Pheochromocytoma-induced Subarachnoid and Intracerebral Hemorrhage.

Pheochromocytomas are rare adrenal tumors that are often diagnosed in workup for endocrine causes of refractory hypertension, as an incidental imaging finding, or in patients with classic symptoms of headache, palpitations, and/or diaphoresis. We describe a case of pheochromocytoma presenting in a 63-year-old woman with spontaneous and multifocal subarachnoid and intracerebral hemorrhage without underlying vasculopathy. The patient previously had no documented episodes of hypertension and took no regular medications. She experienced sudden-onset severe headache and presented with hypertensive crisis. Cranial imaging showed bifrontal and right temporal convexal subarachnoid and intracerebral hemorrhage of unknown etiology. Cranial arterial catheterization showed no vascular malformation underlying the site of hemorrhage. Given concern for potential malignant etiology, cross-sectional body imaging was performed that revealed a 7-cm right adrenal heterogeneous mass. Biochemical workup demonstrated markedly elevated plasma metanephrine and normetanephrine levels, diagnostic of pheochromocytoma. She underwent α- and ß-blockade, and evaluation with a multidisciplinary team including repeat intracranial imaging to ensure resolution of the intracranial bleeding before definitive surgical management. She then underwent successful laparoscopic adrenalectomy. This case demonstrates that the workup of cryptogenic intracranial hemorrhage and hypertensive crisis should include evaluation for catecholamine-secreting tumors.

A Clinical Case of Pheochromocytoma Presenting with Decompensated Diabetes Mellitus and Paroxysmal Hypertension

Introduction: Pheochromocytoma is a rare neuroendocrine tumor that originates from the adrenal medulla, less commonly from extraadrenal chromaffin cells (paraganglioma). In about 90% of cases, the tumor produces abnormal amounts of catecholamines. Pheochromocytomas are usually benign, but in rare cases can be malignant. Typical clinical manifestations are the result of the haemodynamic and metabolic effects of catecholamines and usually include paroxysmal hypertension with the classic triad (headache, excessive sweating, palpitations), carbohydrate disorders, etc. Elevated levels of catecholamine metabolites (metanephrine and normetanephrine) tested in plasma or in 24-hour urine confirm the diagnosis. Surgical removal of the tumor is the only radical treatment. Follow-up of patients postoperatively should be lifelong and performed by a multidisciplinary team in a specialized center of expertise. Case report: A 36-year-old female patient referred to the clinic for decompensated diabetes mellitus. Detailed history revealed paroxysmal hypertension and the classic triad of pheochromocytoma. The diagnosis was confirmed by high urinary metanephrine levels and an abdominal CT scan, showing a tumor in the right adrenal gland with features typical of pheochromocytoma. Surgical removal of the pheochromocytoma and normalization of catecholamine levels led to normalization of blood pressure and reversal of diabetes mellitus. Conclusion(s): Pheochromocytoma is a difficult diagnosis in endocrinology practice as it can mimic many other diseases. Early detection and surgical removal of the tumor are crucial to avoid complications caused by elevated serum catecholamine levels.Copyright © 2022 Medical Information Center. All rights reserved.

Acute Myocarditis: Secondary to Viral Infection or Pheochromocytoma?

Background In a young healthy patient, acute cardiogenic shock with a dilated, thickened left ventricle is strongly suggestive of acute myocarditis. Case SM is a 33 year-old healthy man who presented with decompensated heart failure with severe hypervolemia. Notably, he was exposed to Hand-Foot-Mouth disease (HFMD) two weeks prior. B-type natriuretic peptide was elevated at 3,417 pg/mL (normal range < 50 pg/mL), and troponin was elevated. Echocardiogram revealed dilated, severe systolic dysfunction with thickened left ventricular walls. He progressed to cardiogenic shock and multi-organ failure. Right heart catheterization revealed significantly reduced cardiac output and index of 2.36 and 1.2, respectively. His course was complicated by left ventricular thrombus and subacute embolic stroke, acute renal failure and liver failure. He was treated with afterload reduction, inotropes, and diuresis. His shock resolved, and he improved with medical therapy for cardiomyopathy. Decision-making The clinical course is consistent with acute myocarditis leading to cardiogenic shock with multi-organ failure. A broad differential was considered, including viral etiologies, autoimmune diseases, vasculitis, and toxin-mediated myocarditis. Viral labs including COVID-19 and influenza, as well as HIV, and hepatitis B and C viruses were negative. Coxsackie B2 antibody was positive at 1:80, which is consistent with past or current infection. Rheumatology evaluation was unrevealing, and vasculitis was deemed unlikely given normal inflammatory markers. Urine drug screen was unrevealing. However, adrenergic myocarditis remained on the differential given an adrenal nodule noted on imaging. Plasma free metanephrines were significantly elevated, consistent with pheochromocytoma. Conclusion This is a case of acute myocarditis with two likely etiologies. The patient's presentation correlates temporally with exposure to HFMD, suggesting viral myocarditis. However, he had gross hypervolemia and diuresed 50 pounds, which suggests a more indolent course. We propose that he had adrenergic myocarditis and undetected cardiomyopathy which was exacerbated by a second insult, the Coxsackie virus.Copyright © 2023 American College of Cardiology Foundation

Acute Pulmonary Edema as a Cardiovascular Manifestation of Pheochromocytoma.

Pheochromocytoma most commonly presents with the triad of paroxysms of headache, palpitations, and diaphoresis. Pheochromocytoma crisis, caused by a supra-physiological surge of catecholamine release, is an endocrine emergency that can present with various clinical manifestations. Acute pulmonary edema is one of the manifestations of pheochromocytoma crisis and can be either cardiogenic or non-cardiogenic. Here, we report cases of acute pulmonary edema of each type, related to pheochromocytoma crisis, which were presented to our district general hospital in 2020.

Comprehensive analysis of the novel omicron receptor AXL in cancers.

The SARS-CoV-2 is constantly mutating, and the new coronavirus such as Omicron has spread to many countries around the world. Anexelekto (AXL) is a transmembrane protein with biological functions such as promoting cell growth, migration, aggregation, metastasis and adhesion, and plays an important role in cancers and coronavirus disease 2019 (COVID-19). Unlike angiotensin-converting enzyme 2 (ACE2), AXL was highly expressed in respiratory system cells. In this study, we verified the AXL expression in cancer and normal tissues and found AXL expression was strongly correlated with cancer prognosis, tumor mutation burden (TMB), the microsatellite instability (MSI) in most tumor types. Immune infiltration analysis also demonstrated that there was an inextricable link between AXL expression and immune scores in cancer patients, especially in BLCA, BRCA and CESC. The NK-cells, plasmacytoid dendritic cells, myeloid dendritic cells, as one of the important components of the tumor microenvironment, were highly expressed AXL. In addition, AXL-related tumor neoantigens were identified and might provide the novel potential targets for tumor vaccines or SARS-Cov-2 vaccines research in cancer patients.

Successful diagnosis and treatment of pheochromocytoma during severe coronavirus disease 2019 (COVID-19): a case report.

Pheochromocytoma is a rare but life-threatening condition due to catecholamine release induced by drug treatments such as ß-blockers or glucocorticoids. We present a case of hypertensive crisis due to pheochromocytoma, induced after the initiation of dexamethasone and landiolol during intensive care for severe coronavirus disease 2019 (COVID-19). Based on a detailed medical history review, the patient was previously diagnosed with primary aldosteronism by confirmatory tests, moreover, an abdominal computed tomography scan identified an adrenal tumor 2 years before current admission. We tentatively diagnosed the patient with pheochromocytoma and initiated α-blockers without conducting a catecholamine report, leading to stable hemodynamics. We present a successfully managed case of pheochromocytoma concomitant with COVID-19, which has become a global crisis.

Pheochromocytoma triggered by coronavirus disease 2019: a case report.

BACKGROUND: Coronavirus disease 2019 is an infectious disease with many presentations, and many of its effects on the human body are still unknown. Pheochromocytoma is a neuroendocrine tumor that may occur sporadically or be a manifestation of a hereditary disease line multiple endocrine neoplasia type 2. CASE PRESENTATION: In this study, we report a case of an Iranian patient infected with coronavirus disease 2019, causing unusual presentations of pheochromocytoma, including myocarditis and cerebrovascular involvement. CONCLUSIONS: We discovered a case of pheochromocytoma as an unusual presentation of COVID-19. In further investigations we also discovered thyroid medullary carcinoma and at the end MEN 2 syndrome was diagnosed. After proper treatment many symptoms were eliminated.

Type A aortic dissection and pheochromocytoma: an indirect consequence of the Covid-19 pandemic.

Type A aortic dissection is a cardiovascular emergency. Its incidence seems to have increased in the last few years; it is not clear whether this is a consequence of the ageing population or better awareness of the diagnosis (Erbel R, Alfonso F, Boileau C, Dirsch O, Eber B, Haverich A et al.; Task Force on Aortic Dissection, European Society of Cardiology. Diagnosis and management of aortic dissection Task Force on Aortic Dissection, European Society of Cardiology. Eur Heart J 2001;15;22:1642-81). Acute type A aortic dissection is often lethal without urgent surgical treatment with mortality rates of around 17% (Conzelmann LO, Weigang E, Mehlhorn U, Abugameh A, Hoffmann I, Blettner M et al. Mortality in patients with acute aortic dissection type A: analysis of pre- and intraoperative risk factors from the German Registry for Acute Aortic Dissection Type A (GERAADA). Eur J Cardiothorac Surg 2016;49:e44-e52). Pheochromocytomas are rare tumours, though often asymptomatic, they could be lethal if left untreated. The incidence is around 0.6 per 100,000 persons per year. The association of both aortic dissection and pheochromocytoma is rare. Here, we report a case of a 36-year-old patient with pheochromocytoma and hypertension, whose delay of surgery due to the Covid-19 pandemic led to acute type A aortic dissection.

Impact of COVID-19 on individuals with paraganglioma/pheochromocytoma history and/or hereditary risk

Background: Patients with paraganglioma/pheochromocytoma (PPGL) or hereditary predisposition to PPGL often need screening with biochemical labs, imaging and physical exam. Given the rarity of PPGL and hereditary PPGL, care is often provided through specialty centers. Subsequently, patients may have experienced restrictions on travel and delayed scheduling of non-elective procedures due to COVID-19. This study aimed to analyze the impact of COVID-19 on seeking PPGL management. Methods: Patients with a personal history of PPGL or hereditary PPGL risk from the University of Michigan, Brigham Women's Hospital, and Huntsman Cancer Institute were sent a survey in 2021. The survey included questions regarding tumor history (Y/N), gene status, demographics, and experience with COVID. The survey assessed whether they missed any exams related to PPGL diagnosis or screening. Comparative analyses utilized regression and chi-square tests. Patient factors measured in analyses evaluated COVID surveillance (labs, imaging, doctor visit) as the primary outcome and age, institution, gene status, sex, and PPGL history as predicting variables. Results: In total, 241 respondents across three institutions completed the survey. The cohort was primarily female (n = 158, 65.6%). A majority of the cohort identified as White (n = 222, 92%) and non-Hispanic (n = 226, 93.8%). PPGL history was reported in 158 patients (65.6%), 43 of which were pheochromocytoma and 113 were paraganglioma, primarily in the head and neck (n = 78). At time of survey completion, 209 (87%) respondents answered COVID-related questions. Thirty-nine respondents (19.2%) reported missing doctor visits, while 31 (15.3%) report missing HPPGL imaging and 33 (16.3%) report missing lab tests. There were no differences by institution (p > 0.05) on patient reported missed visits. Logistic regression analysis showed no difference in missing visits based on having a hereditary PPGL predisposition gene or sex of respondent (all p-values > 0.05). There was no difference based on PPGL history, though it is unknown if patients missed PGL follow-up or screening. Individuals who missed imaging (Y/N) were more likely to report missing their lab tests (OR = 1.8, p < 0.01) and doctor visit (OR = 1.25, p < 0.01). Age was a significant predictor for missing doctor visits (p = 0.02) with an odds ratio of 1.002 per 1 year increase in age. Conclusions: Though institutions had different COVID-19 restrictions and guidelines by state, there was no difference on missing surveillance or screening. Over 15% of respondents reported missing at least one aspect of PPGL care, indicating a need to re-engage those with PPGL and hereditary PPGL to return to typical screening and surveillance. Patients who miss one aspect of surveillance are likely to have missed other aspects of surveillance and will require evaluation of all aspects of screening to return up to date on needed visits and procedures.

METASTATIC BLADDER PARAGANGLIOMA WITH UNDERLYING SHDB MUTATION

Introduction Phaeochromocytoma and paraganglioma (PPGL) are rare tumors with up to 40% associated with inherited germline mutations. SHDB mutation is associated with an increased risk of metastasis. Case A 36-year-old male presented with hypertensive emergency. He was diagnosed to have a bladder paraganglioma at age 32 when he presented with hypertensive crisis. Ga-68 DOTANOC PET/CT scan then showed a localized 4.7 x 5.3 cm bladder paraganglioma and he underwent complete surgical resection with resolution of his symptoms. Genetic testing done showed SHDB, deletion (exon 1), heterogenous pathogenic variant. He remained asymptomatic and was lost to follow-up due to COVID-19 until his recent admission. During this admission, he had labile blood pressure with symptoms of palpitations and lethargy. He was found to have a 4.3x elevated urine normetanephrine (1639 ug/day, N<374.7). Metanephrine and 3-methoxytyramine levels were normal. His blood pressure was controlled with phenoxybenzamine 20 mg TDS (1 mg/kg), telmisartan 40 mg OM and carvedilol 25 mg BD with improvement in his symptoms. Subsequent anatomical imaging with CT and functional imaging with Ga-68 DOTATATE showed a small recurrence at the bladder wall with metastatic lesions at the left sacral ala measuring 4.5 x 5.1 cm, and multiple lytic lesions over the spine, ribs and also the left acetabulum with the highest uptake of Ga-68 DOTATATE at the C2 vertebra (SUV max 93). He is now planned for peptide receptor radionuclide therapy (PRRT). SHDB mutation is associated with a higher risk of metastatic disease which has remained unexplained. Treatment for metastatic disease include surgical resection where possible, targeted therapy such as PRRT, meta-iodobenzylguanidine (MIBG) therapy, radiotherapy and also systemic therapy such as chemotherapy and tyrosine kinase inhibitors. Conclusion Patients with PPGL, especially those with SHDB mutation, require monitoring at regular intervals to screen and detect metastasis to reduce mortality and morbidity.

Anaesthetic management of adrenal tumours presenting with combination of rare syndromes during a pandemic: Experience from a tertiary cancer care centre in Northeast India

Background and Aims: Perioperative management of functional adrenal tumours is resource intensive. Due to the covid 19 pandemic, there has been sizeable delay in preparing and conducting these time sensitive surgeries.Quality of care andresource utilisation may worsen. This retrospective review was aimed at finding if care quality deteriorated due to covid 19 related restrictions, from anaesthetic perspective. Methods:Three cases of hormone-secreting adrenal tumours operated during a two year period in a tertiary cancer centre in India were retrospectively reviewed.Summary of the demographic profile, tumour characteristics, and the perioperative care were described using tables and analysed in the discussion. Results: Out of the three cases operated for adrenal gland tumour, pheochromocytoma tumour type with distant metastasis had prolonged hospital stay. One patient developed covid 19 infection in hospital. Cases were adequately managed during the perioperative period and the covid 19 related constraints didn't affect the quality of care. Conclusion:As in any other major surgery, adhering to a unique checklist, multidisciplinary approach, clear communication, knowledge sharing and establishing a care pathway helps to maintainquality care in high risk cases and at times of crisis.

Role of Dexmedetomidine in Perioperative Management of Pheochromocytoma

The prevalence of Pheochromocytoma in pat ient with hypertension is 0.1 -0.6%. These types of tumours are known for unpredictable perioperative course and hemodynamic instability. Various different drugs and anaesthesia techniques can be used to tackle these situations. Dexmedetomidine is emerged as newer agent with better hemodynamic stability, reducing requirement of other anaesthesia drugs, blunting of sympathoadrenal response in resection of Pheochromocytoma. We report four cases operated between January 2021 to June 2021.Preoperative preparation was done with α and β blockade. Dexmedetomidine was used during induction as 1 mcg/kg over 10 mins followed by 0.7mcg/kg/hr intraoperatively. Combination of Dexmedetomidine, Fentanyl, NTG, Isoflurane and Epidural analgesia was used. IF needed boluses of Esmolol and Labetalol were used during tumor manipulation. All the patients had an uneventful perioperative course. Dexmedetomidine with pre-operative α and β blockade reduce the need of other drugs intraoperatively and can be used as anaesthetic adjunct to maintain steady hemodynamic.

Management of Pheochromocytomas and Paragangliomas: A Case-Based Review of Clinical Aspects and Perspectives.

Paraganglioma and pheochromocytoma are rare medical conditions. Thus, there are still a small number of studies, clinical trials, and evidence-based data in this field. This makes clinical decisions more difficult. In this study, we present a case report enriched with a short review of available essential clinical data, indicating the need for constant metoxycatecholamine level observation and a proper diagnostic imaging approach, especially in terms of ongoing pandemics. Our research also provides a summary of the molecular background of these diseases, indicating their future role in clinical management. We analyzed the ClinicalTrials.gov dataset in order to show future perspectives. In this paper, the use of the PET-CT before MRI or CT is proposed in specific cases during diagnosis processes contrary to the guidelines. PET-CT may be as effective as standard procedures and may provide a faster diagnosis, which is important in periods with more difficult access to health care, such as during the COVID-19 pandemic.

26 Congresso Nazionale della Societa Scientifica FADOI

The proceedings contain 316 papers. The topics discussed include: usefulness of NEWS and NEWS-c in predicting dismal outcomes in acute medical unit: a lesson from CoViD-19 pandemic;Efficacy and safety of anti SARS-CoV-2 monoclonal antibodies treatment in the real world;incidental pheochromocytoma: is it really silent a case series;the mesentery as an uncommon site of involvement of IgG-related disease, a rare autoimmune disorder;Risk of venous and arterial thromboembolic events in women with advanced breast cancer treated with CDK 4/6 inhibitors: a systematic review and meta-analysis;nine-year efficacy and safety of azathioprine treatment in the maintenance of steroid-free remission in inflammatory bowel disease patients;helmet CPAP in severe CoViD-19: an experience in an internal medicine ward;disease knowledge and self-care in patients with chronic venous leg ulcers: preliminary short-term results of a randomized controlled study;and Casirivimab-imdevimab combination therapy for inpatients with early diagnosis of hospital-acquired CoViD-19: a single center experience.

Pheochromocytoma presenting as fulminant myocarditis mimicking COVID-19 pneumonia.

Adrenergic cardiomyopathy is uncommon but can be fulminant and life-threatening. Nowadays, the need to exclude the possibility of COVID-19 pneumonia in patients with acute dyspnea in a previously healthy adult may cause a delay in the diagnosis.

Johnson and Johnson COVID-19 Vaccination Triggering Pheochromocytoma Multisystem Crisis.

Pheochromocytomas are rare tumors that may have variable presentations. The presentation may depend on the type of catecholamine secreted, whether there is a paraneoplastic syndrome or not, or some other factor which may not be well understood. One rare presentation is a pheochromocytoma multisystem crisis. Many of these tumors are asymptomatic and found incidentally, but some can be triggered after being previously dormant. In this case report, we describe the first case of pheochromocytoma multisystem crisis triggered by the Johnson and Johnson (J&J) coronavirus disease 2019 (COVID-19) vaccine. We describe a case of a 63-year-old Caucasian male who presented with intractable nausea, vomiting, dyspnea, watery diarrhea, chills, sweats, and heavy chest pain starting one day status post J&J COVID-19 vaccination. He had no symptoms prior to this and no significant past medical history besides daily marijuana use. During his hospital stay, he had persistent high fevers, respiratory failure, cardiogenic shock, cardiomyopathy, and labile blood pressure measurements. After a retroperitoneal ultrasound, he was found to have a 7 cm mass in the right adrenal gland with elevated chromogranin A, urine vanillylmandelic acid (VMA), and urinary 24-hour metanephrines to confirm the diagnosis of a pheochromocytoma.

Inherited/Genetically-Associated Pheochromocytoma/ Paraganglioma Syndromes and COVID-19.

In some subjects with inherited pheochromocytoma/paraganglioma (PPG) syndromes, hypoxia-inducible factor 1 alpha (HIF1α) stabilization/activation could lead to an increase in angiotensin converting enzymes (ACE). This would result in the stimulation of angiotensin (AT) II production and, hence, reduce the availability of ACE 2. The latter would provide decreased numbers of binding sites for the spike protein of SARS-CoV-2 and, therefore, result in less points of viral entry into cells. Thus, subjects with HIF1α-associated PPG syndromes may benefit from an inherent protective effect against COVID-19. Such an implication of HIF1α vis-à-vis COVID-19 could open ways of therapeutic interventions.

Catecholaminergic Crisis After a Bleeding Complication of COVID-19 Infection: A Case Report.

The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) presents in some cases with hemostatic and thrombotic complications. Pheochromocytomas are unusual, though potentially lethal tumors. Herein we describe the first case of hemorrhage in a pheochromocytoma related to SARS-CoV-2 infection. A 62-year-old man consulted for syncope, fever, and palpitations. He was diagnosed with SARS-CoV-2 pneumonia and presented with a hemorrhage in a previously unknown adrenal mass, which resulted in a catecholaminergic crisis. Medical treatment and surgery were required for symptom control and stabilization. We hereby alert clinicians to watch for additional/unreported clinical manifestations in COVID-19 infection.

Management of 3 Cases of Pheochromocytoma During the COVID-19 Pandemic in New York City: Lessons Learned.

The COVID-19 crisis placed a pause on surgical management of nonemergency cases of pheochromocytoma, and it was essential for endocrinologists to provide both resourceful and safe care. At the Mount Sinai Hospital in New York City during the peak of the pandemic, we encountered 3 patients with pheochromocytoma and mild symptoms that were medically managed for a prolonged period of time (7-18 weeks) prior to adrenalectomy. Patients were monitored biweekly via telemedicine, and antihypertensive medications were adjusted according to signs, symptoms, and adrenergic profiles. These cases demonstrate that prolonged medical management prior to surgery is feasible and effective in pheochromocytoma patients with mild symptoms and well-controlled blood pressures.