Resection Of Pheochromocytoma Research Articles

A Retrospective Case Series on Planned and Accidental Pheochromocytoma Resection Surgery: Role of Preoperative Preparation

A Retrospective Case Series on Planned and Accidental Pheochromocytoma Resection Surgery: Role of Preoperative Preparation

Exploration of factors affecting hemodynamic stability following pheochromocytoma resection - cohort study.

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. 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. 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. 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).

Preoperative 3-dimensional visualization technology-assisted laparoscopic resection of ectopic pheochromocytoma surrounding the renal artery: a case description.

Preoperative 3-dimensional visualization technology-assisted laparoscopic resection of ectopic pheochromocytoma surrounding the renal artery: a case description.

Laparoscopic Management of Adrenal and Extra-Adrenal Pheochromocytoma

Background and Purpose Laparoscopic management of extra-adrenal pheochromocytoma requires meticulous surgical procedures because of the variable anatomic position and/or proximity to major blood vessels and the potential catecholamine surge during operation. We present our experiences with laparoscopic management of extra-adrenal pheochromocytoma and compare the intraoperative hemodynamics with those during laparoscopic resection of adrenal pheochromocytoma. Patients and Methods We retrospectively reviewed the medical records of 3 patients who underwent laparoscopic management of extra-adrenal pheochromocytoma (retrocaval, n=1; interaortocaval, n=1; periadrenal, n=1) and 24 patients who underwent laparoscopic management of adrenal pheochromocytoma between June 2005 and June 2023. We also evaluated fluctuations in blood pressure (BP) reported during both surgeries. Results The tumors were successfully resected under laparoscopic guidance in both groups, and one patient had converted to open surgery. Intraoperative hypertension (BP&gt;200 mm Hg) was observed in 1 extra-adrenal pheochromocytoma and 13 adrenal pheochromocytoma patients, whereas intraoperative hypotension (BP &lt;80 mm Hg) was observed in 1 extra-adrenal pheochromocytoma and 12 adrenal pheochromocytoma patients. Conclusion Laparoscopy is the method of choice for extra-adrenal resection because it is feasible and reproducible with appropriate preoperative planning, similar to adrenal resection.

Giant left pheochromocytoma with vascular anomalies and pelvic horseshoe kidney: a case report

BackgroundPheochromocytoma is a neuroendocrine tumor, and its treatment is dependent on surgical resection. Due to the wide availability of cross-sectional imaging, pheochromocytomas are commonly seen as small tumors less than 10 cm in size and are mostly treated with minimally invasive surgery. Their concomitant presence with horseshoe kidney or other anatomical and vascular anomalies is rare. Herein, we present a surgically complex giant pheochromocytoma case who underwent an open left radical adrenalectomy.Case presentationA 41-year-old Hispanic female presented with a 12 × 8 cm left hypervascular adrenal mass, pelvic horseshoe kidney, and severely dilated large left retro-aortic renal vein which branched into a left adrenal vein, congested left ovarian vein, and left uterine plexus. She was managed with insulin and metformin for uncontrolled diabetes with an A1c level of 9% and doxazosin for persistent hypertension. Clinical diagnosis of pheochromocytoma was confirmed with elevated urine and serum metanephrine and normetanephrine. The pre-operative ACTH was within normal range with a normal dexamethasone suppression test and 24-hour urine free cortisol. The adrenalectomy of the highly aggressive adrenal mass was performed via open approach to obtain adequate surgical exposure. Due to the large size of the tumor and its significant involvement with multiple adjacent structures, coordination with multiple surgical teams and close hemodynamic monitoring by anesthesiology was required for successful patient outcomes including preservation of blood supply to the pelvic horseshoe kidney. The histopathological diagnosis was pheochromocytoma with negative surgical margins. The patient was followed at 1, 4, 12, and 24 weeks postoperatively. She had a normal postoperative eGFR and was able to discontinue antihypertensive and antidiabetic medications at four weeks. She had transient adrenal insufficiency, which resolved at five months. The horseshoe kidney was intact except for a minimal area of hypo-enhancement in the left superior renal moiety due to infarction, which was significantly improved at six months.ConclusionOur patient had a giant pheochromocytoma with anatomical variations complicating an already surgically challenging procedure. Nonetheless, with multiple provider collaboration, detailed pre-operative surgical planning, and meticulous perioperative monitoring, radical resection of the giant pheochromocytoma was safe and feasible with successful postoperative outcomes.

FRI194 Pheochromocytoma Presenting As Catecholamine-Induced Cardiomyopathy: A Case Report

Abstract Disclosure: M. Tsikala Vafea: None. D. Lee: None. Introduction: Pheochromocytoma (PCC) is a rare catecholamine-secreting neuroendocrine tumor presenting with the classic triad of episodic headache, sweating and tachycardia. Catecholamine-induced cardiomyopathy (CICMP) can occur as a complication of PCC in 7-11% of patients. It presents more commonly as dilated cardiomyopathy and inverted Takotsubo cardiomyopathy but also as hypertrophic or unspecified cardiomyopathy. Following appropriate pharmacologic treatment and resection of PCC, these cardiac complications usually resolve. Case report: 36 year old man with history of hypertension presented to the hospital with worsening orthopnea and cough. Following antibiotic treatment failure for suspected pneumonia, he was re-admitted with hemoptysis, tachycardia, hypoxia, and hypertensive emergency. Troponin and brain natriuretic peptide were found to be elevated. CT chest and abdomen showed pulmonary nodules and a large left adrenal mass measuring 9.6x9.3x9.4 cm, concerning for metastatic PCC. He was subsequently started on doxazosin. Transthoracic echo showed bi-atrial enlargement, diffuse severe left ventricle hypokinesis, reduced ejection fraction 25% and diastolic dysfunction. Stress cardiac MRI showed non-ischemic cardiomyopathy, corroborating the diagnosis of CICMP. After achieving adequate alpha-adrenergic blockade, he was started on goal-direct medical therapy consisting of valsartan, metoprolol and empagliflozin. His pulmonary nodular opacities improved on repeat imaging, congruent with pulmonary edema rather than metastases. Plasma and 24-hour urine metanephrines (2,964 pg/mL and 23,310 mcg/24 hr, respectively) and normetanephrines (9,266 pg/mL and 33,927 mcg/24 hr, respectively), as well as dopamine (493 pg/mL), epinephrine (3,205 pg/mL) and norepinephrine (above reportable range of 4,800 pg/mL) eventually came back significantly elevated, confirming his diagnosis of PCC. A month after presentation, he underwent left adrenalectomy, which revealed an 11 cm composite PCC ganglioneuroma. One-month postoperatively, urine metanephrines and normetanephrines were normal but his ejection fraction remained unchanged. Discussion: CICMP should be considered in patients presenting with acute heart failure and an incidental adrenal mass to avoid delays in treatment. Acute pulmonary edema may resemble pulmonary nodules on CT, so interpretation of these findings should correlate with the clinical presentation. Timely initiation of alpha-adrenergic blockade upon suspicion of PCC is crucial as it can significantly improve hemodynamics, and biochemical markers take time to result. 96% of CICMP cases improve after resection of PCC within a variable time frame ranging from one week to several months. Our patient’s CICMP had not improved one month after his adrenalectomy, but it remains to be seen whether he eventually recovers his cardiac function. Presentation: Friday, June 16, 2023

SAT311 A Time And Place For Metyrosine: Successful Treatment Of A Pheochromocytoma Presenting With Hypotension And Multisystem Organ Failure

Abstract Disclosure: R. Shafiq: None. J. Giordano: None. Pheochromocytomas are rare neuroendocrine catecholamine-secreting tumors. The surgical excision of pheochromocytoma is definitive management which requires presurgical preparation with an alpha blocker, beta blocker, and sometimes metyrosine to reduce perioperative hemodynamic instability. We present a case of pheochromocytoma complicated with acute on chronic renal failure and acute on chronic heart failure with hypotension requiring metyrosine in presurgical preparation due to hypotension and intolerance to alpha-blocker with successful surgical excision of the tumor. Case: 67 yo M admitted for acute on chronic heart failure and acute on chronic renal failure. He was noted to have an incidental right adrenal mass of 4.3 x 4.5 cm on the CT chest. He denied any prior history of hypertension, episodes of flushing, sweating, palpitations, headaches, or any family history of adrenal malignancies. His labs were consistent with significantly elevated plasma metanephrines to 4.5 nmol/L (normal range &amp;lt; 0.5 nmol/L) and normetanephrine 36 nmol/L (normal range &amp;lt; 0.9 nmol/L). Levels were repeated with slight worsening of metanephrines to 5.6 and normetanephrine to 42. He was hypotensive on presentation initially on milrinone drip that was slowly tapered off. His echo on admission showed left ventricle global hypokinesis with EF 25-30%. He had a worsening renal function on admission and required a few dialysis sessions before being referred to our hospital. He had an MRI adrenal done w/o contrast in the setting of renal failure with a heterogenous mass concerning for pheochromocytoma. PET CT was done which was negative for metastatic disease. Due to strong suspicion of uncontrolled pheochromocytoma as a cause of his heart failure and acute on chronic renal failure, the team and patient opted for unilateral adrenalectomy. He was started on metyrosine pre-surgically as he could not tolerate alpha blockade due to hypotension. His metanephrine levels improved from 5.6 to 1.8 and normetanephrine from 43 to 15 with metyrosine prior to surgery. He had a successful right adrenalectomy and pathology confirmed pheochromocytoma. Of note, he had a history of diabetes with uncontrolled hyperglycemia during admission. Hyperglycemia resolved after adrenalectomy. His plasma metanephrine levels normalized post-adrenalectomy and remained normal on the follow-up visit. Conclusion: Preoperative medical stabilization is a crucial step before surgical resection of pheochromocytoma that traditionally includes an alpha blocker followed by a beta-blocking agent to reduce intra-operative hypertensive crisis. Metyrosine is an often-overlooked agent due to cost, however, should remain in our armamentarium for pheochromocytoma crisis complicated with hypotension when the patient is unable to tolerate an alpha-blocking agent. Presentation: Saturday, June 17, 2023

Two Stage Resection of a Bilateral Pheochromocytoma and Locally Advanced Pancreatic Tumor in a 26 Years Old Pregnant Women: Case Report

Synchronous occurrence of pheochromocytoma and pancreatic tumors is rare and considered as part of Von Hippel Lindau Syndrome. We herein report a such case that was managed by a two staged resection of the pancreatic tumor along with excision of bilateral pheochromocytomas and also discuss in detail our review of literature about this condition.

O159 Nonselective alpha blockade alone is associated with a significant decrease in ICU/CCU requirement following phaeochromocytoma resection

Abstract Introduction Preoperative treatment with alpha blockade is key to optimisation of patients with phaeochromocytoma. Beta blockers may be employed after optimal alpha blockade to prevent reflex tachycardia. The principle of treatment is to avoid severe intraoperative hypertension or hypotension, but the optimal perioperative regimen is unclear. Recent studies have suggested that dual blockade may increase the rate of intraoperative haemodynamic instability We hypothesized that nonselective alpha blockade alone, without addition of beta blockers, would reduce the need for postoperative ICU/CCU supportive care. Methods A retrospective study of all patients undergoing pheochromocytoma/paraganglioma resection between 1998 and 2022 was performed. Patients were categorized into two cohorts: Group 1 patients received alpha and beta blockers preoperatively while Group 2 patients received nonselective alpha blockade alone. The primary endpoint was the need for ICU/CCU support. Results Of 40 patients identified, adrenalectomy was performed in 36. Median age at resection was similar for both groups (63 v 56 years). Of 22 Group 1 patients 8 (36.4%) required ICU/CCU admission due to haemodynamic instability, (MLS 1.22 days) compared to 3 (21.4%) of the 14 Group 2 (MLS 0.21, p=0.035; one-tailed Two-Sample t-test assuming unequal variance). Median maximum diameter of lesions in Group 1 was 5.4cm, while Group 2 was 4.2cm. In Group 1 59% showed right adrenal disease (n=13), Group 2 43% (n=6). Conclusion Our study suggests that nonselective alpha blocker alone, without the addition of beta blockers, is associated with a significantly decreased requirement for post-operative support in the ICU or CCU following pheochromocytoma resection.

Influence of duration of preoperative treatment with phenoxybenzamine and secretory phenotypes on perioperative hemodynamics and postoperative outcomes in pheochromocytoma and paraganglioma.

Resection of pheochromocytoma and paraganglioma (PPGL) carries risks with perioperative hemodynamic instability. Phenoxybenzamine (PXB) is a commonly used α-blockade to prevent it. It is unclear whether lengthening the preoperative duration of PXB is better for hemodynamic stability and postoperative outcomes. Furthermore, different types of catecholamines have varying effects on perioperative hemodynamics. Thus, our study aimed to investigate the impact of the duration of preoperative preparation with PXB and secretory phenotypes of the patients on intraoperative hemodynamic stability and postoperative complications in PPGL. Between Dec 2014 and Jan 2022, 166 patients with PPGL were operated on by the same team at Sun Yat-sen Memorial Hospital. They were divided into group A(1-14d), Group B(15-21d), and Group C(>21d) based on the duration of management with PXB and into the adrenergic and the noradrenergic phenotype group based on secretory profiles. Data on intraoperative hemodynamics and postoperative outcomes were collected and compared among groups. A total of 96 patients occurred intraoperative hemodynamic instability, and 24 patients had 29 postoperative complications related to the surgery. Among the 145 patients treated with PXB, no significant differences were found in the cumulative time outside the target blood pressure(6.67%[0-17.16%] vs. 5.97%[0-23.08%] vs. 1.22%[0-17.27%], p=0.736) or in the median total HI-score(42.00[30.00-91.00] vs. 89.00[30.00-113.00] vs. 49.00[30.00-93.00], p=0.150) among group A(n=45), B(n=51) and C(n=49). Multivariate analysis demonstrated that the level of plasma-free metanephrine(MN) was an independent risk factor for intraoperative hemodynamic instability. And the median cumulative time outside of the target blood pressure in the adrenergic phenotype group was significantly greater than that in the noradrenergic phenotype group(8.17%[0-26.22%] vs. 1.86%[0-11.74%], p=0.029). However, the median total HI-score(99.50[85.00-113.25] vs. 90.00[78.00-105.00], p=0.570) and postoperative outcomes showed no differences between the two groups. A preoperative duration of nearly 14 days with PXB is sufficient for ensuring intraoperative hemodynamic stability in PPGL. And lengthening the preparation duration may not provide additional benefits in the era of widespread application and advanced techniques of laparoscopic surgery. Additionally, patients with the adrenergic phenotype are more prone to intraoperative hemodynamic instability than the noradrenergic phenotype. Thus, more attention should be given to the adrenergic phenotype during surgery.

Preemptively planned en bloc resection of an extensive right adrenal pheochromocytoma involving the right hepatic division, caval thrombus and segmental caudal vena cava in a dog with Budd−Chiari‐like syndrome

Surgical resection is the treatment of choice for canine adrenal pheochromocytomas (PHEOs). Information on en bloc resection of adrenal PHEO with tumour thrombus, right hepatic division and segmental caudal vena cava (CVC) running through the adrenal tumour and right hepatic division is limited. To describe the preemptively planned en bloc resection of an extensive right adrenal PHEO involving the right hepatic division, the caval thrombus and the segmental CVC in a dog with Budd-Chiari-like syndrome (BCLS). A 13-year-old castrated male miniature dachshund was referred for surgical treatment due to anorexia, lethargy and severe abdominal distension caused by abundant ascites. Preoperative computed tomography (CT) revealed a large mass in the right adrenal gland with a large caval thrombus obstructing the CVC and hepatic veins, which caused BCLS. Additionally, collateral vessels were formed between the CVC and azygos veins. No findings suggested obvious metastases. Based on CT findings, an en bloc resection of the adrenal tumour with caval thrombus, right hepatic division and segmental CVC was planned. The preoperatively planned resection was feasible; the tumour was completely resected grossly. The operation time and total Pringle manoeuvre time were 162min and 16min 56 s, respectively. There was no postoperative hindlimb oedema, renal dysfunction, ascites or abdominal distention. The patient's clinical signs, including appetite, fully improved. Hospitalization lasted 16 days. However, the patient died on the 130th postoperative day due to suspected metastases and cachexia. Even in case of an extensive infiltration of adrenal PHEO causing BCLS, an en bloc resection might be successfully achieved based on the preoperative CT findings speculating the collateral vessels formed for caudal venous return.

Comparison of lateral transperitoneal versus retroperitoneal laparoscopic adrenalectomy for pheochromocytoma: a single-centre retrospective study.

Laparoscopic adrenalectomy (LA) has became the standardized treatment for pheochromocytoma. The aim of this study was to evaluate outcomes of lateral transperitoneal and retroperitoneal LA for pheochromocytoma. Between January 2011 and December 2021, 142 patients with pheochromocytoma underwent LA via lateral transperitoneal (123 patients) or retroperitoneal (19 patients) approaches. Data of these patients were assessed to investigate the differences in perioperative outcomes and intraoperative haemodynamic parameters between the two procedures. Clinical parameters at presentation were comparable between the two groups, except for tumour size, which was larger in the transperitoneal group (50 [10-115] mm vs 35 [7-110] mm, P = 0.012). There were no significant differences between the two groups in terms of operation time, estimated blood loss, intraoperative transfusion rate, incidence of complications, conversion to open surgery, postoperative analgesic requirement, time to first oral intake, or mean hospital stay. Intraoperative haemodynamic parameters of the two groups were similar. After adjusting for tumour size using propensity score matching, both perioperative outcomes and haemodynamic parameters were still comparable. Lateral transperitoneal and retroperitoneal laparoscopic adrenalectomies provide similar perioperative and haemodynamic outcomes for surgical resection of pheochromocytoma.

Deliberate Compensated Vasoplegia-A Novel Pharmaceutical Approach for Controlling Blood Pressure During Surgery for Pheochromocytoma.

The most dreaded adverse event of pheochromocytoma surgery is operative severe blood pressure fluctuations. Preoperative protocols with alpha-blockade have achieved controversial results. No study to date evaluated the use of operative protocols in pheochromocytoma patients. Deliberated compensated vasoplegia (DCV) is a novel pharmaceutical regimen developed at our institution to decrease severe hypertensive events. The aim of this study is to compare outcomes of pheochromocytoma resection with and without DCV protocol. A retrospective analysis of all pheochromocytoma resections between the years 2012 and 2021 was performed. Resections performed with and without DCV protocol were compared. The primary outcome measured was the incidence of severe hypertension (MAP > 150mmHg) during surgery. Secondary outcomes included other abnormal blood pressure measurements as well as perioperative data and complications. A total of 41 resections were included, 21 performed under DCV protocol, and 20 without the protocol. Analysis demonstrated no significant difference in preoperative parameters including tumor size, catecholamine levels, and preoperative alpha-blockade protocol. The use of DCV protocol resulted in significant decrease in severe hypertension incidence from 1.95 ± 3.6 to 0.03 ± 0.13 events/h, p = 0.008. Application of the DCV protocol was not associated with any other adverse events. This study suggests that DCV anesthesia protocol significantly decreases the incidence of severe hypertensive episodes during pheochromocytoma resection. This is the first study that describes a highly effective protocol for controlling hypertension in pheochromocytoma patients.

Recovery of the Insulin Secretory Capacity in a Patient With Diabetic Ketoacidosis after Resection of Asymptomatic Pheochromocytoma: A Case Report

Recovery of the Insulin Secretory Capacity in a Patient With Diabetic Ketoacidosis after Resection of Asymptomatic Pheochromocytoma: A Case Report

Pheochromocytoma associated with primary hyperparathyroidism and type 1 neurofibromatosis

Neurofibromatosis type 1 (NF-1) is a predominantly inherited genetic disease with prevalence of 1 per 2500-3000 newborns and based on clear clinical criteria. In addition to common neurofibromas and gliomas of the visual pathways, these patients have a higher risk of various benign and malignant tumors throughout their lives including tumors of the central nervous system, membranes of peripheral nerves, gastrointestinal stromal tumors and leukemia. Endocrine diseases and neoplasms also occur in patients with NF-1 and can include extrarenal paraganglioma, primary hyperparathyroidism, gastroenteropancreatic neuroendocrine tumors, thyroid tumors and other adrenal neoplasms. We demonstrate multiple neuroendocrine neoplasia (MEN 2A) of neurofibromatosis type 1 combined with pheochromocytoma and primary hyperparathyroidism in a woman with a long history of palpitations, paroxysmal hypertension and osteoporosis. Biochemical analysis revealed severe hypercalcemia with elevated parathyroid hormone indicating primary hyperparathyroidism, as well as high fractionated normetanephrine and metanephrine in the urine as a sign of catecholamine-releasing pheochromocytoma/paraganglioma. Further scintigraphy revealed signs of solitary parathyroid adenoma causing primary hyperparathyroidism and right-sided pheochromocytoma. Clinical diagnosis of MEN-2 syndrome is based on combination of at least two major MEN-2-associated endocrine tumors. Resection of parathyroid adenoma and pheochromocytoma normalized biochemical parameters and blood pressure. Combination of pheochromocytoma with primary hyperparathyroidism and type 1 neurofibromatosis is discussed.

Malignant pheochromocytoma resection with hepatic in-situ cooling and graft-based SMV/CAVAL bypass

Malignant pheochromocytoma resection with hepatic in-situ cooling and graft-based SMV/CAVAL bypass

The evolution of peri-operative care in the safe management of pheochromocytoma

The use of pre- and intra-operative vasoactive agents for improved blood pressure control has transformed the outcomes following pheochromocytoma (PC) resection. The first agent utilized was phenoxybenzamine, but selective alpha-1 antagonists (SAA) and calcium channel antagonists (CCA) have also been used with success. We sought to define the relationship between pre-operative treatment and intra-operative hemodynamics associated with each of these agents in patients with PC. A retrospective, single-institution review of patients >18 years with resected PC from 1996-2020 was performed. Pre-operative blockade was at the discretion of the treating providers. Adequacy of pre-operative blockade was determined by the attending surgeon prior to proceeding with PC resection. To determine the effectiveness of pre-operative blockade during surgery, vital signs throughout the operation were examined. The final dataset included 166 patients. Phenoxybenzamine (55%) and SAA (24%) were the most commonly used pre-operative medications but phenoxybenzamine use decreased over time. Patients who experienced a hypertensive crisis had a higher initial systolic blood pressure and were older. There was no predilection for developing a hypertensive crisis based on race, sex, tumor size or type of pre-operative medication used. Patients in the early group (2010-2015) were found to have more pronounced hemodynamic instability when compared to patients in the late group (2016-2020). Although there are no demonstrated differences between antihypertensives, the prescription pattern has evolved away from phenoxybenzamine. However, as this is transition happening, patients exhibit better hemodynamic control. We hypothesize that this is due to greater experience by the intra-operative team.

Von Hippel–Lindau syndrome with a rare complication of dilated cardiomyopathy: a case report

BackgroundVon Hippel–Lindau (VHL) syndrome is an autosomal dominant hereditary disease affecting multiple organs, with pheochromocytoma in 26% of cases. However, VHL syndrome with congestive heart failure and dilated cardiomyopathy as the primary clinical manifestations has been rarely reported.Case presentationA 35-year-old male patient was admitted to the hospital with dyspnea. The patient had a history of cerebellar hemangioblastoma that had been resected, and a one-year history of hypertension. Echocardiography and cardiac magnetic resonance imaging demonstrated a dilated left ventricle, decreased systolic function, and nonischemic myocardial changes. Contrast-enhanced abdominal computed tomography showed pheochromocytoma, neoplastic lesions, and multiple cysts in the kidneys and pancreas. Genetic analysis revealed a missense mutation of the VHL gene, c.269 A > T (p.Asn90Ile), which was identified as the cause of the disease. Dilated cardiomyopathy and VHL syndrome type 2 were diagnosed. The patient was administered a diuretic, α-blocker, β-blocker, and an angiotensin receptor neprilysin inhibitor (ARNI), but refused pheochromocytoma resection. At the six-month follow-up, the patient was asymptomatic with improved cardiac function.ConclusionCardiac involvement is an atypical manifestation in VHL syndrome. Early diagnosis with genetic screening is essential for avoiding life-threatening complications associated with VHL. The management of this rare manifestation of VHL syndrome requires further investigation.

Risk factors for hemodynamic instability during laparoscopic resection of pheochromocytoma

BackgroundLaparoscopic adrenalectomy for pheochromocytoma is associated with high risk of intraoperative hemodynamic instability. Our study aimed to identify predictive factors for hemodynamic instability during laparoscopic resection of pheochromocytoma.MethodsBetween January 2011 and December 2021, 136 patients underwent unilateral laparoscopic adrenalectomy for pheochromocytoma. The patients were divided into 2 groups depending on the presence or absence of hemodynamic instability during surgery. Intraoperative hemodynamic parameters were compared between the 2 groups. Patient demographic characteristics and preoperative evaluations were assessed for their prognostic relevance with respect to intraoperative hemodynamic instability via both univariate analysis and multivariate logistic regression analysis.ResultsThere was greater blood pressure fluctuations and higher maximum blood pressure and heart rate in the hemodynamic instability group. More patients need intraoperative administration of vasoactive drugs in the hemodynamic instability group. In the univariate analysis, presence of coronary artery disease, tumour size, and previous hypertension history were significantly associated with intraoperative hemodynamic instability. The multivariate logistic regression analysis showed that tumour size and previous hypertension history were independent risk factors for intraoperative hemodynamic instability.ConclusionTumour size and previous hypertension history were associated with hemodynamic instability during laparoscopic resection of pheochromocytoma.

Phenoxybenzamine is no longer the standard agent used for alpha blockade before adrenalectomy for pheochromocytoma: A national study of 552 patients

BackgroundPhenoxybenzamine has been the standard agent for blockade before adrenalectomy for pheochromocytoma. However, high cost and limited availability have hampered its use. This study investigated whether other agents have supplanted the use of phenoxybenzamine as the first-line agent for alpha blockade in pheochromocytoma. MethodsWe performed a retrospective analysis of patients in the IBM MarketScan Database who underwent adrenalectomy for pheochromocytoma (2008–2019). Patients were categorized as having been blocked with phenoxybenzamine, selective alpha blockers, calcium channel blockers and/or beta blockers, or none of the above. The outcomes included prescription costs, perioperative costs, and length of stay. ResultsA total of 552 patients were identified; 58.7% were female, and the median age was 49 (interquartile range 40–57) years. In total, 291 (52.7%) patients were blocked with phenoxybenzamine, 114 (20.7%) with selective alpha blockers, 42 (7.6%) with only calcium channel blockers and/or beta blockers, and 76 (13.8%) with none. The proportion of patients blocked with phenoxybenzamine decreased from 71.0% in 2008 to 21.2% in 2019. The proportion of patients blocked with selective alpha blockers increased from 6.5% in 2008 to 42.4% and in 2019. The median cost of phenoxybenzamine increased from $722 (interquartile range $441–$1,514) in 2008 to $9,616 (interquartile range $5,049–$16,373) in 2019 (P < .001). Length of stay (2 [interquartile range 1–4] days vs 2 [interquartile range 0–3] days) and total perioperative costs ($24,250 [interquartile range $17,462–$33,849] vs $22,098 [interquartile range $16,341–$29,178] between phenoxybenzamine and selective alpha blocker groups were similar. ConclusionThere has been a significant shift away from phenoxybenzamine for preoperative blockade before resection of pheochromocytoma. Selective alpha blockers and calcium channel blockers are increasingly used, likely due to reduced costs, without compromised length of stay or intensive care unit admission.