Pheochromocytoma crisis with refractory Acute Respiratory Distress Syndrome (ARDS), Takotsubo syndrome, emergency adrenalectomy, and need for Extracorporeal Membrane Oxygenation (ECMO) in a previously undiagnosed and asymptomatic patient, due to the use of metoclopramide.

BACKGROUND: Pheochromocytoma (PCC) crisis is a rare life-threatening endocrine emergency. The diagnosis and treatment of PCC crisis, with acute respiratory distress syndrome (ARDS) as the first manifestation, is highly challenging, and traditional PCC management strategies are no longer suitable for these patients. CASE PRESENTATION: A 46-year-old female patient was admitted to the Intensive Care Unit (ICU) following sudden-onset acute respiratory distress and subsequent initiation of mechanical ventilation via endotracheal intubation. She was initially suspected of having a PCC crisis through the bedside critical care ultrasonic examination protocol. The computed tomography examination revealed a left adrenal neoplasm of 6.5cm × 5.9cm. The plasma-free metanephrine level was 100 times higher than the reference value. These findings were compatible with her PCC diagnosis. Alpha-blockers and fluid intake were started immediately. The endotracheal intubation was removed on the 11th day after admission to the ICU. The patient progressed to severe ARDS again, and invasive ventilation and continuous renal replacement therapy were needed. Despite aggressive therapy, her condition deteriorated. Therefore, she underwent veno-arterial extracorporeal membrane oxygenation (VA-ECMO)-assisted emergency adrenalectomy after multidisciplinary discussion. Postoperatively, the patient was supported by VA-ECMO for 7days. She was discharged from the hospital on day 30 after tumor resection. CONCLUSIONS: This case highlighted the challenges in diagnosing and managing ARDS associated with PCC crisis. The traditional preoperative preparation protocol and optimal operation timing for patients with PCC are not suitable for patients with PCC crisis. Patients with life-threatening PCC crisis may benefit from early tumor removal, and VA-ECMO could maintain hemodynamic stability during and after surgery.

Cardiac arrest as initial presentation of pheochromocytoma in a young.

We reported a case of pheochromocytoma with initial presentation of cardiac arrest. The patient underwent implantable cardioverter defibrillator for primary prevention but subsequently experienced repeated implantable cardioverter defibrillator shocks and syncopal episodes. A mass was found in the adrenal gland by CT, which was confirmed by anatomopathological analysis of the surgical specimen.

Cardiomyopathy due to pheochromocytoma.

Α 33-year-old woman was admitted to our clinic with electrocardiographic (ECG) manifestations of anterior ST-elevation myocardial infarction, dizziness, weakness, and feeling of oncoming collapse. She underwent coronary angiography that showed normal coronary arteries and an echocardiography study that showed reduced left ventricular contractility with diffuse hypokinesis and an estimated ejection fraction of (EF) 35-40 %. The biochemical testing showed enzymatic activity typical of myocardial necrosis. The patient had hypertensive peaks on a 24-h recording of blood pressure, while immunological and virological test results were negative. Magnetic resonance imaging showed partial epicardial enhancement. A tumor in the right adrenal medulla was detected with computed tomography, and biochemical testing showed increased levels of urinary vanillylmandelic acid as well as serum metanephrines. The diagnosis of pheochromocytoma was made and confirmed by histological findings after surgical resection of the tumor. The left ventricular systolic dysfunction gradually reversed, the EF returned to normal, and the ECG findings were normalized, indicating cardiomyopathy due to pheochromocytoma.

[A Case of Adrenergic Crisis Caused by Spontaneous Rupture of Cystic Pheochromocytoma].

Pheochromocytoma crisis is a life-threatening situation. Herein we report a case of catecholamineinduced crisis caused by the rupture of cystic pheochromocytoma. A 76-year-old man with hypertension was referred to our hospital because of a cystic tumor in the retroperitoneal space adjacent to the aorta, which was suspicious of pheochromocytoma. Two days after admission, lower abdominal pain suddenly appeared, followed by hypertension with systolic pressure of 260 mmHg. Computed tomography revealed that the cystic tumor was ruptured spontaneously, leading to diagnosis of pheochromocytoma crisis. His blood pressure was successfully managed by medical treatment and he could recover from crisis. After adequate medical preparation by an α-adrenergic blocker, the tumor was successfully removed by laparoscopy, though the adhesion around the tumor was severe. To our knowledge adrenergic crisis caused by spontaneous rupture of cystic pheochromocytoma is rare, but we have to keep in mind that cystic pheochromocytoma can cause life-threatening crisis by the release of catecholamine due to rupture.

Pheochromocytoma crisis presenting with cardiogenic shock.

Pheochromocytoma is a catecholamine-secreting tumor of the adrenal glands whose typical presentation includes the triad of headache, palpitations, and diaphoresis. Pheochromocytoma crisis is an urgent medical condition whose diagnosis and management constitute a challenge for physicians. We present the case of a 55-year-old man who developed cardiogenic shock in the setting of a pheochromocytoma crisis. After stabilizing blood pressure with combined administration of α- and ß-blockers, the tumor was surgically removed. Our diagnostic and therapeutic challenges are discussed.

Outcomes of concurrent Caesarean delivery and pheochromocytoma resection in late pregnancy.

Undiagnosed pheochromocytoma in pregnancy is associated with significant maternal and foetal mortality. Herein we reviewed five cases of pheochromocytoma in pregnancy occurring during late pregnancy. The mean age at presentation was 30.6 years, and the gestational age ranged from 26 to 36 weeks. All patients had elevated levels of urinary catecholamines. In three patients, the lesion was located in the adrenal gland, in one patient the urinary bladder, and there was one case of recurrent malignant pheochromocytoma with metastases. Tumour resections were performed at the time of Caesarean section in four patients, two through laparoscopy. When pheochromocytoma is diagnosed in pregnancy after the second trimester, concurrent tumour resection with Caesarean section is feasible and in our series was achieved in four cases without adverse maternal or foetal consequences.

[The observation of bilateral pheochromocytoma after unilateral adrenalectomy : a case report].

We report a case of bilateral pheochromocytoma which was incidentally discovered by ultrasonography at a health check-up. A 46-year-old female was admitted to our hospital for further examination of a right adrenal tumor. Computed tomography and magnetic resonance imaging revealed a right adrenal tumor, 5 cm in size, and a left adrenal tumor, 1.5 cm in size. High serum noradrenaline and urine noradrenaline levels were noted. 131I-MIBG scintigraphy revealed an abnormal accumulation of 131 I in the tumors. Thus, our clinical diagnosis was bilateral pheochromocytoma. Laparoscopic right adrenalectomy was performed. The histopathological examination revealed pheochromocytoma, no capsule injury and no malignancy. We decided to continue watchful waiting of the left adrenal tumor, because the serum and urine levels of catecholamine were within the normal range after the operation. She has been well with no clinical symptoms, no increase in tumor size, and no elevation of catecholamine for 20 months.

Secondary Hypertension Overview and Workup for the Primary Care Physician.

Secondary hypertension occurs in 5% to 10% of all patients with hypertension. Given the majority of patients with hypertension will not have a secondary cause, only select patients with specific characteristics should be screened. The causes include a range of abnormalities, some are quite rare, such as pheochromocytoma, while others are much more common, such as chronic kidney disease. When considering which disorders to test for, it is important to incorporate the clinical history, family history, and prevalence of each disease. Treatment is specific to the underlying cause and includes medications, procedures, surgery, and device therapies.

[Diabetes and prediabetes in endocrine disorders]. / Cukrzyca i stany przedcukrzycowe w chorobach gruczolów wydzielania wewnetrznego.

Complex hormonal regulation of carbohydrate metabolism causes that presence of many endocrine disorders may disturb glucose homeostasis. Impaired fasting glucose, impaired glucose tolerance and frank diabetes are observed in patients with both common and rare endocrine disorders, particularly in patients with polycystic ovary syndrome, hyperthyroidism, Cushing's syndrome, pheochromocytoma, primary aldosteronism, acromegaly, growth hormone deficiency and endocrine tumors of the digestive system. Because most of these disorders may be effectively treated and the treatment often results in a restoration of normal insulin secretion and receptor action as well as glucose absorption, production and metabolism, it is important to differentiate these disorders from other more common types of diabetes. This article reviews the etiology, clinical manifestation, diagnosis and management of endocrine disorders leading to diabetes and prediabetic states with special emphasis on the pathogenesis and clinical consequences of these disorders.

[Adrenal pheochromocytoma associated with "tako-tsubo" syndrome]. / Un caso di feocromocitoma surrenalico manifestatosi con un quadro di cardiomiopatia tipo "tako-tsubo".

"Tako-tsubo" cardiomyopathy is a condition characterized by a transitory left ventricular asinergia, induced by physical and emotional stress. In literature there are few cases of association between cardiomyopathy and pheochromocytoma. We described a case of a 72 year-old woman admitted in Emergency Room for chest pain associated with high blood pressure: the ECG showed non-ST elevation in leads III, AVF and V1. An echocardiogram showed global reduction in contractility (EF 40%) with apical akinesia. The coronary angiography showed coronary without stenosis while left ventriculography showed an average apical akinesia of the anterior wall with enhanced contractility of basal segments. Subsequently, the patient continued to present episodes of tremors associated with high blood pressure and therefore was made the determination of urinary metanephrines and urinary vanilmandelic acid that were both high. A subsequent abdomen MRI showed a 32 mm left adrenal lesion with arterial phase impregnation. The diagnosis of left adrenal pheochromocytoma was made and the tumor, after appropriate preoperative pharmacological preparation with α-blockers, was removed surgically. In conclusion, the unexplained transitory left ventricular asinergia alert the clinician of an underlying disorder, such as pheochromocytoma, the early detection of which is crucial to the prognosis.

Giant secretory multifocal adrenal myelolipoma: a diagnostic dilemma.

A 38-yearr-old man presented with erectile dysfunction and infertility. On examination, he was hypertensive and detected to have a left flank mass. Blood investigations were unremarkable except raised serum noradrenaline levels. Imaging revealed multiple well-defined fat-containing hypodense lesions in left suprarenal area with largest one measuring 14×16 cm, suggestive of left adrenal myelolipoma. Diagnostic dilemma was posed due to discordance between clinical, biochemical and imaging findings. Left adrenal mass resection was planned keeping the possibility of pheochromocytoma. However, histopathology revealed it to be adrenal myelolipoma. Hypertension was resolved in the postoperative period and serum noradrenaline levels were normalised. Final diagnosis of a secretary adrenal myelolipoma was made, which is an extremely rare entity.

Acute hypertensive crisis due to newly diagnosed pheochromocytoma in the ninth decade of life: an unusual presentation.

The patient is an 85-year-old man with multiple comorbidities, including hypertension and coronary artery disease with recent myocardial infarction who underwent cardiac catheterisation. During the procedure, the patient developed profound hypertensive crisis with flash pulmonary oedema, requiring significant intervention for blood pressure (BP) control. His crisis was also marked by wide excursions in his BP. The patient was found to have a large left adrenal mass measuring 9.4×8.7×8.1 cm, with biochemical testing confirming the suspicion of pheochromocytoma. Alpha-blockade was started prior to availability of lab results due to high index of suspicion. Surgical removal, the mainstay of treatment, has yet to transpire as he has no family, and due to his underlying dementia, he was not felt to have capacity for decision-making at the time of diagnosis. The case elucidates the vast presentations of this tumour, the means of diagnosis and the difficulties of treatment.

Pheochromocytoma crisis in a patient with newly diagnosed neurofibromatosis type 1.

Pheochromocytoma occasionally engenders catecholamine-induced hypertension crisis. Pheochromocytoma is clinically identified in 0.1%-5.7% of patients with neurofibromatosis type 1 (NF1), which is 10 times more frequently than in healthy individuals. This report describes a case of newly diagnosed NF1 presenting with pheochromocytoma crisis, with severe electrolyte depletion and deteriorating recurrent ventricular tachycardia storm. Characteristic skin lesions such as café-au-lait macules and neurofibromas contributed to the diagnosis of NF1 and pheochromocytoma. No recurrence of electrolyte depletion was found after the adrenalectomy. Primary care physicians must distinguish the characteristic skin lesions of NF1, such as café-au-lait macules and neurofibromas and recognise the risk for pheochromocytoma.

Surgical management of abdominal manifestations of type 1 neurofibromatosis: experience of a single center.

Neurofibromatosis type 1 (NF1) is a genetic disease characterized by neoplastic and nonneoplastic disorders involving tissues of neuroectodermal and mesenchymal origin. The mainly involved districts are skin, the central nervous system, and eye and there is a wide range of severity of clinical presentations. Abdominal manifestations of NF1 include five kinds of tumors: neurogenic tumors (neurofibromas, malignant peripheral nerve sheath tumors [MPNSTs], and ganglioneuromas); neuroendocrine tumors (pheochromocytomas and carcinoids); nonneurogenic gastrointestinal stromal tumors (GISTs); embryonal tumors; and miscellaneous. The present experience depends on the participation in the National Project for Diagnosis and Treatment of Rare Diseases. In the group of patients with a diagnosis of von Recklinghausen disease, 10 patients underwent surgical treatment for gastrointestinal and retroperitoneal tumors associated with NF1. Three patients underwent adrenalectomy for pheochromocytoma (in one case associated with jejunal wall neurofibroma); two patients were found to be affected by MPNST (recurrent and unresectable in one case). One patient was affected by giant gastric GIST and jejunal neurofibroma; two patients were affected by extraperitoneal neurofibroma (pararenal and pararectal position); one patient was affected by giant colic neurofibroma and one patient was affected by retroperitoneal bilateral plexiform neurofibromas. Early diagnosis of these abdominal manifestations is very important because of the risk of malignancy, organic complications (such as pheochromocytoma), or hemorrhagic-obstructive complications such as in case of tumors of the gastrointestinal tract (GISTs and neurofibromas). The importance of an annual clinical evaluation on the part of a multidisciplinary pool of clinicians in highly specialized centers allows early detection of complications and of neoplastic transformation. Genetic screening allows preclinical diagnosis with a sensibility of 95 per cent. Further studies are necessary to detect predictive factors of malignant tumor development of severe clinical conditions.

Pheochromocytoma presenting as hypertension in pregnancy.

Pheochromocytoma is a curable, rare cause of hypertension, characterized by symptoms and signs related to increased catecholamine secretion. Pregnancy and labour increase the risk of hypertensive crisis. However, antepartum diagnosis reduces both maternal and foetal mortality, allowing for safe cesarean section and resection of tumor. Control of hypertension with alpha blockers and beta blockers is the medical treatment. Surgical removal of the tumour is the definitive treatment. Hypertensive crisis needs to be treated aggressively. We report this case for the rare presentation of pheochromocytoma presenting as hypertension in pregnancy.

Pheochromocytoma during pregnancy treated by surgery. A case report and the review of the literature.

INTRODUCTION: Prenatal diagnosis of pheocromocytoma, although rare, is important as it allows a reduction in both maternal mortality and foetal loss. Pheocromocytoma operated on in the first trimester of pregnancy with survival of both patient and foetus is rare in literature. Our case was operated on with success after a correct and early diagnosis was obtained despite a chronic hypertension which existed long before pregnancy. Our case study well illustrates that a correct multidisciplinary approach involving endocrinologists, anesthesiologists, surgeons and gynecologists is fundamental for a positive outcome. CASE REPORT: The case of a white caucasian pregnant woman at 13th weeks of gestation with pheocromocytoma and severe and unstable hypertension that could not be pharmacologically controlled is described. Morphological diagnosis was safely obtained by Magnetic Resonance Imaging (MRI) without intravenous medium contrast agent. Pre-operative treatment consisted of therapy with alpha-blockers and rehydration. Adrenalectomy was performed through a laparotomy. Postoperative treatment consisted of rehydration and ephedrine continued until the fourth post-operative day. The post-operative period was uneventful and a new ultrasound (US) scan confirmed foetal vitality. The patient was discharged seven days after surgery. A live newborn was physiologically delivered after a nine-month pregnancy. CONCLUSION: A correct diagnosis in all pregnant women with severe hypertension particularly those not screened for secondary hypertension and a multidisciplinary management are mandatory to obtain optimal results and avoid deleterious effects at delivery.

[Case of bladder pheochromocytoma with familial clustering].

We report herein a rare female case of bladder pheochromocytoma with familial clustering. Her mother had received an operation for bladder pheochromocytoma. When the present case was 20 years of age, body weight loss and fever appeared. Thereafter, nausea, vomiting and palpitation occurred especially at urination, and hypertension and tachycardia emerged. She was referred to our hospital for a further check up of hypertension at the age of 28 years. Her blood pressure was 176/130 mmHg, and pulse rate, 103/min. Hemorrhage and hard exudate were observed at the optic fundi. Twenty-four-hour ambulatory blood pressure monitoring disclosed that her hypertension was characterized by non-dipper type features and transient increases in both blood pressure and pulse rate occurring, especially at urination. Plasma noradrenalin level (14,399 pg/mL)was remarkably elevated, although the plasma adrenalin level (52 pg/mL) was within the normal limits. Computed tomography (CT) showed a mass lesion (7 cm in diameter) with central necrosis in the urinary bladder. 123I-MIBG showed strong uptake in the mass detected by CT. Venous blood sampling disclosed that the plasma noradrenalin concentration was highest at the lower level of the inferior vena cava. Therefore, a diagnosis of bladder pheochromocytoma with familial clustering was made and the pheochromocytoma was surgically removed.