Surgery For Pheochromocytoma Research Articles

Circulatory characteristics of early mobilization after surgery for bilateral pheochromocytoma: a case report

BackgroundSurgical 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 presentationA 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.ConclusionCirculatory 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.

Robot-Assisted Surgery and Multigene Panel Testing for Pheochromocytoma and Paraganglioma Syndrome

Robot-Assisted Surgery and Multigene Panel Testing for Pheochromocytoma and Paraganglioma Syndrome

The frequency of postoperative hypoglycemia after pheochromocytoma surgery is decreasing.

Hypoglycemia after pheochromocytoma resection is one of the most common postoperative complications, with a reported incidence of 12%-43%. In recent years, we have rarely experienced postoperative hypoglycemia after pheochromocytoma surgery at our institution. We reviewed our own experience and examined factors associated with postoperative hypoglycemia in pheochromocytoma patients. We collected and retrospectively reviewed medical information from 53 patients with pheochromocytoma who underwent initial surgery in our department between 1996 and 2022, who did not receive steroids in the perioperative period and received the same alpha-blocker preoperatively. Subjects were divided into two groups by the midpoint of the study period: Group 1 (G1), 1996-2009; and Group 2 (G2), 2010-2022. The two groups were compared. Hypoglycemia occurred significantly less often in G2 (0 patients, 0%) than in G1 (7 patients, 28%; p=0.003). Preoperative diabetes was significantly less frequent in G2 (2 patient, 7.1%) than in G1 (8 patients, 32%; p=0.03). Preoperative alpha-blocker dosage was significantly higher in G2 than in G1 (p=0.04). Multivariate logistic regression analysis showed that only alpha-blockers dosage was significantly associated with the occurrence of postoperative hypoglycemia (p=0.004). The current study suggest that the alpha-blocker dosage might be related to the lower incidence of postoperative hypoglycemia in Pheochromocytoma patients.

Preoperative 3D Planning and Intraoperative Navigation in Robot-Assisted Laparoscopic Surgery for Complex Pheochromocytoma.

To evaluate the value of preoperative 3D planning and intraoperative navigation in robot-assisted laparoscopic surgery for complex pheochromocytoma. Observational study. Place and Duration of the Study: Department of Urology, The Affiliated Hospital of Hebei University, from July 2022 to July 2023. 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. 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). 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. 3D planning and navigation, Robot-assisted surgery, Complex pheochromocytoma.

Evolution of metabolic disorders after resection of pheochromocytomas and paragangliomas: a single-center study

Background and aims. Pheochromocytomas and paragangliomas are rare neuroendocrine tumors, responsible for inappropriate secretion of catecholamines, inducing metabolic disorders, increasing basal metabolic rate. Our study aimed to analyze the metabolic profile pre- and post-operatively in patients undergoing surgery for pheochromocytomas and paragangliomas and additionally to determine the predictive factors of metabolic remission. Methods. This was a retrospective, unicentric, descriptive, and analytical study with a duration of 9 years. It includes data from 35 patients followed up for pheochromocytoma or paraganglioma in the Endocrinology-Diabetology and Nutrition Department of a University Hospital Center. All patients underwent surgery for pheochromocytoma or paraganglioma. Results. Among the 35 patients, 51.4% of the patients with pheochromocytomas had diabetes mellitus, and 42.8% had dyslipidemia. We found that high levels of catecholamines were associated with the onset of diabetes. We also noted that patients with a long history of the disease were statistically at greater risk of developing dyslipidemia. After surgery, the body mass index of our patients had statistically increased, and 50% of patients experienced resolution or improvement of their diabetes. Improvement of dyslipidemia was observed in 53% of patients. We also found that the percentage of dyslipidemia was higher in patients who did not resolve their diabetes. Conclusion. Diabetes mellitus and dyslipidemia are metabolic complications that must be investigated in patients with pheochromocytoma. Post-operative monitoring of body mass index and changes in glycemic and lipid levels is essential to adapt therapeutic management.

The Impact of the COVID Pandemic on Adrenal Surgery at an Academic Endocrine Surgery Unit.

COVID-19 has severely affected the delivery of surgical care worldwide. The aim of the present study was to evaluate its impact on adrenal surgery at our academic endocrine center. All primary adrenal surgeries performed at the University Hospital of Cologne, Germany between 01.01.2019 and 31.07.2022 were included. This time frame was divided into pre-Covid (before 02/20), acute Covid (until 05/21), and post acute period (after 05/2021). Demographics, clinic-pathologic characteristics and treatment of these patients were analyzed. One hundred adrenalectomies were included: 22 before, 30 during, and 48 after the acute phase. The percentage of Conn adenomas and pheochromocytomas decreased during the acute phase (from 45.4 to 26.6% and from 18 to 10%, respectively) in favor of Cushing adenomas and suspicious tumors (from 4.5 to 20% and from 31.8 to 36.6%). About 90.9% of tumors resected for suspicion of malignancy were confirmed malignant by final histopathology, as opposed to 71.4% and 52.6% before and after the acute phase. The operative technique was similar during the three phases (63% retroperitoneoscopic, 34% laparoscopic and 2% open resections), with a significantly shorter operative time for retroperitoneoscopy (p=0.04). ICU monitoring demand increased during the acute phase (from 13.6% to 43.3%), according to the increase in Cushing adenomas and malignant tumors. During the acute phase of COVID-19 pandemic adrenal surgery for Cushing and malignant tumors increased, while a delay in pheochromocytoma surgery to the post acute phase was observed. The suspicion of malignancy formulated by the endocrine tumor board was accurate in 90.9% of cases.

Anaesthetic Management for Pheochromocytoma Surgery: A Never Ending Challenge

Pheochromocytoma is a challenging case for any anesthesiologist and a team approach is required throughout the perioperative period to prevent anticipated difficulties and best manage any unforeseen complications that develop. We discuss successful management of certain unique challenges, like multifocal arrhythmias and surgical rebleed during transport, during pheochromocytoma surgery in an adult, in addition to the usual perioperative problems.

Modified 30-Degree Head-Up Tilt Park Bench Position in Semielective Posterior Fossa Surgery in a Patient with Pheochromocytoma

AbstractVon Hippel–Lindau (VHL) disease is a rare genetic disorder associated with the central nervous system and visceral organs. Pheochromocytomas occur in 10% of VHL patients, while cerebellar tumors are common tumors in VHL syndrome, with an incidence of 60%. The most common position for posterior fossa operations is the park bench or lateral decubitus position. These positions have primarily replaced the sitting position. However, the advantages of the supine position cannot be overlooked. The coexistence of pheochromocytoma and the cerebellar tumor may require modification in surgical position and anesthesia management in line with possible pathophysiological changes. We present the anesthesia management in posterior fossa surgery in patients with postponed pheochromocytoma surgery. The present case highlights the importance of a multidisciplinary team approach and anesthetic management.

Changes in blood pressure, blood sugar and creatinine in patients undergoing pheochromocytoma surgery

Objective: To discover the variables that may predict changes in blood pressure and blood sugar levels and to pinpoint the time frame during which such changes occur in patient with pheochromocytoma. Materials and Methodology: Consecutive patients undergoing surgery for pheochromocytoma over a 20-month period were included in this ethics review board-approved, prospective cohort study. Blood pressure and sugar levels were serially monitored using a fixed protocol in the perioperative period and subsequently at 3 months after surgery. Changes were compared and assessed for the predictive factors. Result: Fifty patients undergoing surgical procedures were included in the study of whom 32% developed hypotension and 10% developed hypoglycaemias after surgery. All hypotension episodes occurred within 6 hours of surgery. However, while 7 of 8 patients who developed hypoglycaemia manifest in the first 4 h after surgery, one occurred after 12 h. Occurrence of hypotension correlated with preoperative 24-h urinary vanillylmandelic acid levels (P = 0.024). Out of 21 hypertensive patients, 15 had persistent hypertension at 3 months and this was associated with age (P = 0.04) and diabetes mellitus at presentation. Conclusion: 32% of patients receiving pheochromocytoma surgery experienced postoperative hypotension needing inotropic support. Patients who require greater dosages of alpha-blockers or those with higher 24-h urine VMA levels are more prone to experience this. This happens within the first six hours following surgery. Patients who are older or who have had DM for a long time are more likely to suffer chronic HTN following surgery. Keywords: Pheochromocytoma, hypertension, hypoglycaemia, creatinine.

Change in Perioperative Management of 14 Consecutive Patients with Pheochromocytoma – Is Alpha Blockade Still Necessary?

Abstract Background Pretreatment with alpha-blockers before surgery for pheochromocytoma or paraganglioma is considered the gold standard and is recommended in the 2014 Endocrine Consensus Guidelines. Meanwhile, there is evidence at specialized clinics that alpha blockade does not provide any benefit preoperatively. Due to an index case with severe side effects from alpha blockade and the recent evidence, we have refrained from preoperative alpha blockade before planned surgery for a catecholamine-secreting tumor in our hospital since 2018. Aims The aim of the study is to evaluate the outcome after change of perioperative management. Methods A retrospective analysis of patients was performed using data prospectively collected in the Eurocrine quality registry of all patients with pheochromocytomas and paragangliomas operated on at our hospital from 2018 to 2022. Results During this period, 14 patients with pheochromocytomas (n=10) and paragangliomas (n=4) underwent surgery. 21% (3 of 14) patients were pretreated by alpha blockers for 7-14 days, 79% (11/14) patients did not receive preoperative alpha blockade after interdisciplinary discussion. All patients with alpha blockade had significant orthostasis symptoms, and 2 patients experienced syncope despite adequate volume delivery. One of these patients suffered a traumatic brain injury after a fall. Intraoperatively, 50% (7 of 14) of the patients experienced a hypertensive crisis &amp;gt;200 mmHg, which was resolved with rapid anesthetic intervention. The 3 patients with alpha blockade had prolonged hypotension postoperatively, so catecholamines had to be administered after extubation in 2 patients. No relevant complications occurred. Conclusions In our hospital, no relevant complications occurred despite omission of alpha blockade for pretreatment in pheochromocytomas and paragangliomas. However, the number of cases in these rare tumors is low, nevertheless this seems to be feasible in a good interdisciplinary setting.

Long-Term Outcomes after Surgery for Pheochromocytoma and Sympathetic Paraganglioma.

The prognosis of pheochromocytoma and sympathetic paraganglioma (PHEO/sPGL) is difficult to predict at the time of diagnosis and long-term follow-up data are scarce, especially for apparently benign and sporadic variants. The aim of the study was to analyze the long-term outcomes in PHEO/sPGL patients. A monocentric series of 170 patients who underwent surgery for PHEO/sPGL was analyzed. The study cohort included 91 female and 79 males with a median age of 48 years (range 6-83). The majority of PHEO/sPGL cases were considered apparently benign at the time of diagnosis; evident malignant behavior was found in 5% of cases. The overall 10-year risk of recurrence was 13%, but it rose up to 33% at 30 years. The risk of new tumor recurrence was higher in patients with hereditary tumors, but the risk was still significant in patients with apparently sporadic variants (20-year risk: 38% vs. 6.5%, respectively; p < 0.0001). The risk of metastatic recurrence was higher in patients with locally aggressive tumors at diagnosis, but the risk was present also in apparently benign variants (5-year risk: 100% vs. 1%, respectively; p < 0.0001). Lifelong follow-up is required not only for hereditary PHEO/sPGL but also for apparently benign and sporadic tumors at diagnosis because of the risk of long-term recurrent disease.

A-240 PHEOCHROMOCYTOMATOSIS – A RARE DISEASE WITH OMINOUS PROGNOSIS

Abstract Background Pheochromocytomatosis is a rare but dramatic complication of pheochromocytoma surgery. In this study, we collected and analysed our experience with the surgical treatment of cases of this rare condition. Methods In total 9 patients were operated on between December 2001 and August 2022 at the Kliniken Essen-Mitte due to pheochromocytomatosis. There were 5 female and 4 male patients, mean age of 58 ± 18 (range: 22 to 86). The data was prospectively collected and retrospectively analysed. Results All patients were initially operated on due to pheochromocytoma (2 with SDHB-Syndrome and 1 with MEN-IIa-Syndrome). In 4 cases an intraoperative tumour capsule injury during the initial surgery was described. The operation due to pheochromocytomatosis was performed minimally invasive in 8 cases, 1 patient was operated on by open approach. The mean time between the primary surgery and the pheochromocytomatosis operation was 107 ± 60 months. Operation time was 246 + 119 minutes. Follow-up data were available for 8 patients (mean follow-up time 12 years). 2 patients were cured at the time of the last follow-up. 7 patients developed recurrent disease: loco-regional recurrence in 6 cases and distant metastasis in 3 cases. 4 patients with loco-regional recurrence were reoperated within 54 ± 50 months (range 10–137 months). By the follow-up, 3 patients died. Conclusion Pheochromocytomatosis is a potentially lethal complication of pheochromocytoma surgery. Even if the initial pheochomocytomatosis could be managed minimally invasive, the recurrence rate is extremely high.

Risk factors for postoperative complications after pheochromocytoma and/or paraganglioma: a single-center retrospective study.

Perioperative complications are higher in patients with pheochromocytoma and/or paraganglioma. The aim of this study was to identify the risk factors of postoperative complications after pheochromocytoma and/or paraganglioma resection surgery. We retrospectively reviewed 438 patients who underwent laparoscopic or open surgery for pheochromocytoma and/or paraganglioma at our center between January 2014 and December 2019. Demographic characteristics and intraoperative and postoperative data were recorded. Complications were defined as deviations from the normal postoperative course and Clavien-Dindo classification was used to grade the severity of postoperative complication. Patients with complications of grade II or above were included for analysis. Binary logistic regression was used to determine the risk factors for postoperative complications. The median age of the patients was 47 years old. There were 295 cases (67.4%) of phepchromocytoma and 143 cases (32.6%) of paraganglioma. Three hundred sixty-seven (87.8%) patients had laparoscopic approach, and 55 (12.6%) patients had laparotomy; the conversion rate from laparoscopy to laparotomy was 3.7%. Eighty-seven complications occurred in 65 patients (14.8%). No death occurred in our study and transfusion (36, 8.2%) was the most common complication. The mean follow-up length was 14 months. Independent risk factors for postoperative complications included a tumor size larger than 5.6cm (OR = 2.427, 95% CI 1.284-4.587, P = 0.006), laparotomy (OR 2.590, 95% CI 1.230-5.453, P = 0.012), conversion to laparotomy (OR = 8.384, 95% CI 2.247-31.285, P = 0.002), and the operation time longer than 188min (OR = 3.709, 95% CI 1.847-7.450, P < 0.001). Complications were not uncommon after pheochromocytoma and/or paraganglioma surgery. Tumor size, surgical type, and operation time were determined as the risk factors for postoperative complications. These factors should be considered to improve perioperative management.

Pathophysiology and Management of Glycemic Alterations before and after Surgery for Pheochromocytoma and Paraganglioma

Glycemic alterations are frequent in patients with pheochromocytoma and paraganglioma (PPGL), but the real incidence of secondary diabetes mellitus (DM) is uncertain, because prospective multicenter studies on this topic are lacking in the literature. The main pathophysiological mechanisms of glucose homeostasis alterations in PPGL, related to catecholamine hypersecretion, are impaired insulin and glucagon-like peptide type 1 (GLP-1) secretion and increased insulin resistance. Moreover, it has been reported that different pathways leading to glucose intolerance may be related to the secretory phenotype of the chromaffin tumor. Predictive factors for the development of glucose intolerance in PPGL patients are a higher age at diagnosis, the need for a higher number of anti-hypertensive drugs, and the presence of secreting neoplasms. Tumor resection is strongly related to the resolution of DM in PPGL patients, with a significant improvement of glycemic control in most cases. We can hypothesize a different personalized therapeutic approach based on the secretory phenotype. The adrenergic phenotype is more closely related to reduced insulin secretion, so insulin therapy may be required. On the other hand, the noradrenergic phenotype mainly acts by increasing insulin resistance and, therefore, insulin-sensitizing antidiabetic agents can find a greater application. Regarding GLP-1 receptor agonists, the data suggest a possible promising therapeutic effect, based on the assumption that GLP-1 secretion is impaired in patients with PPGL. The principal predictors of remission of glycemic alterations after surgery for PPGL are a lower preoperative body mass index (BMI), a larger tumor, higher preoperative catecholamine levels, and a shorter duration of the disease (under three years). Otherwise, after resection of PPGL, hypoglycemia can occur as the result of an excessive rebound of preoperative hyperinsulinemia. It is a rare, but potentially severe complication reported in a lot of case reports and a few small retrospective studies. Higher 24-h urinary metanephrine levels, longer operative times and larger tumors are predictive factors for hypoglycemia in this setting. In conclusion, alterations of carbohydrate metabolism are clinically relevant manifestations of PPGL before and after surgery, but there is the need to conduct multicenter prospective studies to obtain an adequate sample size, and to allow the creation of shared strategies for the clinical management of these potentially severe manifestations of PPGL.

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.

Perioperative hemodynamic management in surgery for phaeochromocytoma: A narrative review

Pheochromocytomas are rare adrenal tumors that produce excessive catecholamines and their surgical removal poses significant risks of intraoperative hemodynamic instability (HI) due to catecholamine release. This review article discusses the perioperative factors that impact hemodynamic lability and its management in patients undergoing surgical removal. A literature review was conducted by searching the electronic databases - Ovid MEDLINE, Embase, and Cochrane Library using appropriate Medical Subject Heading terms and keywords such as phaeochromocytoma, HI, and perioperative hemodynamic monitoring. The advancements in surgical and anesthetic techniques and appropriate preoperative medical optimization have contributed to a significant decrease in mortality rates. However, perioperative HI remains the biggest surgical and anesthetic challenge in treating pheochromocytomas. Patients with larger and more hormonally active tumors are at higher risk for extreme hypertensive episodes during surgery. Preoperative α-blockade, higher phenoxybenzamine doses, and laparoscopic approach improve the outcomes in phaeochromocytoma removal. Anesthetic techniques and drugs used during surgery may help prevent HI, but careful intraoperative management is essential. Perioperative HI can lead to increased surgical blood loss, patient morbidity, and prolonged intensive care unit (ICU) and hospital stay. Therefore, a multidisciplinary approach involving the surgeon, anesthesiologist, and ICU team is essential to ensure optimal perioperative management of patients with pheochromocytoma. Intensive hemodynamic monitoring may be required in the postoperative period to manage hypotension seen after tumor removal. In conclusion, perioperative HI is a significant risk during the surgical removal of pheochromocytomas, even with preoperative pharmacological treatment. Therefore, the use of appropriate preoperative medical optimization, surgical and anesthetic techniques, and careful intraoperative management can significantly improve the outcomes.

PS-BPR01-8: A CASE OF PHEOCHROMOCYTOMA WITH CONCOMITANT SPLENIC ARTERY ANEURYSM

A 62-year-old man with a 30-year history of headaches and a 20-year history of treatment-resistant hypertension and diabetes mellitus was referred to our hospital for evaluation of his 5 cm retroperitoneal tumor found by abdominal ultrasound examination at a medical checkup. A simple CT and MRI scan showed a heterogeneous tumor (diameter, 5 cm) in the left adrenal gland and a splenic artery aneurysm (diameter, 2 cm). Endocrine examinations revealed increased levels of plasma-free normetanephrine (NMN) (853 pg/mL), urinary noradrenaline (NA) (670 μg/day), and urinary NMN (2.6 mg/day). 123I-MIBG scintigraphy showed high accumulation in the left adrenal region. He was diagnosed with NA-dominant left pheochromocytoma. His blood pressure was controlled at around 140/90 mmHg with 16 mg of doxazosin and 10 mg of amlodipine. His insulin-dependent diabetes was treated with multiple daily insulin injections (63 units/day), metformin, and miglitol. First, he underwent splenic artery aneurysm embolization under general anesthesia, which was completed without developing a hypertensive crisis. Two months later, he underwent laparoscopic left adrenalectomy, and the resected tumor was pathologically diagnosed as pheochromocytoma with a GAPP score of 1. He did not develop postoperative hypotension. His blood pressure was well controlled with only 10 mg of amlodipine, and his insulin requirement was reduced to 23 units/day after surgery. Since few cases of pheochromocytoma with concomitant splenic artery aneurysm have been reported, we discussed the treatment plan with much consideration. Splenic artery aneurysms have a high risk of rupture due to intraoperative blood pressure elevation in pheochromocytoma, which could be fatal. Although the first choice of treatment for a splenic artery aneurysm is endovascular therapy, the use of contrast media is generally contraindicated in pheochromocytoma in Japan due to the risk of hypertensive crisis. In the present case, systemic management with a sufficiently high dose of alpha-blocker allowed the embolization to be preceded avoiding a crisis, and subsequent surgery for pheochromocytoma could be performed safely. A long history of hypertension and diabetes exacerbated by the pheochromocytoma may have contributed to the arteriosclerosis leading to the splenic artery aneurysm. Here we report a rare case of pheochromocytoma with concomitant splenic artery aneurysm and will discuss the pathogenesis and management of this case with a review of the literature.

A MEN-2A Case Developing Transient Adrenal Insufficiency after Unilateral Pheochromocytoma Surgery

A MEN-2A Case Developing Transient Adrenal Insufficiency after Unilateral Pheochromocytoma Surgery

Hemodynamic Instability during Surgery for Pheochromocytoma: A Retrospective Cohort Analysis.

Perioperative hemodynamic instability is one of the most common adverse events in patients undergoing adrenalectomy for pheochromocytoma. The aim of this study was to analyze the impact of perioperative severe hemodynamic instability. We present a retrospective, single-center analysis in a major tertiary hospital of all consecutive patients undergoing elective adrenalectomy from 2005 to 2019 for pheochromocytoma. Severe perioperative hypertension and hypotension were evaluated, defined as changes in blood pressure larger than 30% of the preoperative patient-specific mean arterial pressure (MAP). Unilateral adrenalectomy was performed in 67 patients. Intraoperative episodes of hemodynamic instability occurred in 97% of all patients (n = 65), severe hypertension occurred in 24 patients (36%), and severe hypotensive episodes occurred in 62 patients (93%). Patients with more than five severe hypotensive episodes (n = 29) received higher preoperative alpha-adrenergic blockades (phenoxybenzamine 51 ± 50 mg d-1 vs. 29 ± 27 mg d-1; p = 0.023) and had a longer mean ICU stay (39.6 ± 41.5 h vs. 20.6 ± 19.1 h, p = 0.015). Intraoperative hypotensive, rather than hypertensive, episodes occurred during adrenalectomy. The occurrence of more than five hypotensive episodes correlated well with a significantly longer hospital stay and ICU time.

Metanephrine and normetanephrine associated with subclinical myocardial injuries in pheochromocytoma and paraganglioma.

ObjectiveTo analyze the correlation between metanephrine and normetanephrines (MNs) and subclinical myocardial injuries (SMI) diagnosed by low left ventricular global longitudinal strain (LV GLS) in patients with pheochromocytoma and paraganglioma (PPGL).MethodsSeventy-six patients who underwent surgery for pheochromocytoma or paraganglioma from September 2017 to April 2022 were examined. All the patients enrolled had normal left ventricular ejection fraction (LVEF) and myocardial injury biomarkers including cardiac troponin I and B-natriuretic peptide. Univariate analysis and multivariate analysis were performed to evaluate the association of MNs and subclinical myocardial injury (SMI)(defined as LV GLS<18).ResultsLV GLS of 13(17.11%) PPGL patients was less than 18. The percentage of patients with elevation of single hormone (metanephrine, normetanephrine, 3-methoxytyramine) or any one of MNs was not significantly correlated with SMI (P=0.987, 0.666, 0.128 and 0.918, respectively). All MNs elevation was associated with SMI (OR: 11.27; 95% CI, 0.94—135.24; P= 0.056). After adjusting for age, All MNs elevation was significantly correlated with SMI (OR: 16.54; 95% CI, 1.22—223.62; P= 0.035).ConclusionMNs might be an important factor influencing myocardial function. All MNs elevation might indicate SMI. If all MNs elevated, LV GLS measurement was recommended for PPGL patients to detect SMI in the absence of decrease LVEF or other heart disease in clinical practice.