Management Of Pheochromocytoma Research Articles

Variability in perioperative management of pheochromocytoma in Canada

Variability in perioperative management of pheochromocytoma in Canada

Maternal pheochromocytoma and childbirth in Sweden 1973-2015: a population-based study on short and long-term outcome.

Data guiding management of pheochromocytoma and paraganglioma (PPGL) in pregnant women is limited, and long-term effects on the child are unknown. The aim of this retrospective registry-based case-cohort study was to assess how maternal PPGL and treatment impacts maternal and fetal outcome, including long-term outcome for the child. The main outcomes were maternal and fetal mortality and morbidity at delivery and relative healthcare consumption in children born by mothers with PPGL during pregnancy. The National Birth Register identified 4,390,869 pregnancies between 1973-2015. Data was crosslinked with three Swedish national registers to identify women diagnosed with pheochromocytoma or paraganglioma within one year before or after childbirth. Hospital records were reviewed and register data was collected for five age-matched controls for each child until age 18. 21 women and 23 children were identified (incidence 4.8/1.000.000 births/year), all women with adrenal pheochromocytomas (Pc). The majority (71%) were diagnosed post-partum. Nine women (43%) were hypertensive during pregnancy. Preterm delivery was more common in Pc patients compared to controls (30% vs 6%, p < 0.001). There was no maternal or fetal mortality. Timing of tumor removal did not affect gestational weight or APGAR scores. There was no observed difference in hospital admissions between children affected by maternal Pc and controls. Pc was commonly diagnosed after delivery and raised the risk of pre-term delivery, suggesting a need for an increased awareness of this diagnosis. However, reassuringly, there was no fetal or maternal mortality or any observed long-term impact on the children.

A Narrative Review of Pheochromocytoma in VHL.

This systematic review aims to investigate the clinical presentation, diagnostic methods, and management strategies for pheochromocytoma in patients with von Hippel-Lindau (VHL) disease, an autosomal dominant disorder that predisposes individuals to the development of various tumors, including pheochromocytomas. Pheochromocytoma is a rare neuroendocrine tumor of the adrenal medulla that occurs sporadically or as part of an inherited syndrome. The incidence of pheochromocytoma in VHL patients is estimated to be between 10-20%, making it the second most common tumor associated with VHL. Early detection and management of pheochromocytoma in VHL patients are critical for patient outcomes, as these tumors can cause severe hypertension, cardiovascular complications, and death. This review highlights the importance of screening for pheochromocytoma in VHL patients and discusses the current diagnostic and management strategies to optimize patient care.

Management of phaeochromocytoma and paraganglioma in patients with germline SDHB pathogenic variants: an international expert Consensus statement.

Adult and paediatric patients with pathogenic variants in the gene encoding succinate dehydrogenase (SDH) subunit B (SDHB) often have locally aggressive, recurrent or metastatic phaeochromocytomas and paragangliomas (PPGLs). Furthermore, SDHB PPGLs have the highest rates of disease-specific morbidity and mortality compared with other hereditary PPGLs. PPGLs with SDHB pathogenic variants are often less differentiated and do not produce substantial amounts of catecholamines (in some patients, they produce only dopamine) compared with other hereditary subtypes, which enables these tumours to grow subclinically for a long time. In addition, SDHB pathogenic variants support tumour growth through high levels of the oncometabolite succinate and other mechanisms related to cancer initiation and progression. As a result, pseudohypoxia and upregulation of genes related to the hypoxia signalling pathway occur, promoting the growth, migration, invasiveness and metastasis of cancer cells. These factors, along with a high rate of metastasis, support early surgical intervention and total resection of PPGLs, regardless of the tumour size. The treatment of metastases is challenging and relies on either local or systemic therapies, or sometimes both. This Consensus statement should help guide clinicians in the diagnosis and management of patients with SDHB PPGLs.

Advances in management of pheochromocytoma – a short review

Advances in management of pheochromocytoma – a short review

FRI207 Challenges Of Pheochromocytoma Management In A Heart Failure Patient

Abstract Disclosure: M. Novitskaya: None. M. Sonbol: None. S. Jafri: None. J. Monye: None. E. Japp: None. Introduction: Successful management of pheochromocytoma in patients with heart failure requires a careful balance between blood pressure control and appropriate perioperative volume resuscitation. Clinical Case: A 38-year-old male with heart failure with reduced ejection fraction (EF 15-20%), HTN, and polysubstance use disorder presented with dyspnea and subsequent in-house PEA arrest. Whole body CT showed an incidental 7.3 cm left adrenal mass. Laboratory work-up was notable for elevated plasma free metanephrines 47.30 nmol/L (n&amp;lt;0.89) and normetanephrines 39.8 nmol/L (n&amp;lt;0.49). Medical management of presumed pheochromocytoma was initiated with doxazosin. Left and right heart catheterization showed elevated intracardiac filling pressures (mmHg): RA 10, RV 66/5 (12), PA 55/29 (43), PCWP 35. Subsequent coronary angiogram was aborted due to hypertensive emergency and flash pulmonary edema for which he required IV hydralazine 20 mg, furosemide 80 mg, labetalol 30 mg, verapamil 5 mg, nitroglycerin 800 mg bolus then gtt at 100 mcg/min to achieve hemodynamic stability. He reached the preoperative BP goal after up titration of doxazosin to 12 mg daily and carvedilol to 25 mg twice daily. He subsequently underwent left adrenalectomy. Intraoperatively, the patient’s abdomen was insufflated to 13 mmHg given his reduced EF, and he received 2 L crystalloid prior to adrenal vein division. After a left adrenalectomy, he developed hypotension despite receiving an additional 3 L crystalloid, and was started on norepinephrine, epinephrine, and vasopressin gtt. Postoperatively, he remained intubated for two days due to distributive shock. With continued diuresis, mean arterial pressure recovered to &amp;gt;65 mmHg and he was extubated. He was successfully discharged on metoprolol succinate 25 mg, spironolactone 25 mg and losartan 25 mg daily. Clinical Lessons: Preoperative management of pheochromocytoma involves alpha-adrenergic blockade followed by beta-adrenergic blockade to target a BP of &amp;lt;130/80. A high sodium diet is recommended after adequate alpha-adrenergic blockade, and volume resuscitation is started preoperatively due to vasodilation after adrenalectomy. Successful management of patients with pheochromocytoma and heart failure requires extra attention to BP and volume status. Our patient’s case was complicated by severe HTN during cardiac catheterization resulting in flash pulmonary edema, and postoperative hypotension requiring vasopressor support due to loss of circulating catecholamines. His estimated risk of morbidity/mortality from adrenalectomy was &amp;gt;25% due to these factors. Multidisciplinary collaboration among endocrinologists, cardiologists, anesthesiologists, surgeons, and perfusionists is crucial to optimize patient outcomes. This patient case adds to the literature in presenting a positive adrenalectomy outcome in a patient with heart failure. Presentation: Friday, June 16, 2023

FRI264 A Case Of Composite Pheochromocytoma And Ganglioneuroma

Abstract Disclosure: D. He: None. J. Pullman: None. S. Stefan: None. B.Y. Wong: None. Background: Pheochromocytomas are catecholamine-secreting tumors that arise from chromaffin cells in the adrenal medulla or paraganglia. Representing 1-9% of pheochromocytomas, composite pheochromocytomas are rare tumors composed of pheochromocytoma and neural tumor components such as neuroblastoma, ganglioneuroblastoma, ganglioneuroma or peripheral nerve sheath tumor1. We present a case of composite pheochromocytoma and ganglioneuroma. Clinical Case: A 69-year-old man presented with recurrent dyspnea at rest, diaphoresis and tachycardia. He was diagnosed with heart failure with reduced ejection fraction and management included metoprolol 50 mg daily. On work-up, he was incidentally found to have a 1.2 cm left upper lobe lung lesion and an indeterminate 2.4 cm right adrenal mass on CTA chest. PET/CT scan showed an intensely avid left upper lobe lung nodule and a right adrenal mass with mild FDG uptake (SUV max 3.8). MRI abdomen demonstrated a 3.1 cm right adrenal lesion with a mildly thickened enhancing rim and a central enhancing nodular component suspicious for pheochromocytoma, metastases, schwannoma or ganglioneuroma. He then had a stroke with mild residual right-sided weakness. Oncological work-up of his lung and adrenal lesions eventually directed tissue sampling to biopsy of the adrenal lesion and pathology was consistent with pheochromocytoma. Subsequent biochemical evaluation revealed elevated plasma metanephrines of 194 pg/mL (&amp;lt;= 57 pg/mL), plasma normetanephrines of 474 pg/mL (&amp;lt;= 148 pg/mL), 24-hour urine metanephrines of 389 mcg (90-315 mcg) and 24-hour urine normetanephrines of 428 mcg (122-676 mcg). Pre-operatively, he was placed on phenoxybenazmine 10 mg twice daily and metoprolol 62.5 mg every 8 hours. The patient then underwent right adrenalectomy. The tumor nodule was adherent to and dissected off the inferior vena cava. Pathology showed composite pheochromocytoma with a small component of ganglioneuroma. Conclusion: This rare case of a composite pheochromocytoma with ganglioneuroma was diagnosed through histopathology as composite pheochromocytomas are clinically and radiographically indistinguishable from pheochromocytomas1. Management of composite pheochromocytomas with ganglioneuroma is similar to the management of pheochromocytomas, and surgical resection remains the first line treatment. Combined alpha and beta-adrenergic blockade is recommended to prevent intraoperative hypertensive crisis. However, more studies are needed to further understand these rare composite pheochromocytomas.

Biochemical analysis and management of phaeochromocytoma: A case report

Phaeochromocytoma is a tumour from chromaffin cells in adrenal medulla or extra-adrenal autonomic ganglia, usually releasing catecholamines and metanephrines. It is an unusual cause of severe hypertension in the young. This is the first report from our hospital, the Federal Medical Centre, Umuahia. There are important steps in management which if unattended to results in high peri-operative mortality. This article highlight these issues in its management.

Management of pheochromocytomas and paragangliomas: Review of current diagnosis and treatment options.

Pheochromocytomas (PCCs) are rare neuroendocrine tumors derived from the chromaffin cells of the adrenal medulla. When these tumors have an extra-adrenal location, they are called paragangliomas (PGLs) and arise from sympathetic and parasympathetic ganglia, particularly of the para-aortic location. Up to 25% of PCCs/PGLs are associated with inherited genetic disorders. The majority of PCCs/PGLs exhibit indolent behavior. However, according to their affiliation to molecular clusters based on underlying genetic aberrations, their tumorigenesis, location, clinical symptomatology, and potential to metastasize are heterogenous. Thus, PCCs/PGLs are often associated with diagnostic difficulties. In recent years, extensive research revealed a broad genetic background and multiple signaling pathways leading to tumor development. Along with this, the diagnostic and therapeutic options were also expanded. In this review, we focus on the current knowledge and recent advancements in the diagnosis and treatment of PCCs/PGLs with respect to the underlying gene alterations while also discussing future perspectives in this field.

Preoperative management of Pheochromocytoma with fluctuation of Blood Pressure: A case report

Searchable abstracts of presentations at key conferences in endocrinology ISSN 1470-3947 (print) | ISSN 1479-6848 (online)

Abstract #1396724: Cryptic Catecholamines

The textbook presentation of a pheochromocytoma is the pentad of severe headache, palpitations, significant perspiration, hypertension (HTN), and pallor. However, many patients present with subclinical findings, adding to the challenge for physicians to make this diagnosis. The following case is unusual in the patient’s history of three procedures under general anesthesia, with one being an ablation in 2019. A review of previous CT images showed an adrenal adenoma from 2019. This demonstrates that pheochromocytomas may have biochemically silent periods further complicating the diagnosis and management of pheochromocytoma. 78-year-old female presents s/p elective ablation with atrial tachycardia. PMH is remarkable for T2D, HTN, hypothyroidism, CAD, and atrial fibrillation/AVNRT. In 2019 patient had the first ablation without any complications related to blood pressure fluctuations. Due to unknown reasons, the patient had persistent atypical atrial fibrillation with dyspnea, with a plan to redo ablation for worsening symptoms of shortness of breath. Then in 2022 patient underwent a second ablation and developed labile blood pressure with initial findings of 77/48 with signs of cardiogenic shock and subsequent pulmonary edema. Due to the patient’s presentation of hypovolemia secondary to the development of cardiogenic shock, the patient required vasopressors. The patient was started on a beta-blockade in response to vasopressor treatment, which caused her blood pressure to increase significantly, with systolic pressures ranging between 230-240. The metoprolol was stopped, and the patient was transitioned to doxazosin, due to high suspicion of a pheochromocytoma. Initial stabilization of BP was achieved after starting an alpha blockade. BP remained somewhat labile but continued a downward trend with doxazosin. CT was later ordered and appreciable for a left adrenal mass. Past imaging records showed the mass had been present since 2019. Metanephrine labs were collected and found to be 6332 pg/ml, confirming the diagnosis of a pheochromocytoma. The patient was further stabilized with doxazosin and adequate volume resuscitation then transitioned to metoprolol with tight control of blood pressure. Her hospital course proved to be difficult due to prior comorbidities and age, but she was ultimately stabilized and discharged after a three-week hospital stay. A vast majority of patients with AFib have chronic HTN. There are many different etiologies of secondary HTN, including a more uncommon cause, pheochromocytomas. Common subclinical symptoms are as follows: headache, palpitations, sweating, HTN, and anxiety, which are also common presentations of AFib. Long-term management for pheochromocytomas would improve significantly if screenings were done in a patient presenting with symptomatic Afib and history of HTN. Positive screening would initiate early treatment, consisting of a short alpha blockade followed by a chronic, low-dose beta blockade. In this patient, the pheochromocytoma might have contributed to the failure of ablation treatments and the persistence of atrial fibrillation.

Abandonment of intravenous volume expansion after preoperative receipt of α-blockers in patients with adrenal pheochromocytoma was not an independent risk factor for intraoperative hemodynamic instability.

There is no consensus on whether intravenous rehydration must be added after preoperative phenoxybenzamine (PXB) administration for pheochromocytoma. The aim of this study is to investigate whether abandonment of intravenous volume expansion after PXB administration is associated with intraoperative hemodynamic instability. 83 Patients with pheochromocytoma received surgical treatment in the Department of Urology, Handan First Hospital, between October 2014 and July 2022. All patients were subclassified into either the hemodynamic stability group (HS group) or the hemodynamic instability group (HU group) according to whether intraoperative hemodynamic instability occurred, with 51 cases in HS group and 32 cases in HU group. Differences in data between the two groups were examined, and the risk factors for intraoperative hemodynamic instability were analyzed using logistic regression. The results of the analysis showed no statistically significant differences in age, sex, location of the tumor, surgical method, body mass index (BMI) ≥ 24 kg/m2, blood and urine catecholamine test results, preoperative oral PXB followed by combined intravenous volume expansion, proportion of patients with hypertension or diabetes mellitus or coronary heart disease between the two groups (P>0.05). The size of the tumor in the HS group was smaller than that in the HU group (5.3 ± 1.9cm vs 6.2 ± 2.4cm P=0.010). Multivariate analyses demonstrated that abandonment of intravenous volume expansion after preoperative receipt of α-blockers in patients with adrenal pheochromocytoma was not an independent risk factor for intraoperative hemodynamic instability. Only the tumor size (P=0.025) was an independent risk factor for intraoperative hemodynamic instability. The purpose of general preoperative intravenous fluid expansion is to prevent hypotension after the tumor has been resected. In the current study, we indicated that preoperative management of pheochromocytomas using the α-blocker PXB in combination with intravenous volume expansion does not further reduce the risk of intraoperative hemodynamic instability or postoperative complications compared with oral PXB alone. Therefore, our study supports preoperative management of pheochromocytoma with a single α-blocker, PXB, as sufficient.

Anesthetic management of pheochromocytoma and paraganglioma for patients with Fontan circulation: a case series

BackgroundAnesthetic management of pheochromocytoma and paraganglioma with Fontan circulation is challenging for physicians, with attention to cardiovascular physiology.Case presentationWe performed anesthetic management for pheochromocytoma and paraganglioma in three patients with Fontan circulation. We maintained intraoperative central venous pressure at preoperative level under fluid infusion and administrating nitric oxide to decrease pulmonary arterial resistance. We administered noradrenaline or vasopressin if low blood pressure was present despite adequate central venous pressure. Although noradrenaline is prevalent for the case of noradrenaline-secreting tumor especially after resection, we could maintain blood pressure to administrate vasopressin without increasing central venous pressure. Retroperitoneal laparoscopic approach which could avoid intra-abdominal adhesions might be selectable as case 3.ConclusionsSophisticated management is required for pheochromocytoma and paraganglioma with Fontan circulation.

226: THE PHEO-HEPATO PHENOMENON: TRANSAMINITIS IN THE SETTING OF ACTIVE PHEOCHROMOCYTOMA

Introduction: Pheochromocytomas are neuroendocrine tumors that cause release of excess catecholamines. This release causes commonly known signs and symptoms of hypertension, tachycardia, headache, flushing, and palpitations. Less commonly seen is abdominal pain with transaminitis. Description: A 47-year-old woman with no medical history presented with one day of severe abdominal pain, nausea, vomiting, palpitations, and symptoms of feeling flushed. Initial evaluation found the patient to be hypertensive, tachycardic, and tachypneic. On CT imaging, the patient was found to have a 6.5cm ovoid adrenal mass and lab work showed elevated 24-hour urine and plasma metanephrines, consistent with pheochromocytoma. The patient’s course was complicated by the development of transaminitis (had been normal on initial lab work), with elevation to AST 4882 U/L, ALT 4183 U/L, with normal alkaline phosphatase and bilirubin. Both ultrasound and CT imaging showed intrinsic fatty liver disease with patent vasculature, though no underlying pathology in the setting of no personal history of alcohol abuse, IV drug use, and non-reactive hepatitis panel. Hepatology consulted, and etiology thought to be in the setting of poor perfusion due to catecholamine storm. With medical treatment of the pheochromocytoma, AST and ALT significantly down-trended (75% within one week), and had normalized by the patient’s adrenalectomy and have remained normal in the post-operative period. Discussion: Pheochromocytoma is a rare neuroendocrine tumor, which has varying symptomatology on presentation, though commonly includes severe hypertension with headache, sweating, and palpitations related to catecholamine surge. A less common, though still reported presenting symptom, is abdominal pain. There have been cases in the literature wherein patients with pheochromocytoma present with abdominal pain and transaminitis, though not to the degree that has been seen in our patient. The pathogenesis of this in the setting of pheochromocytoma is not completely understood, though is thought to be related to increased vascular resistance due to catecholamine release, leading to decreased blood flow and oxygenation. With medical and ultimate surgical management of pheochromocytoma, liver function tests can be expected to return to baseline values.

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.

PSAT007 Pheochromocytoma Presenting With Small Bowel Obstruction

Abstract Background Pheochromocytoma is a rare catecholamine producing tumor arising from the adrenal gland with estimated prevalence of 0.1%-0.6%. Most common signs and symptoms of pheochromocytoma, i.e. headache, palpitations and sweating are non-specific and often mimic other diseases. The association between pheochromocytoma and small bowel obstruction however is rarely recognized. Case 55-year-old Russian male with past medical history of alcohol abuse, smoking and hypertension presented to ER with chest pain, abdominal pain, nausea, vomiting, poor appetite, diarrhea and 20 lbs weight loss for 2-3 months. On presentation, patient was hypertensive with blood pressure of 225/137 mm/Hg and heart rate of 126 beats/minute. He appeared uncomfortable, skin was flushed, and he was noted to be tachycardic with distended abdomen and sluggish bowel sounds. Initial laboratory studies revealed hyperkalemia of 7.8 mmol/L, CO2 13 mmol/L, anion gap 21 mEq/L, acute kidney injury with creatinine 1.34 mg/dL, venous pH 7.18, lactic acid of 5.7 mmol/L. A polymerase chain reaction test for COVID-19 was negative. Alcohol and urine drug screen were negative. Persistent diarrhea, abdominal pain with distention prompted evaluation with abdominal X-ray which showed distended small bowel loops. As part of the hypertensive crisis work up, CT angiogram of the chest, abdomen and pelvis with contrast was done and it demonstrated an enhancing left adrenal mass 4.6×4.5 cm with 35 Hounsfield units on the non-contrast study and 95 Hounsfield units on the venous phase. Evaluation of plasma hormone levels revealed elevated metanephrines &amp;gt;5,000 pg/mL (n 0-88 pg/mL) and normetanephrine &amp;gt;6,000 pg/mL (n 0-137 pg/mL). The 24-h urinary catecholamines were highly elevated with metanephrine &amp;gt;9,000 ug (n 58-276 ug/24hr) and normetanephrine &amp;gt;12,000 ug (n 156-729 ug/24hr). Patient was initially treated with Labetalol infusion with noted improvement in diarrhea. His hospital course was complicated by an acute myocardial infarction with cardiac thrombus. Patient was stabilized with Prazosin and discharged from the hospital. One month later he underwent left laparoscopic adrenalectomy. Gross pathology showed 5.1×4.5×3.8 cm adrenal mass with immunohistochemical staining supporting a diagnosis of pheochromocytoma. Discussion Recognition of clinical features of pheochromocytoma is often challenging but important due to its high morbidity and mortality rates. Small bowel obstruction in association with pheochromocytoma is uncommon. In smaller case series, ileus, megacolon, and bowel infarction was observed in patients with larger adrenal masses, typically &amp;gt;70 grams and catecholamine levels more than 4 times the upper limit of normal. This case highlights the importance of maintaining high index of clinical suspicion in early recognition of the gastrointestinal manifestations of pheochromocytoma and its potentially catastrophic consequences. References (1) Hodin R, Diagnosis, and management of pheochromocytoma. Curr Probl Surg. April 2014 Apr;151-87. Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.

PSAT021 Hemorrhagic Pheochromocytoma Presenting With Cardiogenic Shock: Challenges in Diagnosis and Treatment.

Abstract Introduction Pheochromocytomas are rare catecholamine producing tumors with heterogenous clinical presentations. Although acute catecholamine-induced cardiomyopathy is well described in the literature, it is rarely suspected as a cause of cardiogenic shock. Timely diagnosis is challenging and appropriate preoperative treatment is crucial for successful management, as this condition carries a high mortality rate. We describe a case of hemorrhagic pheochromocytoma presenting with refractory hypotension and discuss the difficulties in diagnosis and treatment we encountered. Case Presentation A 57-year-old man presented with pallor, severe headache, and hypotension. After administration of intravenous fluids, the patient developed flash pulmonary edema and was found to have acute systolic heart failure. He was taken for emergent left heart catheterization, which did not show any evidence of coronary obstruction. Therefore, he was treated for presumed myocarditis and received stress dose steroids. His condition quickly deteriorated to cardiogenic shock and was further complicated by cardiac arrest. He required extracorporeal membrane oxygenation (ECMO) treatment for 2 days, during which time his systolic blood pressures were extremely labile (47–270 mmHg). CT angiography incidentally noted a 4.3 cm right para-adrenal lesion, at which time urine metanephrines were ordered and noted to be markedly high. Ga-68 DOTATATE scan was negative, however, dedicated abdominal CT revealed a likely hemorrhagic right adrenal mass. With supportive care, the patient's cardiac function recovered by the fifth hospital day, and labetalol was slowly introduced, followed by doxazosin. Sixteen days after the initial presentation, laparoscopic right adrenalectomy was successfully performed. Pathology confirmed pheochromocytoma with few positive cells in a hemorrhagic and necrotic adrenal lesion. Discussion Our case underlines several important points in the diagnosis and management of pheochromocytoma. First, it is crucial to have a high level of suspicion for a catecholamine-secreting tumor in the setting of labile blood pressures and acute cardiomyopathy, especially in cases without evidence of coronary artery or valvular disease. Notably, steroids should be used cautiously, as they may further precipitate catecholamine crisis and have been associated with hemorrhagic pheochromocytomas. While catecholamine-induced cardiomyopathy is associated with a high mortality rate, the majority of cases are reversible with timely management. Treatment is complex, as standard initial pheochromocytoma management with alpha-blockers and hydration is often precluded due to cardiogenic shock, and vasopressors such as norepinephrine and epinephrine can worsen cardiomyopathy. Furthermore, early initiation of mechanical circulatory support (specifically ECMO) was a life-saving modality, as it facilitated cardiac function recovery and enabled bridging with alpha blockers. Better prognosis is associated with surgery performed after improvement of cardiomyopathy and alpha blockade. And finally, a multidisciplinary approach is crucial for the successful treatment of pheochromocytoma in emergency situations. Given this rare presentation of pheochromocytoma more, data are needed to define an optimal strategy. Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.

PSAT006 Two Cases of Pheochromocytoma Associated Takotsubo Syndrome- Importance of Early Recognition and Treatment

Abstract Introduction Pheochromocytoma is a rare catecholamine producing adrenal medullary tumor that classically presents with hypertension. Takutsubo cardiomyopathy (TC) is a rare but increasingly recognized presentation of pheochromocytoma. We present two cases of TC likely caused by pheochromocytoma. Case 1 A 60 y/o male who presented with acute onset nausea, vomiting and abdominal pain. Systolic BP was 150-160 mm Hg, and diastolic BP was 100 mmHg. Troponin was elevated (4.15 ng/ml; nl = 0-0.04). An echocardiogram showed a reduced left ventricular ejection fraction (LVEF) of 15-20% (nl = 50-75%), a mildly dilated left ventricle, and apical ballooning suggestive of TC. Coronary arteries were normal on cardiac catheterization. CT scan identified a 5.3×5.6×5.7 cm adrenal mass of 50 HU pre-contrast. Further evaluation revealed markedly elevated plasma metanephrine concentration of 858 pg/ml (nl&amp;lt;57) and normetanpehrine concentration of 1729 pg/ml (&amp;lt;148). His presentation was strongly suggestive of pheochromocytoma associated TC. He was prepared for surgery with doxazosin 2 mg twice daily followed by metoprolol succinate and metyrosine up to 500 mg/d. Final pathology revealed a 5.3 cm left pheochromocytoma with negative surgical margins. Germline genetic testing was negative for pheochromocytoma/paraganglioma pathogenic gene mutations, and an MIBG scan did not reveal metastatic disease. Echocardiogram one month after adrenalectomy showed a marked improvement in LVEF to 55-60% with no regional wall motion abnormalities, and plasma metanephrine concentrations normalized. Case 2 A 61 y/o female smoker with type 2 diabetes mellitus, hypertension, Graves disease and obesity presented with sudden onset of nausea followed by chest discomfort, bilateral shoulder discomfort and shortness of breath. Troponin was 0.6 ng/ml (nl = 0-0.04), EKG showed ST elevations in leads I, 2, V5 V6 with hyperacute T waves. Coronary arteries were normal on catheterization. She required intubation, and systolic BP ranged from 110 to 200 mm Hg. On echocardiogram the LVEF was 35% to 40% (nl = 50-75), and there was diffuse hypokinesis of the mid to apical LV myocardium with hypercontractility of the LV base, suggestive of TC. The plasma metanephrine concentration was 3.31 nmol/l (nl= 0-0.49), and normetanephrine concentration was 5.02 nmol/l (nl= 0-0.89). CT imaging revealed a 2.9 cm left adrenal mass. She underwent successful left adrenalectomy after preparation with alpha, beta blockade and metyrosine. Germline genetic testing was negative for pheochromocytoma/paraganglioma pathogenic gene mutations . Echocardiogram nine months post-adrenalectomy showed normal EF of 50-55% and resolution of apical hypokinesis. Discussion We present two pheochromocytoma patients presenting with TC that reversed following appropriate pheochromocytoma management. Few case series report the association of pheochromocytoma and TC. These patients usually present acutely without prodromal stressors commonly found with classic TC. Conclusion In the setting of TC, pheochromocytoma should be considered. Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.

PSAT018 Management of Pheochromocytoma in the Setting of a Patient with Multiple Vasculopathies

Abstract A 66-year-old male with history of Type 2 diabetes and adrenal incidentaloma presented to the hospital with significant left lower limb edema and was found to have severe peripheral vascular disease. CT surgery was consulted and performed an angiogram as well as an angioplasty; however, the patient had severe stenosis and stenting was not possible. Femoral popliteal bypass was planned. Due to the patient's acute kidney injury from renovacular hypertension, preoperative evaluation included a MRI abdomen w/wo contrast which showed a 1.4 cm right adrenal nodule with early contrast enhancement which appeared fairly avid and uniform. This nodule had been previously noted by CT scan three years prior. Previous outpatient labs showed elevated metanephrines of 543 pg/mL, which determined the adrenal nodule to be consistent with a pheochromocytoma. Subsequently, femoral popliteal bypass was postponed and the patient was started on an alpha blocker, doxazosin. Urology was consulted and right laproscopic adrenalectomy was planned. Preoperatively, patient developed chest pain due to ventricular tachycardia and NSTEMI was diagnosed by EKG. Patient then underwent a LHC which revealed severe left main 3 vessel coronary artery disease, postponing planned adrenalectomy. CABG was performed with pericardiectomy. After initial recovery, patient was discharged to in-patient rehabilitation, indefinitely delaying definitive pheochromocytoma therapy. Reference Jacques W. M. Lenders, Quan-Yang Duh, Graeme Eisenhofer, Anne-Paule Gimenez-Roqueplo, Stefan K. G. Grebe, Mohammad Hassan Murad, Mitsuhide Naruse, Karel Pacak, William F. Young, Jr, Pheochromocytoma and Paraganglioma: An Endocrine Society Clinical Practice Guideline, The Journal of Clinical Endocrinology &amp; Metabolism, Volume 99, Issue 6, 1 June 2014, Pages 1915–1942, https://doi.org/10.1210/jc.2014-1498 Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.

ODP032 Case Series: Prazosin in the Preoperative Management of Pheochromocytoma and Paraganglioma

Abstract Background Prazosin is a competitive alpha-1 adrenoreceptor antagonist. It is an ideal agent for preoperative blockade in neuroendocrine tumors compared to more commonly used agents such as phenoxybenzamine, a non-selective alpha blocker or doxazosin a longer acting alpha-1 blocker. Its affinity for alpha-1 receptors lessens the risk for systemic side effects while its short half-life and a relatively short duration of action of about 10-24 hours decreases the risk for intra and post-operative hypotension. Case 1 38 year old male was found to have resistant hypertension during routine follow up visit. Work up for secondary causes of hypertension revealed plasma metanephrines 5.1nmol/L, normetanephrine 6.7nmol/L, epinephrine 5990pg/mL, norepinephrine 15318 pg/mL, with 24 hour urine epinephrine 1329ug/24hr and norepinephrine 3115ug/24hr. Imaging findings shows 4.8cm right adrenal mass consistent with Pheochromocytoma. He was started on Prazosin titrated up to a dose of 5mg TID and labetalol 200mg TID to a goal systolic BP &amp;lt;130 mmHg. A right laparoscopic adrenalectomy was done and intraoperatively, he remained hemodynamically stable with no vasopressor or antihypertensive requirement. His blood pressure normalized immediately post op and did not require further antihypertensives for the rest of his hospitalization. Case 2 62 year old female was found to have an incidental 4.7cm left adrenal mass after a routine surveillance for pulmonary nodule. Biochemical data showed plasma metanephrines 14nmol/L, normetanephrine 3nmol/L, epinephrine 2719pg/mL, norepinephrine 701pg/mL, and chromogranin A 3507ng/m with a 24 hour urine epinephrine 938uh/24hr, consistent with Pheochromocytoma. The patient was started on Prazosin, titrated up to a maximum tolerated dose of 2mg TID before symptomatic hypotension occurred. Labetalol 100mg BID was later added. She underwent a left laparoscopic adrenalectomy but required Clevidipine drip and IV pushes of norepinephrine PRN intraoperatively for low blood pressure. However, she was taken off antihypertensives immediately post-op, and did not have any hypotensive episodes events thereafter. Case 3 50 year old female presented for left sided chest pain where a CT angiogram was done and incidentally found a para-aortic mass measuring 2.7×1.6×2.3cm in the organ of Zuckerlandl. Biochemical findings showed plasma normetanephrine 5.6nmol/L, norepinephrine 4564pg/mL, and chromogranin A 440ng/mL, consistent with a Paraganglioma. She was started on Prazosin titrated up to 6mg TID, labetalol 100mg TID and amlodipine 10mg daily to reach goal systolic blood pressure and heart rate. She underwent an open resection where there were no hypotensive events intraoperatively but did require Clevidipine drip for hypertension. Immediately post-op her BP normalized, the drip was discontinued, and she did not have any post-op hypotensive events. Conclusion This case series highlights the advantages of short acting alpha-1 blockade in the preoperative management of neuroendocrine tumors. All three patients did not have any post-operative hypotension and had an uncomplicated post-op course. Presentation: No date and time listed