Excessive Catecholamine Secretion Research Articles

6997 Atypical Presentation of Hereditary Paraganglioma

Abstract Disclosure: O. Abdulhussein: None. R.R. Correa: None. M.D. Lundholm: None. J. Ferri-Guerra: None. G. Madrigol: None. Introduction: Paraganglioma (PGL) is a rare neuroendocrine, highly vascular tumor arising from sympathetic or parasympathetic ganglia chromaffin cells. Some PGL functional while others biochemically silent. Given the possible lack of classic symptoms or convincing catecholamine elevation. They are closely related to pheochromocytomas.• However, the distinction between pheochromocytoma and paraganglioma is essential because of the implication for associated neoplasm and syndromes; paraganglioma is more metastatic. Case: 32-year-old otherwise healthy female presented to primary care clinic for 1 year of dry cough. She was a non-smoker. She denied voice changes, palpitations, hypertension, palpitations, pallor, headache, or diaphoresis, but had noticed a mass growing in her right neck for a year. On exam her blood pressure 107/73 and there was a mobile, round mass felt at the right mandible. CT neck at the time showed reactive lymphadenopathy. She referred to pulmonology and ENT, spirometry and flexible laryngoscopy were unremarkable. For persistent mass 1year later, an MRI of the neck showed a 2.8x2.3x3.8 cm right carotid mass, likely PGL. Biochemical testing revealed elevations in plasma normetanephrine (NM) (269 pg/mL, 18-101 pg/mL) and 24-hour urinary NM (848 ug/24hr, 88-444 ug/24hr) with mild elevation in 24-hour urinary metanephrine (982 ug/24 hr, 140-785 ug/24 hr) and normal plasma metanephrine. The mass was intensely avid on DOTATATE scan without other lesions. Genetic testing uncovered SDHB mutation. She was started on preoperative alpha-blockade with successful resection. She had pathology confirmation of PGL and was set up for annual SDHB surveillance testing. Discussion: A minority of patients with PCC or PGL present with classical “5 P” catecholamine symptoms: pallor, pain, pressure, perspiration, or palpitations. One main reason is a lack of excess catecholamine secretion; 17 years of retrospective data at our center showed that 61% (N=50/82) of patients with PGL with preoperative catecholamine labs had normal levels. Additionally, mild to moderate increases to 2-3x upper limit of normal may be attributed to stress or medications, which obfuscates the distinction between secretory disease and physiologic elevation.Given the infrequency of catecholamine symptoms or secretion, PGL is frequently discovered incidentally. Indeed, 92% (N=110/119) of our PGL cases were found incidentally on imaging or pathology. Alpha-blockade is the preoperative standard of care, many patients are only diagnosed post-operatively by pathology findings. Further study is needed to determine if pre-operative alpha-blockade is needed in all PGL cases, and if so, how we can better identify these patients before they go to the operating room. For now, a high level of suspicion is needed to make a timely PGL diagnosis and facilitate genetic testing. Presentation: 6/1/2024

Pregnancies complicated with functioning adrenal adenomas causing severe obstetric outcomes: a 20-year experience at a tertiary center

BackgroundFunctioning adrenal adenoma during pregnancy is rare, and the diagnosis is challenging owing to unspecific symptoms and restricted investigations. The obstetric outcomes of patients who undergo surgery during pregnancy or who receive only medical treatment are poorly described.ObjectiveThe aim was to investigate the associations between functioning adrenal adenomas and obstetric outcomes.MethodsA retrospective study was performed in a tertiary center over 20 years. The clinical characteristics, management and obstetric outcomes of the diagnosed pregnant women were reviewed.ResultsA total of 12 women were diagnosed with functioning adrenal adenomas during pregnancy from January 2002 to September 2022. Eight women had cortisol-secreting adrenal adenomas, two had excessive catecholamine secretion, and two had primary aldosteronism. The initial symptoms of adrenal adenoma during pregnancy included hypertension or preeclampsia, gestational diabetes mellitus or prepregnancy diabetes mellitus, hypokalemia and ecchymosis. Four women underwent adrenalectomy during pregnancy, while 8 women received only medical therapy. Preterm birth occurred in all patients who received medicine, whereas 1 patient who underwent surgery experienced preterm birth. Among the 8 women in the medical treatment group, 3 had neonates who died.ConclusionsOnce hypertension, hyperglycemia and hypokalemia occur during the 1st or 2nd trimester, pregnant women with adrenal adenomas should be evaluated via laboratory and imaging examinations. The maternal and fetal outcomes were unpredictable owing to the severity of adrenal adenoma, particularly in patients who received only medical treatment. Adrenalectomy should be recommended during pregnancy.

P-06 Catecholamine-induced cardiomyopathy: A rare presentiation of pheochromocytoma

Abstract Introduction Pheochromocytoma and paraganglioma (PPGL) are rare neuroendocrine tumors. Clinical manifestations consist of various cardiovascular signs and symptoms related to excessive secretion of catecholamines. Catecholamine-induced cardiomyopathy is a rare but life-threatening complication of PPGL. Excessive discharge of catecholamines results in vasoconstriction, coronary vasospasm and myocardial ischemia. Three types of cardiomyopathies can be observed in patients with PPGL; dilated cardiomyopathy, Takotsuba cardiomyopathy and hypertrophic cardiomyopathy. Clinical Case Herein, we present a case with PPGL complicated with heart failure probably due to excessive secretion of catecholamines. A 19 years-old man was admitted to emergency department with dyspnea and hypotension. He exhibited severe signs of heart failure with very high liver function tests; ALT: 3647 U/L (8-65) and AST: 4542U/L (7-40), probably due to hepatic congestion. Echocardiogram performed in ICU reported %10 left venticular ejection fraction with diffuse akinesia. Remarkable improvement of liver function was recorded following the introduction of heart failure medicines. A computed tomography scan that was performed for abdominal pain and distension revealed a six cm tumour at the left adrenal gland compatible with pheochromocytoma. Laboratory analyses were; 24-hour urine metanephrines: 1938mcg (44-261), normetanephrines: 17934mcg (103-390). Following necessary preoperative preparations, laparoscopic left adrenalectomy was performed and a 70 mm adrenal tumor, histopathologically compatible with pheochromocytoma, was excised. His postoperative laboratory findings were; 24-hour urine metanephrines: 248 mcg (44-261), normetanephrines: 4368 mcg (103-390), chromogranin A: 554.9 ng/ml (0-100), 3-methoxytyramine: 0.34 nmol/l (0-0.17). A 68-Ga-DOTA-Peptide PET/CT following 12 weeks of surgery demonstrated hypermetabolic lesions at left suprarenal area. A second open surgery was performed and lymph node metastases were detected (Figure 1). His cardiac functions ameliorated significantly following the removal of tumors. Genetic analysis for PPGL syndromes were negative for KIF1B, SDHB, NTRK1, TMEME127, VHL, RET, SDHD genes. Work-up is still pending for the mutations of SDHA, SDHC and SDHAF2 genes. Conclusion Cathecolamine-induced cardiomyopathy is a rare entity associated with PPGL. Urgent recognition of related clinical signs and symptoms and detection of PPGL is lifesaving. It should be kept in mind in patients presenting with symptoms of heart failure, hypotension and multisystem crisis in the absence of coronary or valvular heart disease.Figure 1.Pheochromocytoma with lymph node metastasis. (H&E x100)

Canine phaeochromocytoma: a guide to diagnosis and treatment

Phaeochromocytomas are neuroendocrine tumours arising from chromaffin cells of the adrenal medulla. Clinical signs are primarily associated with excessive catecholamine secretion and, to a lesser extent, with the space-occupying or invasive nature of the tumour. The diagnosis of phaeochromocytoma relies on clinical suspicion, biochemical testing, diagnostic imaging and histopathology. Biochemical testing mainly depends on the measurement of levels of plasma or urinary metanephrines, with normetanephrine demonstrating superior diagnostic performance compared to metanephrine. Adrenalectomy is the treatment of choice, but may not be possible in cases with extensive local invasion, concurrent disorders or distant metastasis. Contrast-enhanced computed tomography is recommended for surgical planning and metastasis screening. Vascular invasion is frequently observed, yet surgery remains a viable option in many cases. Recent studies question the necessity of alpha-blockade before surgery, and randomised controlled clinical trials are necessary to evaluate this. Long-term survival is often seen in dogs that survive the perioperative period. For patients in which surgery is not deemed feasible, medical treatment with tyrosine-kinase inhibitors may be considered. Given the current scarcity of effective medical treatment, there is an urgent need to identify novel therapeutic options. This review provides recommendations on the diagnosis and management of canine phaeochromocytoma.

A rare case of giant adrenal ganglioneuroma

Ganglioneuromas (GN) are rare benign tumors that typically occur in the posterior mediastinum or retroperitoneal region, but can also be found in adrenal glands. Up to 50% of patients are asymptomatic, but symptoms can arise from the mass effect or excessive secretion of catecholamines. Careful evaluation is necessary to differentiate GNs from other adrenal tumors. A 19-year-old female patient with no previous comorbidities was diagnosed with a right adrenal incidentaloma. Physical examination and routine laboratory tests were normal. Imaging revealed a solid lesion in the right adrenal gland, and excisional adrenalectomy was performed. Pathological examination confirmed the diagnosis of a right adrenal GN. The patient had an uneventful recovery and showed no recurrence during the follow-up period. Conclusion: After surgical removal of a benign neurogenic tumor, our patient had no tumor recurrence after five months, indicating a positive prognosis. However, long-term follow-up is still recommended because of reported cases of metastases and recurrence in some patients.

The connection between tricarboxylic acid cycle enzyme mutations and pseudohypoxic signaling in pheochromocytoma and paraganglioma.

Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors originating from chromaffin cells, holding significant clinical importance due to their capacity for excessive catecholamine secretion and associated cardiovascular complications. Roughly 80% of cases are associated with genetic mutations. Based on the functionality of these mutated genes, PPGLs can be categorized into distinct molecular clusters: the pseudohypoxia signaling cluster (Cluster-1), the kinase signaling cluster (Cluster-2), and the WNT signaling cluster (Cluster-3). A pivotal factor in the pathogenesis of PPGLs is hypoxia-inducible factor-2α (HIF2α), which becomes upregulated even under normoxic conditions, activating downstream transcriptional processes associated with pseudohypoxia. This adaptation provides tumor cells with a growth advantage and enhances their ability to thrive in adverse microenvironments. Moreover, pseudohypoxia disrupts immune cell communication, leading to the development of an immunosuppressive tumor microenvironment. Within Cluster-1a, metabolic perturbations are particularly pronounced. Mutations in enzymes associated with the tricarboxylic acid (TCA) cycle, such as succinate dehydrogenase (SDHx), fumarate hydratase (FH), isocitrate dehydrogenase (IDH), and malate dehydrogenase type 2 (MDH2), result in the accumulation of critical oncogenic metabolic intermediates. Notable among these intermediates are succinate, fumarate, and 2-hydroxyglutarate (2-HG), which promote activation of the HIFs signaling pathway through various mechanisms, thus inducing pseudohypoxia and facilitating tumorigenesis. SDHx mutations are prevalent in PPGLs, disrupting mitochondrial function and causing succinate accumulation, which competitively inhibits α-ketoglutarate-dependent dioxygenases. Consequently, this leads to global hypermethylation, epigenetic changes, and activation of HIFs. In FH-deficient cells, fumarate accumulation leads to protein succination, impacting cell function. FH mutations also trigger metabolic reprogramming towards glycolysis and lactate synthesis. IDH1/2 mutations generate D-2HG, inhibiting α-ketoglutarate-dependent dioxygenases and stabilizing HIFs. Similarly, MDH2 mutations are associated with HIF stability and pseudohypoxic response. Understanding the intricate relationship between metabolic enzyme mutations in the TCA cycle and pseudohypoxic signaling is crucial for unraveling the pathogenesis of PPGLs and developing targeted therapies. This knowledge enhances our comprehension of the pivotal role of cellular metabolism in PPGLs and holds implications for potential therapeutic advancements.

Acute heart failure with incidentally found cystic adrenal mass.

Pheochromocytomas are rare adrenal tumors characterized by excessive catecholamine secretion. Symptoms and signs associated with pheochromocytomas are usually intermittent and chronic but can rarely develop into life-threatening crises. We describe a case of acute severe congestive heart failure in a previously healthy female, who recovered rapidly (4 days after admission) with acute medical therapy. The etiology on evaluation was a spontaneous bleed in a previously undiagnosed pheochromocytoma, resulting in a pheochromocytoma crisis and transient stress cardiomyopathy, followed by quick recovery of cardiac function. Our aim is to describe pheochromocytoma as a rare cause of stress cardiomyopathy. We discuss the evaluation of pheochromocytoma during critical illness and triggers/treatment strategies for pheochromocytoma crises. Hemorrhage in a pheochromocytoma can result in a pheochromocytoma crisis, with sudden release of excess catecholamines resulting in multisystem organ dysfunction and high mortality. Acute decompensated heart failure can be a rare presentation of pheochromocytoma, in a patient with no cardiac risk factors. Measurement of metanephrines in acutely stressful clinical situations can have considerable overlap with the biochemical picture of pheochromocytoma. Early imaging studies may help with the differential diagnosis. Pheochromocytoma should be ruled out before performing an adrenal biopsy. Emergent adrenalectomy in pheochromocytoma crisis results in high mortality. Medical management of the acute crisis followed by elective adrenalectomy after alpha-blockade results in better outcomes.

Phaeochromocytoma associated with cardiomyopathy and leukocytoclastic vasculitis in a dog

Phaeochromocytomas are rare tumours of the adrenal medulla that can be associated with various presentations. Many of the better characterized clinical signs, including weakness, tachycardia and tachypnoea, are attributable to excessive and unregulated catecholamine secretion from functional tumours. In addition to catecholamine-induced cardiomyopathy and vasospasm, the invasive nature of phaeochromocytomas can lead to occlusion of the caudal vena cava contributing to systemic cardiovascular compromise. In humans, leukocytoclastic vasculitis is a rarely reported manifestation of catecholamine excess associated with phaeochromocytomas. We now describe a dog that had an invasive unilateral phaeochromocytoma with histological evidence of myocardial damage, consistent with catecholamine-induced cardiomyopathy, and leukocytoclastic vasculitis of small vessels in a range of tissues. We conclude that catecholamine excess may have played a role in the pathogenesis of vasculitis in this case. To the best of our knowledge, this is the first documented association between phaeochromocytoma and leukocytoclastic vasculitis in a non-human species.

Adrenal Medullary Hyperplasia: A Systematic Review and Meta-analysis.

Adrenal medullary hyperplasia (AMH) is a rare, incompletely described disorder of the adrenal medulla that is associated with catecholamine excess. To increase knowledge about AMH by reviewing the reported cases of this disorder. Systematic review and meta-analysis of the genotype/phenotype relationship in all reported cases of AMH. Literature review and analysis. All cases of AMH published to date. Characteristics of AMH cases and genotype-phenotype relationships. A total of 66 patients, median age of 48 years, were identified from 29 reports. More than one-half were male (n = 39, 59%). The majority had unilateral (73%, n = 48) disease; 71% (n = 47) were sporadic and 23% (n = 15) were associated with the MEN2. Most (91%, n = 60) displayed signs and symptoms of excess catecholamine secretion, particularly hypertension. Elevated catecholamine concentrations (86%, n = 57) and adrenal abnormalities on imaging were common (80%, n = 53). More than one-half (58%, n = 38) had concurrent tumors: pheochromocytoma (42%, n = 16/38); medullary thyroid cancer (24%, n = 9/38); and adrenocortical adenoma (29%, n = 11/38). Most (88%, n = 58) underwent adrenalectomy with 45/58 achieving symptom resolution. Adrenalectomy was less common in patients under 40 years and those with bilateral disease (both P < .05). AMH may be sporadic or associated with MEN2, most have catecholamine excess and imaging abnormalities. Unilateral involvement is more common. Most reported patients have been treated with adrenalectomy, which is usually curative with regard to catecholamine hypersecretion.

PS-R04-4: A CASE OF PHEOCHROMOCYTOMA IN A 17-YEAR-OLD MALE

Background: Pheochromocytoma is a rare catecholamine secreting tumor arising from the adrenal medulla and produces signs and symptoms due to excessive catecholamine secretion from the tumor. It is a rare cause of secondary hypertension and a delay in recognition and treatment can present serious consequences to the patient. Case Summary: We present a case of a 17-year-old male with a history of hypertension for 4 years with episodic headache, palpitations, and sweating. He does not have any history of serious illnesses, hospitalizations, or trauma. He was not on any medications. He has a family history of hypertension. He does not drink alcohol, smoke cigarettes, or do any illicit drugs. Upon arrival to the ER, he was conscious, coherent with BP of 170/140mmHg, PR 111bpm, RR 26cpm, and afebrile. Laboratory work-up showed proteinuria, elevated BUN and creatinine levels, hyponatremia, hypokalemia, and hypocalcemia. A 2D echocardiography showed left ventricular hypertrophy. The whole abdominal CT scan demonstrated a fairly defined hypodense mass in the left suprarenal measuring 4.8 x 6.4 x 4.7 cm. Pheochromocytoma was confirmed by biochemical tests that showed elevated plasma normetadrenaline and 24-hour urine metanephrine. The patient was initially started on an alpha-adrenergic blocker, Terazosin, and a beta-blocker, Propranolol. Blood pressure was still uncontrolled hence calcium-channel blocker, Nicardipine drip was also given to control the blood pressure. During surgery, they noted the mass to be 8x5 cm invading the superior pole to mid pole and renal hilum hence the patient underwent left open adrenalectomy with left radical nephrectomy. Immediately upon removal of the mass, the patient had a hypotensive episode, which was addressed immediately. The histopathologic finding showed no extension of the mass to the kidneys and it is consistent with pheochromocytoma. Blood pressure control without medication was achieved 13 days post-operatively. The patient is regularly monitored for the recurrence of hypertension due to the increased risk of recurrence. The patient is now living with a solitary kidney hence should also be followed up through. Conclusion: Although it is a rare condition, pheochromocytoma should be considered a differential diagnosis in young patients presenting with unexplained hypertension, headache, and palpitations. A multidisciplinary approach is the best way to achieve success in the management of a patient with pheochromocytoma.

Pheochromocytoma crisis precipitated by dexamethasone with profound lactic acidosis, but without severe hypertension.

We describe a case of a 47-year-old patient who presented with severe lactic acidosis, troponinemia, and acute kidney injury after receiving 8 mg of intramuscular dexamethasone for seasonal allergies in the setting of an undiagnosed epinephrine-secreting pheochromocytoma. This case was atypical, however, in that the patient exhibited only mildly elevated noninvasive measured blood pressures. Following a period of alpha-adrenergic blockade, the tumor was resected successfully. Steroid administration can precipitate pheochromocytoma crisis that may present unusually as in our patient with mild hypertension but profound lactic acidosis. Steroids administered via any route can precipitate pheochromocytoma crisis, manifested by excessive catecholamine secretion and associated sequelae from vasoconstriction. Lack of moderate/severe hypertension on presentation detracts from consideration of pheochromocytoma as a diagnosis. Lactatemia after steroid administration should prompt work-up for pheochromocytoma, as it can be seen in epinephrine-secreting tumors. Noninvasive blood pressure measurements may be unreliable during pheochromocytoma crisis due to excessive peripheral vasoconstriction.

RF21 | PSUN08 Morphologic or Molecular Imaging - Diagnostic Capabilities in von Hippel-Lindau related pheochromocytoma

Abstract Background Within a spectrum of cancers implicated by VHL germline pathogenic variants, one rare cancer which may present in patients are pheochromocytomas (PHEOs). These tumors occur in the adrenal glands and predispose patients to a variety of morbid clinical symptoms due to the excess secretion of catecholamines. As a part of these patients’ management, imaging is important to localize the tumors of which there are both morphologic (CT and MRI) and molecular (PET/CT with 18F-FDG, 68Ga-DOTATATE, 18F-DOPA, and 18F-DA) imaging modalities. Purpose To determine which imaging modalities provided the optimal detection of VHL-related PHEO. Methods The study compared detection of 19 PHEOs (18 histologically proven and 1 is a contralateral tumor awaiting resection) in 12 patients. The CT and MRI were evaluated by a body radiologist and the PET/CT of18F-FDG, 68Ga-DOTATATE, 18F-DOPA, and 18F-DA were evaluated by a nuclear medicine physician in a blinded fashion. Both experts graded using a five-point Likert scale (1 – not PHEO and 5 – PHEO). For analysis, the Likert scale was dichotomized with 1-3 designating negative for PHEO and 4-5 positive for PHEO. The findings were reported as detection rates of positive PHEOs found on imaging to the gold standard of pathologically (aside from one contralateral PHEO awaiting surgery) proven PHEOs. The McNemar test was performed to compare the statistical relevance of detection rates between histology and the imaging modalities. Two-sided p values &amp;lt;0.05 were considered significant. Results The detection rates were CT 17/19 [89.5%, 95% CI = 66.9%-98.7%], MRI 17/17 [100%, 95% CI = 80.5%-100%], 18F-FDG PET/CT 16/17 [94.1%, 95% CI = 80.5%-100%], 68Ga-DOTATATE 5/9 [55.6%, 95% CI = 21.2%-86.3%], 18F-DOPA 12/14 [85.7%, 95% CI = 57.2%-98.2%], and 18F-DA 4/6 [66.7%, 95% CI = 22.3%–95.7%]. All McNemar tests showed p-values greater than 0.05, which were not statistically significant. Conclusion The study was performed in a small patient set with VHL-related PHEO showed MRI, with the highest detection percent followed by 18F-FDG PET/CT. More trials with a larger patient set are needed to support the statistical relevance as it was not seen in this study. Furthermore, performing a study on a patient cohort with additional VHL associated cancers could prove clinically beneficial for patients. Presentation: Sunday, June 12, 2022 12:30 p.m. - 2:30 p.m., Sunday, June 12, 2022 1:18 p.m. - 1:23 p.m.

Paragangliomas da Cabeça e Pescoço: A Experiência de um Centro Oncológico do Sul da Europa

Paragangliomas are usually benign slow-growing tumors, but they are locally invasive and can cause significant morbidity. The aim of this study was to characterize the presenting symptoms, secretory status, genetics, imaging features, treatment modalities, post-treatment complications and survival of patients with head and neck paragangliomas treated at a single institution. We retrospectively reviewed the clinical records of patients managed at our center between 1997 and 2020. Seventy-three patients were included in the study, encompassing 89 head and neck paragangliomas. Forty-eight patients (65.8%) were female and 15 (20.5%) had multiple tumor sites (including 10 patients with multicentric benign paragangliomas and five with disseminated malignant disease). Regarding location, our series encompassed 40 temporal bone paragangliomas (44.9%), 24 carotid body paragangliomas (27%), 22 vagal paragangliomas (24.7%), two laryngeal paragangliomas (2.2%) and one sinonasal paraganglioma (1.1%). Excessive catecholamine secretion was detected in 11 patients (15.1%). Sixty-four patients (87.7%) underwent genetic testing. Of those, 24 (37.5%) exhibited pathogenic succinate dehydrogenase complex germline mutations. Regarding patients who presented with untreated disease, 45 patients (66.2%), encompassing 55 tumors, underwent surgery as primary treatment modality, 20 (29.4%; 23 tumors) were initially treated with radiotherapy and three patients (4.4%, encompassing three solitary tumors) were kept solely under watchful waiting. Five-year overall survival was 94.9% and disease-free survival was 31.9%. Head and neck paragangliomas are rare, slow-growing but locally aggressive tumors resulting in high morbidity but low mortality rates.

Somatostatin analogue pasireotide (SOM230) inhibits catecholamine secretion in human pheochromocytoma cells.

Increasingly common, neuroendocrine tumors (NETs) are regarded nowadays as neoplasms potentially causing debilitating symptoms and life-threatening medical conditions. Pheochromocytoma is a NET that develops from chromaffin cells of the adrenal medulla, and is responsible for an excessive secretion of catecholamines. Consequently, patients have an increased risk for clinical symptoms such as hypertension, elevated stroke risk and various cardiovascular complications. Somatostatin analogues are among the main anti-secretory medical drugs used in current clinical practice in patients with NETs. However, their impact on pheochromocytoma-associated catecholamine hypersecretion remains incompletely explored. This study investigated the potential efficacy of octreotide and pasireotide (SOM230) on human tumor cells directly cultured from freshly resected pheochromocytomas using an implemented catecholamine secretion measurement by carbon fiber amperometry. SOM230 treatment efficiently inhibited nicotine-induced catecholamine secretion both in bovine chromaffin cells and in human tumor cells whereas octreotide had no effect. Moreover, SOM230 specifically decreased the number of exocytic events by impairing the stimulation-evoked calcium influx as well as the nicotinic receptor-activated inward current in human pheochromocytoma cells. Altogether, our findings indicate that SOM230 acts as an inhibitor of catecholamine secretion through a mechanism involving the nicotinic receptor and might be considered as a potential anti-secretory treatment for patients with pheochromocytoma.

Cardiovascular Presentation in Pheochromocytoma: What We Should be Aware

Pheochromocytoma is a catecholamine-producing tumor that although being a rare disease, it poses diagnostic problems because its clinical presentation often mimics certain diseases, including cardiovascular disorders. The effects of excessive catecholamine secretion cause a variety of cardiovascular presentations ranging from hypertension to life-threatening cases such as hypertensive emergency, shock, supraventricular or ventricular arrhythmias, pulmonary edema, and acute coronary syndromes. The principal medical treatment for pheochromocytoma is a blockade of adrenergic receptors. However, surgical or tumor resection often provides complete resolution of abnormal myocardial dysfunction or arrhythmias, so this approach remains the mainstay of treatment that should be performed as soon as the diagnosis of pheochromocytoma is established. As clinicians, we must be aware of the characteristics of the cardiovascular manifestations of pheochromocytoma to make an earlier diagnosis and more appropriate management.

Adrenal Pheochromocytoma: A Case Report

Pheochromocytoma is a rare catecholamine secreting tumour originating usually from adrenal medulla and produces signs and symptoms due excessive catecholamine secretion from tumour. A young male patient of 36 yrs age presented with hypertension since 2 yrs, palpitation, profuse sweating, weight loss. Clinical suspicion of Pheochromocytoma was confirmed by CT scan and USG abdomen followed by catecholamines levels in plasma and urine. After preoperative preparation laparascopic removal of Pheochromocytoma was done. Postoperative recovery was uneventful and BP regains to normal range from 1st postoperative day. Pheochromocytoma is a rare cause of hypertension. If the diagnosis of Pheochromocytoma is overlooked, the consequences could be disastrous, even fatal; however, if a pheochromocytoma is identified, it is potentially curable, as being one of the cause of surgically correctable hypertension.

Pheochromocytoma Manifestation in Cardiovascular System: A Comprehensive Explanatory Review

Pheochromocytoma is a catecholamine-producing tumour that poses diagnostic problems because its clinical presentation often mimic certain diseases, including cardiovascular disorders. It is a rare disease. The effects of excessive catecholamine secretion cause a variety of cardiovascular presentation in pheochromocytoma, ranging from the most common, namely hypertension, to life-threatening case such as hypertensive emergency, shock, supraventricular or ventricular arrhythmias, pulmonary edema, and acute coronary syndromes. The principal medical treatment for pheochromocytoma is blockade of adrenergic receptors. However, surgical or tumour resection often provides complete resolution of abnormal myocardial dysfunction or arrhythmias. As clinicians, we must be aware of the characteristics of the cardiovascular manifestations of pheochromocytoma so that we can make an earlier diagnosis and more appropriate management.

Intrarenal hemodynamics and kidney function in pheochromocytoma and paraganglioma before and after surgical treatment

Purpose Current evidence regarding renal involvement in pheochromocytoma and paraganglioma (PPGL) is scant. More accurate diagnostic methods, such as renal Doppler ultrasound for intrarenal hemodynamic studies, may provide more detailed information on renal function. It might be postulated that renal function in PPGL patients might be altered by high blood pressure and excess secretion of catecholamines. The aim of this prospective study was to assess intrarenal blood flow parameters in PPGL patients included in the prospective monoamine-producing tumour (PMT) study and to evaluate the effects of normalisation of catecholamine production after surgical treatment on long-term renal function. Materials and methods Seventy consecutive patients (aged 46.5 ± 14.0 years) with PPGL were included. Forty-eight patients from the PMT study cohort, matched for age, gender, blood pressure level and presence of hypertension, served as a control group. Renal artery doppler ultrasound spectral analysis included mean resistance index (RRI) and pulsatility index (PI). Forty-seven patients completed 12 months follow-up. Results There were no differences in renal parameters such as RRI, PI and kidney function between PPGL and non-PPGL patients as assessed by renal ultrasound, serum creatinine, eGFR and albumin excretion rate. No correlations between kidney function parameters, intrarenal doppler flow parameters and plasma catecholamines were observed in PPGL patients. At 12 months after surgery, no differences in creatinine level, eGFR, albumin excretion rate, RI and PI were found as compared to baseline results. Conclusions In contrast to patients with other forms of secondary hypertension, our study did not show differences in intrarenal blood flow parameters and renal function between PPGL and non-PPGL subjects. Intrarenal hemodynamics and renal function did not change after normalisation of catecholamine levels by surgical treatment.

Metabolism and secretion mechanism of catecholamine syndrome and related treatment strategies

: Catecholamine syndrome, also known as pheochromocytoma, is a kind of tumor that originate from adrenal or extra-adrenal chromaffin tissue, secreting a large amount of catecholamines. Some typical gene mutations such as VHL, RET and NF1 play roles in the development of pheochromocytoma. Besides, headache, sweating, and palpitations are the usual manifestations of pheochromocytoma. Most clinical symptoms such as hypertension, headache, sweating and palpitation are related to excessive catecholamine secretion. Though preoperative pretreatment with α-blockers was routine before pheochromocytoma surgery, it showed a lot of limitations because the blockage took effect after catecholamine releasing. Thus, blocking catecholamine production before its releasing shows great significance. For this purpose, in this review, we described the synthesis and metabolic process of catecholamines, the ion channels associated with catecholamine secretion and the influencing factors of the section of catecholamine. We then discussed the related treatment strategies which based on the metabolism and secretion of catecholamine to lay a foundation for the clinical medication of pheochromocytoma.

131I-metaiodobenzylguanidine and peptide receptor radionuclide therapy in pheochromocytoma and paraganglioma.

Pheochromocytomas and paragangliomas are rare tumors arising, respectively, from the adrenal medulla and extra-adrenal sympathetic or parasympathetic paraganglia. The main therapeutic objectives in case of metastatic disease are the reduction of tumor burden and the control of symptoms resulting from excessive catecholamine secretion. Treatment choices constitute not only a wait and see attitude, locoregional approaches, chemotherapy regiments but also radiopharmaceutical agents, and they should be discussed in a specialized multidisciplinary board. This review will briefly discuss the radiopharmaceutical modalities in patients with pheochromocytomas and paragangliomas (I-MIBG and PRRT). I-MIBG (Azedra) has received FDA approval for patients with iobenguane-scan-positive, unresectable, locally advanced or metastatic pheochromocytomas and paragangliomas who require systemic anticancer therapy, whereas peptide receptor radionuclide therapy using radiolabelled somatostatin analogues is currently performed in compassionate use, with very promising results. No prospective head-to-head comparison between the modalities has been conducted to date. Promising results have been reported for both radiopharmaceutical agents, mostly in the setting of retrospective series. No prospective head-to-head comparison between the modalities is yet available.