abdomen in 43 (69.4%). The median size was 3.2 cm. Fifty-five (88.7%) were assessed for functionality and 27 (49.1%) were functional. Among the 62 individuals, 14 (20.2%) had hypercortisolism, 11 (15.9%) had pheochromocytoma, 5 (7.24%) had primary hyperaldosteronism (PA), and 4 (5.7%) had hyperandrogenism including plurihormonal in 7. A mass size of 3.2 cm was of great value in distinguishing functional tumors with a sensitivity of 72% and specificity of 66% with an area under the curve of 0.682. A total of 34 (54.8%) patients underwent adrenalectomy. On histopathological examination, Adenoma (44.1%) was the most common followed pheochromocytoma (26.5%), adrenal cysts (8.8%), and Myelolipoma (5.9%). Two (5.9%) incidentalomas were adrenocortical carcinoma (ACC). Eight (53.3%) adenomas were functional with 6 having hypercortisolism (including 1 with hyperandrogenism) and 2 with PA., Conclusion: In our experience, the incidence of pheochromocytoma was second most common after adenoma. Since most functional tumors (60%) and all ACCs were ≥4 cm, a thorough biochemical evaluation for hormonal excess and evaluation for malignancy followed by surgery should be considered for lesions, especially ≥4 cm. Thus, we report the baseline demographic and clinical characteristics of patients with AI from a single center in South India., (Copyright © 2024 Copyright: © 2024 Annals of African Medicine.)

Background: Surgical resection for pheochromocytoma (PCC) is still challenging. This study assessed the perioperative outcomes of adrenalectomy for PCC and investigated the risk factors for intraoperative hemodynamic instability (HI)., Methods: This retrospective study included 571 patients with adrenal tumors who underwent adrenalectomy at Kobe University Hospital and other related hospitals between April 2008 and October 2023. The perioperative outcomes of laparoscopic adrenalectomy were compared between PCC (n = 92) and non-PCC (n = 464) groups. In addition, we investigated several potential risk factors for intraoperative HI in patients with PCC (n = 107; open, n = 11; laparoscopic, n = 92; robot-assisted, n = 4)., Results: While patients with PCC had a significantly larger amount of blood loss in comparison to those with non-PCC (mean, 70 and 30 mL, respectively; p = 0.004), no significant difference was observed in the rate of perioperative grade ≥III complications (1.1% vs. 0.6%; p = 0.516), and no perioperative mortality was observed in either group. A tumor size of ≥40 mm, with preoperative hypertension and urinary metanephrines at a level ≥3 times the upper limit of the normal value, were found to be significant predictors of HI, with odds ratios of 2.74 (p = 0.025), 3.91 (p = 0.005), and 3.83 (p = 0.004), respectively., Conclusions: Our data suggest that laparoscopic adrenalectomy for PCC may be as safe as that for other types of adrenal tumors and that large tumors and hormonally active disease may be risk factors for intraoperative HI. The optimal perioperative management for PCC with these risk factors should be established., (© 2024 The Japanese Urological Association.)

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., (© 2024. The Author(s).)

Approximately 40% of pheochromocytoma and paraganglioma (PPGL) cases are familial, typically presenting earlier with more complex symptoms. This paper synthesizes literature and guidelines to inform on clinical characteristics and perioperative care for PPGL. Pheochromocytoma in von Hippel-Lindau (VHL) disease exhibits heightened secretion activity without significant perioperative hemodynamic changes. Tumors in multiple endocrine neoplasia type 2 (MEN2) have a stronger endocrine function, which may induce hemodynamic fluctuations during surgery. Therefore, pheochromocytoma screening is essential at all stages of MEN2. Neurofibromatosis type 1 (NF1) often presents multisystem lesions and can result in difficult airway. Pheochromocytoma should be evaluated when NF1 patients present hypertension. Pheochromocytoma and paraganglioma type 5 may present multiple lesions of pheochromocytoma or paraganglioma. In summary, hereditary PPGLs may present with severe lesions in other systems, beyond tumor function. A multi-disciplinary team (MDT) approach is often invaluable in perioperative management.

Pheochromocytomas are rare neuroendocrine tumors that derive from the chromaffin cells of the adrenal medulla and secrete catecholamines. The measurement of plasma or fractionated urine metanephrines is the hormonal determination of choice for the biochemical diagnosis. Once the biochemical diagnosis is confirmed, the next step is the localization study. It is recommended to request a genetic study in all patients with pheochromocytomas since 40% of cases are hereditary. Once the diagnostic study is completed, preoperative treatment with alpha blockers should be instituted at least 7-14 days before adrenalectomy. However, in low-risk patients, the omission of presurgical treatment could be considered if the surgery is performed in centers with experience and a strict monitoring of the patient is carried out during the perioperative period. This document offers a practical guide on the diagnosis and perioperative approach in patients with pheochromocytomas., (Copyright © 2024 Elsevier España, S.L.U. All rights reserved.)

BACKGROUND Pheochromocytomas, rare tumors arising from the adrenal medulla, can present with highly variable symptoms; therefore, pheochromocytomas frequently remain undiagnosed, leaving the potential for physiological complications. Acutely, these complications include pheochromocytoma crisis, in which high levels of catecholamines are released and cause a life-threatening hypertensive emergency. Over time, undiagnosed pheochromocytomas can lead to cardiovascular damage and end-organ disease related to chronic exposure to elevated blood pressure. CASE REPORT We share a case of pheochromocytoma in a 45-year-old woman who presented with gastrointestinal symptoms of intractable nausea, vomiting, and abdominal pain. Imaging revealed an adrenal mass that had radiographic features that were most consistent with myelolipoma. Before exposing the patient to anesthesia and endoscopy for further diagnostic workup of her gastrointestinal symptoms, which can trigger a catecholamine surge in individuals with a pheochromocytoma, further biochemical testing was performed. Testing of plasma and urine confirmed pheochromocytoma, and surgical resection was performed for definitive treatment. Ultimately, the patient had resolution of her symptoms following the removal of the tumor. CONCLUSIONS The resolution of symptoms following surgical resection suggests that symptoms may have been related to the mass effect of the tumor or as an atypical manifestation of increased catecholamine levels. Additionally, by screening for pheochromocytoma, the patient was able to avoid potential complications that can result from common gastroenterological diagnostic procedures. This case report highlights the potential benefit for screening for pheochromocytoma when faced with symptoms that may be non-specific or related to mass effect upon surrounding organs.

Pheochromocytoma crisis is rare but potentially fatal if not recognized early and properly managed. Here, a woman in her 20s with a paraganglioma-induced pheochromocytoma crisis, who was successfully treated by veno-arterial extracorporeal membrane oxygenation (VA-ECMO) and interval tumor resection, is described. In July 2022, the patient was brought to hospital with a complaint of sudden-onset of palpitations with vomiting. The patient developed cardiorespiratory failure with hypoxia. Computed tomography scan showed pulmonary oedema and a mass anterior to the inferior vena cava. She was transferred to the intensive care unit and treated with VA-ECMO. The patient's ECMO was withdrawn after 6 days without any complications. After hemodynamic stabilization, the patient underwent tumor resection 4 months later. The postoperative course was uneventful and she was discharged on postoperative day 7. Histopathological analysis confirmed a paraganglioma. VA-ECMO may play a significant role in saving lives and providing time for accurate diagnosis and specific treatment of a patient with pheochromocytoma crisis. Appropriate individual management can help avoid the occurrence of ECMO complications., Competing Interests: Declaration of conflicting interestThe authors declare that there is no conflict of interest.

Background: Surgical resection is the primary management for pheochromocytoma. However, some patients with pheochromocytoma still suffer from hypertension after successful adrenalectomy. The risk factors for postoperative persistent hypertension remain unclear. Thus the aim of the present study was to identify the risk factors of postoperative persistent hypertension in patients with pheochromocytoma concomitant with hypertension., Methods: We retrospectively analyzed 259 patients with pheochromocytoma who underwent adrenalectomy in our hospital between January 1, 2007, and December 31, 2018. Patients' demographics, comorbidities, and perioperative data were recorded. The odds ratio (OR) and 95% confidence interval were measured through binary logistic regression. The cutoff values and the area under the curve (AUC) for continuous risk factors of persistent hypertension after adrenalectomy were calculated through receiver operating characteristic curve analysis., Results: Of the 259 patients, 40.9% (106/259) patients experienced postoperative persistent hypertension. Three independent risk factors of persistent hypertension in patients with pheochromocytoma after adrenalectomy were found to be older age (OR = 1.16, p = 0.037), longer duration of hypertension (OR = 3.10, p = 0.01), and concomitance with cardiovascular events (yes vs. no, OR = 17.17, p = 0.049). The cutoff value of age and duration of hypertension was 66 years (AUC = 0.741, p < 0.0001) and 27 months (AUC = 0.991, p < 0.0001), respectively., Conclusions: Collectively, older age, a longer duration of hypertension, and concomitance with cardiovascular events were independent risk factors of persistent hypertension in patients with pheochromocytoma after adrenalectomy. These findings may help in improving perioperative management and follow-up strategies., (© 2024 Wiley Periodicals LLC.)

Introduction: There is wide variability in the perioperative management of phaeochromocytoma and paraganglioma (PPGL) in different centres. This study aimed to summarise the management of PPGLs as reported in the United Kingdom Registry for Endocrine and Thyroid Surgery (UKRETS) database and to determine current perioperative management of PPGLs by surveying UK clinicians., Methods: Data recorded on UKRETS from 2005 to 2021 were subjected to descriptive analyses. British Association of Endocrine and Thyroid Surgeons members were invited to participate in an open survey relating to the perioperative management of patients with PPGLs., Results: A total of 2,007 operations for PPGL from 49 participating centres were included. The median annual workload in each centre was four cases. Operations were performed predominantly laparoscopically (69%). The median length of stay (4 days) was the same in groups of surgeons stratified by volume. The survey had 29 respondents from 22 centres across the UK, and a formal protocol for perioperative management exists in 48% of the centres. Phenoxybenzamine (72%) was preferred for alpha-blockade. The practice of admitting patients for optimisation from 1 to 7 days before the day of surgery was common (62%). Central venous pressure and blood glucose monitoring were mentioned as routine intraoperative adjuncts by 72% of the responders., Conclusions: There is significant variation in the workload and perioperative management of PPGLs in the UK. This is potentially detrimental to patient outcomes and a consensus document might be beneficial to harmonise practice across the UK.

Objective: Hereditary pheochromocytoma (hPCC) commonly develops bilaterally, causing adrenal insufficiency when standard treatment, radical adrenalectomy (RA), is performed. Partial adrenalectomy (PA) aims to preserve adrenal function, but with higher recurrence rates. This study compares outcomes of PA versus RA in hPCC., Methods: Patients with hPCC due to pathogenic variants in RET, VHL, NF1, MAX, and TMEM127 from 12 European centers (1974-2023) were studied retrospectively. Stratified analysis based on surgery type and initial presentation was conducted. The main outcomes included recurrence, adrenal insufficiency, metastasis, and mortality., Results: The study included 256 patients (223 RA, 33 PA). Ipsilateral recurrence rates were 9/223 (4%) after RA versus 5/33 (15%) after PA (P = 0.02). Metastasis and mortality did not differ between groups. Overall, 103 patients (40%) underwent bilateral adrenalectomy either synchronously or metachronously (75 RA, 28 PA). Of these, 46% developed adrenal insufficiency after PA.In total, 191 patients presented with initial unilateral disease, of whom 50 (26%) developed metachronous contralateral disease, most commonly in RET, VHL, and MAX. In patients with metachronous bilateral disease, adrenal insufficiency developed in 3/4 (75%) when PA was performed as the first operation followed by RA, compared to 1/7 (14%) when PA was performed as the second operation after prior RA (P = 0.09)., Conclusion: In patients with hPCC undergoing PA, local recurrence rates are higher than after RA, but metastasis and disease-specific mortality are similar. Therefore, PA seems a safe method to preserve adrenal function in patients with hPCC, in cases of both synchronous and metachronous bilateral disease, when performed as a second operation., Competing Interests: Conflict of interest: None., (© The Author(s) 2024. Published by Oxford University Press on behalf of European Society of Endocrinology.)

Adrenal gland-induced hypertension results from underlying adrenal gland disorders including Conn's syndrome, Cushing's syndrome, and Pheochromocytoma. These adrenal disorders are a risk for cardiovascular and renal morbidity and mortality. Clinically, treatment for adrenal gland-induced hypertension involves a pharmaceutical or surgical approach. The former presents very significant side effects whereas the latter can be ineffective in cases where the adrenal disorder reoccurs in the remaining contralateral adrenal gland. Due to the limitations of existing treatment methods, minimally invasive treatment options like microwave ablation (MWA) have received significant attention for treating adrenal gland disorders. A precise comprehension of the dielectric properties of human adrenal glands will help to tailor energy delivery for MWA therapy, thus offering the potential to optimise treatments and minimise damage to surrounding tissues. This study reports the ex vivo dielectric properties of human adrenal glands, including the cortex, medulla, capsule, and tumours, based on the data obtained from four patients (diagnosed with Conn's syndrome, Cushing's syndrome, and Pheochromocytoma) who underwent unilateral adrenalectomy at the University Hospital Galway, Ireland. An open-ended coaxial probe measurement technique was used to measure the dielectric properties for a frequency range of 0.5-8.5 GHz. The dielectric properties were fitted using a two-pole Debye model, and a weighted least squares method was employed to optimise the model parameters. Moreover, the dielectric properties of adrenal tissues and tumours were compared across frequencies commonly used in MWA, including 915 MHz, 2.45 GHz, and 5.8 GHz. The study found that the dielectric properties of adrenal tumours were influenced by the presence of lipid-rich adenomas, and the dielectric properties of Cushing's syndrome tumour were lowest in comparison to the tumours in patients diagnosed with Conn's syndrome and Pheochromocytoma. Furthermore, a notable difference was observed in the dielectric properties of the medulla and cortex among patients diagnosed with Conn's syndrome, Cushing's syndrome, and Pheochromocytoma. These findings have significant implications for the diagnosis and treatment of adrenal tumours, including the optimisation of MWA therapy for precise ablation of adrenal masses., (© 2024. The Author(s).)

Rationale: 3P association (3PA) is a rare condition with co-occurrence of pituitary adenoma and pheochromocytoma/paraganglioma. There have been less than a hundred documented cases of 3PA, which can be sporadic or related to genetic mutations. The present case report describes the first Iranian patient with 3PA and a 90th case of 3PA in the available literature., Patient Concerns and Interventions: A 36-year-old Caucasian male was admitted with headache and sudden increase in blood pressure. An abdominal CT scan revealed a retroperitoneal mass posterior to the inferior vena cava, later removed and diagnosed as a pheochromocytoma. Four years later, he noticed occasional mild headaches and a painless mass on the right side of his neck. The ultrasonography evaluations suggested a carotid body tumor, which was surgically removed. About a month after his second surgery, the severity of the patient's headaches worsened, and he developed right homonymous hemianopia. A brain MRI showed a mass in favor of macroadenoma, craniopharyngioma, or meningioma, and elevated prolactin level led to the diagnosis of macroprolactinoma., Diagnoses: Based on the provided history, this patient was diagnosed with 3PA, and a genetic study identified a positive succinate-dehydrogenase-complex subunit b mutation, possibly linked to his family history of carotid body tumor., Outcomes: He has remained symptom-free during his visits every 3 months., Lessons: The number of cases diagnosed with 3PA worldwide is increasing. Using clinical and genetic assessments, we can timely diagnose and adequately monitor individuals with or at risk of 3PA., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.)

Background: Many adrenal tumors are deemed radiologically indeterminate and surgically removed. Adrenal tissue, like parathyroid glands, exhibits near-infrared autofluorescence (NIRAF) properties. This study was designed to investigate the potential of NIRAF to differentiate benign versus malignant adrenal tumors., Methods: Patients undergoing adrenalectomy between October 2021 and May 2023 were prospectively studied. Adrenalectomy specimens were inspected with NIRAF imaging. Specimen autofluorescence (AF) characteristics were recorded. Comparisons were made between different tumor types and a logistic regression model was constructed to differentiate benign versus malignant tumors. A receiver operating characteristic curve was used to identify an optimal AF threshold differentiating benign versus malignant tumors., Results: A total of 108 adrenal specimens were examined: adrenocortical adenomas/other benign lesions (n = 72), pheochromocytomas (n = 18), adrenocortical neoplasms of uncertain behavior (n = 4), and malignant tumors (n = 14). A significant difference in normalized AF intensity was identified when comparing adrenocortical adenomas (3.08 times background) with pheochromocytomas (1.95, p = 0.001) and malignant tumors (1.11, p < 0.0001). The Area Under the Curve differentiating benign vs malignant tumors was 0.87, with an optimal normalized AF threshold at 1.93., Conclusions: Different adrenal pathologies exhibit diverse AF properties. These findings suggest a potential intraoperative utility of NIRAF in predicting benign versus malignant nature for radiologically indeterminate adrenal tumors., (© 2024. The Author(s).)

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., (© 2024. The Author(s).)

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., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Importance: This case report presents the successful surgical removal of a pheochromocytoma in a dog using indocyanine green (ICG) fluorescence imaging., Case Presentation: A 10-year-old, 6.87 kg, spayed female poodle was referred for an abdominal mass and eight episodes of vomiting. Computed tomography revealed masses in the spleen and left adrenal gland. Based on the plasma normetanephrine levels, the patient was diagnosed with pheochromocytoma. Subsequently, a total splenectomy and left adrenalectomy were performed. A dose of 5 mg/kg ICG was administered intravenously 24 h prior to surgery. Using ICG allowed visualization of tumor margins, aiding in complete resection and minimizing anesthesia-related risks., Conclusions and Relevance: No evidence of recurrence or metastasis has been observed for 12 months. To our knowledge, this is the first report of the surgical removal of a pheochromocytoma using intraoperative ICG imaging in veterinary medicine. This case highlights the efficacy of ICG fluorescence imaging as a valuable tool for the surgical management of adrenal tumors in veterinary medicine., Competing Interests: The authors declare no conflicts of interest., (© 2024 The Korean Society of Veterinary Science.)

Background: Up to 41% of intra- and extra-adrenal paragangliomas are linked to germline mutations with autosomal dominant transmission, which necessitates genetic testing for patients and their relatives. 1-4 Certain alterations, such as the succinate dehydrogenase (SDH) subunit B gene mutation, are associated with a significant risk of extra-adrenal, malignant, and metastatic disease forms. 4-7 This highlights the need for routine genetic counseling and diligent surveillance, as well as surgeon awareness of hereditary paraganglioma-pheochromocytoma syndrome (HPPS)., Methods: We present a multimedia article featuring a step-by-step video of a complex retroperitoneal resection, enriched with perioperative management insights., Results: A 17-year-old female presented with episodes of hypertension, tachycardia, and diffuse diaphoresis. CT revealed a paraaortic mass adjacent to the left renal hilum later confirmed by a SPECT/CT with iodine-123 meta-iodobenzylguanidine. 8 Additional imaging with gallium-68 DOTATATE was not performed then due to unknown mutation status. The patient underwent robotic removal of the tumor and adjacent lymph nodes. Pathology confirmed a poorly differentiated paraganglioma with 0/6 lymph node metastases. Genetic tests revealed SDHB gene mutation, indicative of HPPS. 9,10 At 12 months, the patient remained disease-free on CT with normalized metanephrines levels and no detectable circulating tumor DNA. Familial screening detected her mother, maternal uncle, and maternal grandfather to be SDHB mutation carriers, although phenotypically silent., Conclusions: Robotic-assisted resection can be safe and effective for retroperitoneal malignant paragangliomas. However, management extends beyond surgery and requires cascade genetic testing to address familial risks. Because of the high probability of cancer associated with SDHB mutation, lifelong patient surveillance is imperative., (© 2024. Society of Surgical Oncology.)

Background: Hypertension (HT) is one of the most common manifestations in patients with catecholamine-secreting neuroendocrine tumors. Although the cardiovascular manifestations of these tumors have been described, there have been no large-scale investigations of the profile of HT and changes in cardiac structure and function that occur in patients with pheochromocytomas and paragangliomas (PPGL)., Materials and Methods: In this study, we investigated the prevalence of HT and left ventricular remodeling (LVR) in a cohort of 598 patients who underwent surgery for PPGL at our center between January 2001 and April 2022. Information on demographics, reason for hospitalization, medical history, biochemical parameters, findings on echocardiography, and tumor characteristics were recorded. The LVR index was compared according to whether or not there was a history of HT., Results: The average age was 47.07 ± 15.07 years, and 277 (46.32%) of the patients were male. A history of HT was found in 423 (70.74%) of the 598 patients. Paraganglioma was significantly more common in the group with HT (26.00% vs. 17.71%, P = 0.030) and significantly less likely to be found incidentally during a health check-up in this group (22.93% vs. 59.43%, P < 0.001). Among 365 patients with complete echocardiography data, left ventricular mass index (86.58 ± 26.70 vs. 75.80 ± 17.26, P < 0.001) and relative wall thickness (0.43 ± 0. 08 vs. 0.41 ± 0.06, P = 0.012) were significantly higher in patients with PPGL and a history of HT. The proportions with left ventricular hypertrophy (LVH) (19.40% vs. 8.25%, P = 0.011) and LVR (53.73% vs. 39.18%, P = 0.014) were also higher when there was a history of HT. After adjusting for age, gender, body mass index, alcohol consumption, smoking status, diabetes, stroke, creatinine level, tumor location, and tumor size, a history of HT was significantly correlated with LVH (odds ratio 2.71, 95% confidence interval 1.18-6.19; P = 0.018) and LVR (odds ratio 1.83, 95% confidence interval 1.11-3.03; P = 0.018)., Conclusion: HT is common in patients with PPGL (70.74% in this cohort). PPGL without a history of HT is more likely to be found incidentally (59.43% in our cohort). HT is associated with LVR in PPGL patients with complete echocardiography data. These patients should be observed carefully for cardiac damage, especially those with a history of HT., (© 2024. The Author(s).)

The following case discusses the surgical considerations for a patient presenting with cardiogenic shock secondary to a phaeochromocytoma crisis with stress cardiomyopathy. The patient underwent an interval laparoscopic adrenalectomy. Pneumoperitoneum insufflation was performed at lower pressures; manipulation of the adrenal tumour was minimised, and the adrenal vein was ligated early. However, as intraoperative blood pressure (BP) remained elevated and rising, further gentle dissection revealed an aberrant inferior phrenic vein draining the adrenal nodule. BP was finally reduced following ligation of the inferior phrenic vein, demonstrating the clinical significance of an unusual dual venous drainage from the adrenal nodule in this patient., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: The anesthetic management of adrenalectomies for phaeochromocytoma excision, a catecholamine-secreting tumor, is challenging due to the potential for fatal complications following severe hemodynamic variations, including hypertensive crisis following tumor manipulation or sympathetic stimulation, but also severe hypotension and volume depletion post resection., Case Description: An 11 kg, 15-year-old male neutered Jack Russel Terrier, with mitral valve disease stage B2, was referred for adrenalectomy for phaeochromocytoma resection. The patient was administered per os prazosin 0.11 mg/kg twice a day and amlodipine 0.125 mg/kg once a day for preoperative stabilization. On the day of surgery, the dog received maropitant 1 mg/kg intravenously (IV) and was premedicated with 0.2 mg/kg methadone IV. Anesthesia was induced with alfaxalone 1 mg/kg IV and midazolam 0.2 mg/kg IV and maintained with partial intravenous anesthesia using sevoflurane in 70% oxygen and constant rate infusions of dexmedetomidine 0.5 μg/kg/hour and maropitant 100 μg/kg/hour. After induction of anesthesia, the dog was mechanically ventilated, and a transversus abdominal plane block was performed with ropivacaine 0.2%. The dog remained remarkably stable with a single, self-limiting, hypertension episode recorded intraoperatively. Postoperative rescue analgesia consisted of methadone and ketamine. The dog was discharged 48 hours after surgery, but persistent hypertension was reported at suture removal., Conclusion: The use of a low-dose dexmedetomidine CRI, a maropitant CRI, and a transversus abdominal plane block provided stable perioperative hemodynamic conditions for phaeochromocytoma excision in a dog., Competing Interests: The authors declare that there is no conflict of interest.

Introduction: Elevated metanephrine and catecholamine levels 3-fold upper limit of normal (ULN) are diagnostic for pheochromocytoma. We sought to determine whether size correlates with biochemical activity or symptoms which could guide timing of surgery., Methods: Data from consecutive patients undergoing adrenalectomy for pheochromocytoma at our institution over a 10-year period were retrospectively collected. These included maximal lesion diameter on preoperative imaging, plasma/urine metanephrine and/or catecholamine levels, demographic variables and presence of typical paroxysmal symptoms. Receiver operating characteristic curves were used to assess predictive accuracy., Results: Sixty-three patients were included in the analysis (41 females and 22 males). Median age was 56 (43, 69) years. Due to various referring practices, 31 patients had documented 24-h urine metanephrine, 26 had 24-h urine catecholamine, and 52 had fractionated plasma metanephrine levels available for review. Values were converted to fold change compared to ULN and the maximum of all measured values was used for logistic regression. Median tumor size was 3.40 (2.25, 4.55) cm in greatest dimension. Tumor size at which pheochromocytoma produced > 3-fold ULN was ≥2.3 cm (AUC of 0.84). Biochemical activity increased with doubling tumor size (odds ratio = 8, P = 0.0004) or ≥ 1 cm increase in tumor size (odds ratio = 3.03, P = 0.001). 40 patients had paroxysmal symptoms, but there was no significant correlation between tumor size/biochemical activity and symptoms., Conclusions: In our study, tumor size directly correlated with the degree of biochemical activity and pheochromocytomas ≥2.3 cm produced levels 3 times ULN. These findings may allow clinicians to adjust timing of operative intervention., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Introduction: Adrenalectomy generally has favorable outcomes. It is unknown if patients with functional adrenal tumors experience different clinical outcomes than those with benign adrenal tumors, due to the presence of comorbid conditions secondary to the functional tumor. We investigated outcomes following open and laparoscopic adrenalectomy for benign nonfunctional (BNF) versus functional adrenal masses., Methods: Patients undergoing adrenalectomy were identified in the 2015-2020 National Surgical Quality Improvement Program database, then categorized as BNF, hyperaldosteronism, hypercortisolism, and pheochromocytoma. The primary outcome of interest was 30-d morbidity and secondary outcomes included 30-d mortality, 30-d readmission, and postoperative length of stay (LOS). Subgroup analysis was performed based upon surgical approach. Univariate analysis was performed, followed by multivariable logistic regression for individual outcomes that differed significantly between patients with BNF and functional neoplasm, factoring in patient demographics and operative approach with statistical significance on univariate analysis. Descriptive statistics and outcomes were analyzed using Pearson's χ 2 test and Mann-Whitney U-test as appropriate., Results: There were 3291 patients with BNF while 484 had hyperaldosteronism, 263 hypercortisolism, and 46 pheochromocytomas. Within the laparoscopic group of 3615 (88.5%) of adrenalectomy patients, compared to BNF patients, patients with hyperaldosteronism had lower rates of postoperative morbidity (1.9% versus 5.2%, P < 0.001) and shorter LOS (1 d, interquartile range (IQR) [1-1] versus 1d IQR [1-2], P = 0.003); these persisted on multivariate analysis (OR 0.32, 95% confidence interval [CI] 0.14-0.74 and odds ratio 0.47, 95% CI 0.36-0.60, P < 0.001). Patients with hypercortisolism had higher morbidity (7.3% versus 5.2%, P < 0.001), 30-d readmission rates (5.3% versus 2.9%, P = 0.042) and longer LOS (2d, IQR [1-3] versus 1d, IQR [1-2, P < 0.001). On multivariate analysis, presence of hypercortisolism was independently associated with increased likelihood of readmission within 30 d (OR 2.20, 95% CI 1.11-2.99, P = 0.012) and longer LOS (>1 d) (OR 1.79, 95% CI 1.33-2.40, P < 0.001). Compared to BNF patients, patients with pheochromocytoma had higher rates of postoperative morbidity (6.2% versus 5.2%, P < 0.001). Within the open group of 469 (11.5% of adrenalectomy patients), there were no statistically significant differences in outcomes between patients with BNF and functional adrenal masses., Conclusions: Outcomes after adrenalectomy performed for functional neoplasms differ based on surgical indication., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Pheochromocytoma is a tumor that usually originating from adrenal medullary chromaffin cells and producing one or more catecholamines, can manifest as hereditary or sporadic. While the majority pheochromocytomas are sporadic, hereditary forms are often associated with genetic syndromes such as von Hippel-Lindau, multiple endocrine neoplasia type 2, and neurofibromatosis type 1. This study aims to analyze data from our series of surgically excited pheochromocytoma patients and compare the characteristics between hereditary and sporadic cases. We retrospectively evaluated 33 diagnosed pheochromocytoma patients, documenting clinical features, surgical complications, and tumor characteristics in both hereditary and sporadic cases. Among the patients, 21% (7 individuals) had hereditary pheochromocytoma, while 79% (26 individuals) had sporadic cases. During diagnosis, hereditary pheochromocytoma patients exhibited a significantly lower mean age compared to the sporadic group (26.4 ± 9.9 years vs. 50.4 ± 14.0 years; p < 0.001). The maximum tumor size was also lower in hereditary cases compared to sporadic cases (p = 0.004). Adrenal tumor localization analysis showed that 63.6% were right-sided, 24.2% were left-sided, and 12.1% were bilateral. Laboratory analysis revealed significantly higher urinary norepinephrine levels in hereditary pheochromocytoma patients (p = 0.021). Our findings suggest that hereditary pheochromocytoma cases are characterized by a younger age at diagnosis, smaller tumor size, and a higher prevalence of multiple bilateral adrenal adenomas. We recommend genetic testing for all pheochromocytoma patients, particularly those with early-onset disease and bilateral adrenal tumors., (© 2024 The Authors. The Kaohsiung Journal of Medical Sciences published by John Wiley & Sons Australia, Ltd on behalf of Kaohsiung Medical University.)

Objectives: Pheochromocytomas (PHEO) are neuroendocrine tumors rarely diagnosed in children. We are reporting on the management challenges of three adolescent patients who present with hereditary PHEO., Case Presentation: The index patient and his male sibling presented with bilateral PHEO, while a third patient presented with a unilateral PHEO, all associated with von Hippel-Lindau (VHL) syndrome. The patients were treated with computed tomography (CT)-guided percutaneous cryoablation (CRA) of the adrenal lesions, with varying degrees of success., Conclusions: CT-guided percutaneous CRA of hereditary PHEO has not been reported in the pediatric population and may represent a novel treatment strategy that reduces the risk of intraprocedural complications and adrenal insufficiency (AI)., (© 2024 Walter de Gruyter GmbH, Berlin/Boston.)

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., (© 2024. The Author(s).)

Background: Pheochromocytoma is rare in pregnant women. It presents as diverse symptoms, including hypertension and sweating. The symptoms of pregnant women with pheochromocytoma and comorbid hypertension often mimic the clinical manifestations of preeclampsia, and these women are often misdiagnosed with preeclampsia., Case Presentation: In this case, a pregnant woman presented with chest pain as the primary symptom, and a diagnosis of pheochromocytoma was considered after ruling out myocardial ischemia and aortic dissection with the relevant diagnostic tools. This patient then underwent successful surgical resection using a nontraditional management approach, which resulted in a positive clinical outcome., Conclusions: It is essential to consider pheochromocytoma as a potential cause of chest pain and myocardial infarction-like electrocardiographic changes in pregnant women, even if they do not have a history of hypertension., (© 2024. The Author(s).)

Purpose: Preoperative medical management is critical to prevent intraoperative cardiovascular complications in patients with pheochromocytomas and paragangliomas (PPGLs). Initial treatment involves α-adrenergic receptor blockers. However, while the routine use of metyrosine alongside these blockers is not strongly recommended due to a lack of evidence supporting its efficacy and associated safety concerns, there are previous studies on combination therapy with phenoxybenzamine and metyrosine. There are few reports on combination therapy with the selective α1-adrenergic receptor blocker doxazosin. Therefore, we investigated this combination treatment, which theoretically can affect perioperative outcomes in patients with PPGLs. To our knowledge, this is the first such study., Methods: This retrospective single-center observational study involved 51 patients who underwent surgical resection of PPGLs at Kobe University Hospital between 2014 and 2022. All patients received doxazosin at maximum doses. Fourteen patients received concomitant metyrosine, while 37 received doxazosin alone. Their perioperative outcomes were compared., Results: No severe event, such as acute coronary syndrome, was observed in either group. Intraoperatively, the doxazosin + metyrosine group exhibited a lower median minimum systolic blood pressure (56 [54-60] vs. 68 [59-74] mmHg, P = 0.03) and required lower median remifentanil (P = 0.04) and diltiazem (P = 0.02) doses than the doxazosin-alone group., Conclusion: The combination of metyrosine and doxazosin as a preoperative treatment for PPGLs affects intraoperative circulatory hemodynamics, such as a reduced occurrence of blood pressure elevation during surgery. Further research is necessary to identify patients who will benefit most from this combination treatment., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Pheochromocytoma is a chromaffin cell-derived adrenal medullary tumour and usually presents with paroxysms of hypertension, palpitations, sweating and headache due to excessive catecholamine release. These tumours can also secrete a variety of bioactive neuropeptides and hormones other than catecholamines, resulting in unusual clinical manifestations. We report a female in her mid-30s who presented with fever, anaemia, thrombocytosis and markedly elevated inflammatory markers. The fever profile, including cultures, was negative. Contrast-enhanced CT of abdomen showed a large solid-cystic right adrenal lesion with elevated plasma-free normetanephrine levels suggestive of pheochromocytoma. The fever persisted despite empirical antibiotics and antipyretics. Interleukin-6 (IL-6) levels were elevated (41.2 pg/mL (3-4 pg/mL)). She was initiated on naproxen (NPX) at a dose of 250 mg two times per day. The patient responded to NPX, and after stabilisation, she underwent an adrenalectomy. There was a complete resolution of fever with normalisation of IL-6 levels postoperatively., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Laparoscopic adrenalectomy is widely performed for a number of hormone-producing tumors and postoperative management depends on the hormones produced. In the present study, we conducted a retrospective analysis to clarify the risk factors for postoperative complications, particularly postoperative fever after laparoscopic adrenalectomy., Methods: We analyzed 406 patients who underwent laparoscopic adrenalectomy at our hospital between 2003 and 2019. Postoperative fever was defined as a fever of 38 °C or higher within 72 h after surgery. We investigated the risk factors for postoperative fever after laparoscopic adrenalectomy., Results: There were 188 males (46%) and 218 females (54%) with a median age of 52 years. Among these patients, tumor pathologies included 188 primary aldosteronism (46%), 75 Cushing syndrome (18%), and 80 pheochromocytoma (20%). Postoperative fever developed in 124 of all patients (31%), 30% of those with primary aldosteronism, 53% of those with pheochromocytoma, and 8% of those with Cushing syndrome. A multivariate logistic regression analysis identified pheochromocytoma and non-Cushing syndrome as independent predictors of postoperative fever. Postoperative fever was observed in 42 out of 80 cases of pheochromocytoma (53%), which was significantly higher than in cases of non-pheochromocytoma (82/326, 25%, p < 0.01). In contrast, postoperative fever developed in 6 out of 75 cases of Cushing syndrome (8%), which was significantly lower than in cases of non-Cushing syndrome (118/331, 35.6%, p < 0.01)., Conclusion: Since postoperative fever after laparoscopic adrenalectomy is markedly affected by the hormone produced by pheochromocytoma and Cushing syndrome, it is important to carefully consider the need for treatment., (© 2024. The Author(s).)

Objective: Thromboelastography (TEG) is a whole blood assay that yields global assessment of hemostasis, as it evaluates clot time, strength, and kinematics of clot formation and lysis. The main objective was to describe preoperative TEG findings in dogs that had an adrenalectomy performed and, secondarily, to describe TEG findings in the dogs with or without hyperadrenocorticism (HAC)., Animals: 30 dogs that had preoperative TEG and adrenalectomy performed., Methods: Medical records between 2018 and 2022 were reviewed. Signalment, diagnostic data, and perioperative treatment were abstracted., Results: 53% (16/30) of the dogs were hypercoagulable, and none were hypocoagulable. Based on histopathology, 6 of 9 dogs with adenocarcinoma were hypercoagulable, 4 of 8 with pheochromocytoma were hypercoagulable, and 6 of 10 with adenoma were hypercoagulable. None of the 3 dogs with other histopathologic diagnoses or combinations of diagnoses (adrenocortical hyperplasia, poorly differentiated sarcoma, and both adrenocortical adenocarcinoma and pheochromocytoma) were hypercoagulable. Of the 14 dogs tested preoperatively for HAC, 4 of 8 HAC dogs were hypercoagulable and 2 of 6 non-HAC dogs were hypercoagulable., Clinical Relevance: The present report describes for the first time TEG findings for dogs undergoing adrenalectomy and suggests that the majority of dogs with adrenal neoplasia are hypercoagulable based on TEG results.

Purpose: Surgery is the only way to cure pheochromocytoma; however, postoperative hemodynamic instability is one of the main causes of serious complications and even death. This study's findings provide some guidance for improved clinical management., Patients and Methods: This study was to investigate the factors leading to postoperative hemodynamic instability in the postoperative pathology indicated pheochromocytoma from May 2016 to May 2022. They were divided into two groups according to whether vasoactive drugs were used for a median number of days or more postoperatively. The factors affecting the postoperative hemodynamics in the perioperative period (preoperative, intraoperative, and postoperative) were then evaluated., Results: The median number of days requiring vasoactive drug support postoperatively was three in 234 patients, while 118 (50.4%) patients required vasoactive drug support for three days or more postoperatively. The results of the multivariate analysis indicated more preoperative colloid use (odds ratio [OR]=1.834, confidence interval [CI]:1.265-2.659, P=0.001), intraoperative use of vasoactive drug (OR=4.174, CI:1.882-9.258, P<0.001), and more postoperative crystalloid solution input per unit of body weight per day (ml/kg/d) (OR=1.087, CI:1.062-1.112, P<0.001) were risk factors for predicting postoperative hemodynamic instability. The optimal cutoff point of postoperative crystalloid use were 42.37 ml/kg/d., Conclusion: Hemodynamic instability is a key issue for consideration in the perioperative period of pheochromocytoma. The amount of preoperative colloid use, the need for intraoperative vasoactive drugs, and postoperative crystalloid solution are risk factors for predicting postoperative hemodynamic instability (registration number: ChiCT2300071166)., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 Liu, Shang, Zhuang, Su, Li, Sun and Long.)

&lt;b&gt;Introduction:&lt;/b&gt; Adrenal hemorrhage (AH) is a very rare and potentially life-threatening disease which may be secondary to trauma or of non-traumatic etiology.&lt;b&gt;Aim:&lt;/b&gt; The aim of the study was to present the characteristics and management of adrenal hemorrhage and show that adrenal hemorrhage is more common than expected and that the clinical symptoms are not specific.&lt;b&gt;Materials and methods:&lt;/b&gt; This retrospective study involved 199 patients with postoperative diagnosis of adrenal hemorrhage.&lt;b&gt;Discussion:&lt;/b&gt; The factors identified as potential causes of adrenal hemorrhage are adrenocortical carcinoma, pheochromocytoma, and adrenal adenoma. The study group included 199 patients with postoperative diagnosis of AH. It showed that all patients with postoperative diagnosis had pheochromocytoma (n = 54), adrenal adenoma (n = 68), or adenocarcinoma (n = 17). If we look more careful at the results, we can find only 30% of patients (n = 39) with preoperative diagnosis of AH. This group of 39 patients was prepared for expedited surgery. In this group of patients, the preoperative diagnosis of AH was pheochromocytoma 28% (n = 11), adenocarcinoma (n = 4), and adrenal adenoma (n = 9).&lt;b&gt;Conclusions:&lt;/b&gt; Bleeding into adrenal tumors is still an insufficiently understood topic due to its unpredictability and, as can be seen in our material, of varying severity. Out of 199 patients, only 30% (n = 39) were prepared for surgery with a preoperative diagnosis of AH; most of them had pheochromocytoma. We suggest that is very important to prepare patients for surgery with a preoperative diagnosis of AH using α-adrenoreceptor antagonists. Prolongation of the diagnostic process (time between the imaging examination and the surgery) may result in the disease progressing and adrenal bleeding.

Takotsubo syndrome (TTS) is a transient left ventricle dysfunction usually caused by a stressful trigger (emotional or physical). We report the case of a 77 year-old female patient who presented with TTS caused by a pheochromocytoma, a catecholamine-producing neuroendocrine tumour. Diagnosis was facilitated by acute kidney injury prompting renal ultrasound, recurrence of TTS and symptoms of episodic palpitations, profuse sweating and labile blood pressure. Furthermore, during her hospitalisation the patient also developed an Ogilvie syndrome, an acute colonic pseudo-obstruction, due to the catecholamine-excess. Treatment consisted of betablocker and angiotensin-converting enzyme inhibitor for TTS, neostigmine for Ogilvie syndrome, in combination with alpha-blocker and surgical removal of the tumour after recuperation of left ventricular function and colonic pseudo-obstruction. To our knowledge, this is the first case report of the pathophysiological triad of pheochromocytoma leading to Takotsubo and Ogilvie syndrome in a single patient.

Background: This report presents a case of cannabinoid-induced hyperemesis syndrome causing repeated violent retching in a patient with a large (8 cm) adrenal pheochromocytoma resulting in hypertensive urgency., Case Presentation: A 69-year-old white male patient with a previously diagnosed pheochromocytoma presented to the emergency department with nausea and vomiting and was found to have hypertensive urgency. Computed tomography scan did not show any acute abdominal pathology and history was inconsistent with a gastrointestinal etiology. Patient had a history of daily cannabinoid use for many years and repeated self-limited hyperemesis episodes, and thus a diagnosis of cannabinoid-induced hyperemesis syndrome was made. It was concluded that the likely explanation for the hypertensive urgency was from physical compression of his adrenal tumor during the episodes of retching resulting in a catecholamine surge. The patient was given antiemetics and admitted to the intensive care unit for blood pressure management. Blood pressure was initially controlled with phentolamine and a clevidipine infusion, then transitioned to oral doxazosin and phenoxybenzamine. Hyperemesis and abdominal pain resolved after 24 hours, and his blood pressure returned to baseline. The patient was discharged with the recommendation to stop all cannabis use. On follow-up, his blood pressure remained well controlled, and he subsequently underwent adrenalectomy for tumor removal., Conclusion: Hyperemesis can cause hypertensive events in patients with pheochromocytoma by increasing abdominal pressure, leading to catecholamine release., (© 2024. The Author(s).)

To compare the clinical efficacy and safety of robot-assisted adrenalectomy (RA) and laparoscopic adrenalectomy (LA) for pheochromocytoma (PHEO). We conducted a comprehensive search of PubMed, the Cochrane Library, and Embase databases for studies comparing RA and LA treatment for PHEO, covering the period from database inception to January 1, 2024. Two researchers will independently screen literature and extract data, followed by meta-analysis using Review Manager 5.3 software. Six studies with 658 patients were included in the analysis. There were no significant differences in operation time [MD = -8.03, 95% CI (-25.68,9.62), P > 0.05], transfusion rate [OR = 1.10, 95% CI (0.55, 2.19) , P > 0.05], conversion rate [OR = 0.31, 95% CI (0.08, 1.12), P > 0.05], complication rate [OR = 0.93, 95% CI (0.52, 1.70), P > 0.05], Intraoperative max SBP [MD = -4.08, 95% CI (-10.13,1.97), P > 0.05], Intraoperative min SBP [MD = -2.71, 95% CI (-9.60,4.18), P > 0.05] among patients undergoing RA and LA. However, compared with patients who underwent LA, patients who underwent RA had less estimated blood loss [MD = -37.72, 95% CI (-64.11,-11.33), P < 0.05], a shorter length of hospital stay [MD = -0.43, 95% CI (-0.65,-0.21) P < 0.05]. RA has higher advantages in some aspects compared to LA. RA is a feasible, safe, and comparable treatment option for PHEO., (© 2024. The Author(s), under exclusive licence to Springer-Verlag London Ltd., part of Springer Nature.)

Background: This case report documents a case of malignant pheochromocytoma manifested as vision changes with lung metastasis and recurrence., Case Presentation: A 10-year-old Han Chinese girl presented with vision changes and was eventually diagnosed with pheochromocytoma by contrast-enhanced computed tomography, urine vanillylmandelic acid. After medication for hypertension and surgery, clinical symptoms disappeared. Malignant pheochromocytoma with lung metastasis was confirmed histologically using the Pheochromocytoma of the Adrenal Gland Scaled Score scoring system and genetically with succinate dehydrogenase complex iron sulfur subunit B mutation, and 3 months later, unplanned surgery was performed because of the high risks and signs of recurrence. She is asymptomatic as of the writing of this case report. Our patient's case highlights the importance of considering a diagnosis of malignant pheochromocytoma, and long-term follow-up for possible recurrence., Conclusion: Although there are well-recognized classic clinical manifestations associated with pheochromocytoma, atypical presentation, such as vision changes in children, should be considered. In addition, malignant pheochromocytoma children with a high Pheochromocytoma of the Adrenal Gland Scaled Score and succinate dehydrogenase complex iron sulfur subunit B mutation require a long-term follow-up or even unplanned surgery because of the higher risk of recurrence., (© 2024. The Author(s).)