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Reviews in Cardiovascular Medicine Dec 2021Although many endocrine diseases can be associated with acquired cardiomyopathy and heart failure, conditions except hypothyroidism, hyperthyroidism,... (Review)
Review
Although many endocrine diseases can be associated with acquired cardiomyopathy and heart failure, conditions except hypothyroidism, hyperthyroidism, phaeochromocytoma-paraganglioma (PPGL), and primary hyperaldosteronism are rare. PPGL is a rare catecholamine-secreting neuroendocrine tumour arising from the adrenal gland in 80-85% or extra-adrenal chromaffin cells of the autonomic neural ganglia in the remainder. The annual incidence of PPGL is 3-8 cases per million per year in the general population. Catecholamine-induced cardiomyopathy (CICMP) has got a prevalence of 8-11% among patients with PPGL. Hypertension, either sustained or episodic, is present in the vast majority (95%) of PPGL patients. However, among patients with CICMP, hypertension is present only in 65% of cases and the classical triad of paroxysmal headache, sweating, and palpitation is present only in 4%. Based on the cardiac remodelling in response to endogenous catecholamine excess, PPGL patients might present with one of the three CICMPs, including dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), or Takotsubo cardiomyopathy (TCM). Regardless of the subtypes, all CICMPs have many features in common - a dramatic clinical presentation, reversible cardiomyopathy, similar repolarisation electrocardiography changes, mild-moderate cardiac biomarker elevation, and normal coronary arteries on coronary angiography. CICMP should be suspected in patients with non-ischaemic, non-valvular forms of cardiomyopathy, even in those without definite features of catecholamine excess. PPGL associated TCM should be suspected in all acute coronary syndrome (ACS) patients exhibiting pronounced blood pressure variability with no culprit lesions on coronary angiography. This article will provide a review of the various CICMPs, their pathophysiology, clinical features, and the management options.
Topics: Adrenal Gland Neoplasms; Cardiomyopathies; Catecholamines; Endocrinologists; Humans; Pheochromocytoma
PubMed: 34957765
DOI: 10.31083/j.rcm2204130 -
Dakar Medical 2003Pheochromocytoma is a catecholamine-secreting neoplasm of chromaffin tissue. The most common symptom is hypertension but there are incidentally discovered forms at... (Review)
Review
Pheochromocytoma is a catecholamine-secreting neoplasm of chromaffin tissue. The most common symptom is hypertension but there are incidentally discovered forms at imaging. From 1981 to 1998, the authors observed nine pheochromocytomas through three hospitals in Dakar. This study included seven male and two female patients. The average age was 31.61 years. Hypertension was present in 77% of the cases. Headache, palpitations and sweating were the authors most frequent symptoms, occuring in 55% of cases. Measurement of vanillyl mandelic acid level in six cases and urinary metanephrines in three cases made the diagnosis. There were six adrenal and three extra adrenal pheochromocytomas with two malignant tumors features. In our countries, measurement of urinary metanephrines and computed tomography processing may improve detection of pheochromocytomas.
Topics: Adrenal Gland Neoplasms; Adult; Female; Humans; Male; Middle Aged; Pheochromocytoma; Senegal
PubMed: 15770796
DOI: No ID Found -
Best Practice & Research. Clinical... Aug 2012Phaeochromocytomas and paragangliomas are neural crest-derived tumours. Autopsy studies indicate that relatively large numbers of these tumours remain undiagnosed during... (Review)
Review
Phaeochromocytomas and paragangliomas are neural crest-derived tumours. Autopsy studies indicate that relatively large numbers of these tumours remain undiagnosed during life. This may reflect non-specific signs and symptoms and low medical alertness in evaluating the clinical picture or it may reflect a silent clinical presentation - the subclinical phaeochromocytoma. The associated clinical picture depends on the capacity of the tumours to release catecholamines and sometimes biologically active peptides. Hypertension is the hallmark of catecholamine release, but the amount, type and pattern of catecholamine secretion is extremely variable. Some tumours have low or intermittent secretory activity, some produce mainly or solely dopamine, while others very rarely do not synthesize or release any catecholamines (non-secretory or non-functional tumours). Such tumours may present with mild or even absent signs and symptoms of catecholamine excess. Low secretory activity may reflect small tumour size or differences in secretory phenotypes associated with the biochemical and genetic background of the tumours. Tumours due to succinate dehydrogenase subunit B mutations are often subclinical, poorly differentiated, contain low amounts of catecholamines, and are usually malignant at diagnosis. Adrenoceptor desensitization can result in a subclinical presentation, even when catecholamine levels are high. Subclinical phaeochromocytomas are often discovered as incidentalomas during radiological procedures or during routine screening for phaeochromocytoma in carriers of mutations in one of the ten currently identified tumour susceptibility genes. Undiagnosed phaeochromocytomas, whether or not subclinical and even if biologically benign, may cause extremely deleterious consequences or even death, following abrupt release of catecholamines.
Topics: Adrenal Gland Neoplasms; Catecholamines; Humans; Pheochromocytoma
PubMed: 22863392
DOI: 10.1016/j.beem.2011.10.008 -
Annals of Clinical Biochemistry Jan 2018Phaeochromocytoma and extra adrenal paraganglioma are rare neuroendocrine tumours and have the potential to secrete adrenaline, noradrenaline and dopamine causing a... (Review)
Review
Phaeochromocytoma and extra adrenal paraganglioma are rare neuroendocrine tumours and have the potential to secrete adrenaline, noradrenaline and dopamine causing a myriad of clinical symptoms. Prompt diagnosis is essential for clinicians and requires a multidisciplinary specialist approach for the clinical and laboratory investigation, diagnosis, treatment and follow-up of patients. This paper is an integrated review of the clinical and laboratory evaluation and treatment of patients suspected to have phaeochromocytoma or paraganglioma, highlighting recent developments and best practices from recent published clinical guidelines.
Topics: Adrenal Gland Neoplasms; Follow-Up Studies; Humans; Paraganglioma; Pheochromocytoma; Practice Guidelines as Topic
PubMed: 29027806
DOI: 10.1177/0004563217739931 -
Journal of Neuro-oncology Jun 2005Intracranial pheochromocytomas are extremely rare tumors. Reported cases include metastatic tumors without known cases of primary pheochromocytomas.
OBJECTIVE AND IMPORTANCE
Intracranial pheochromocytomas are extremely rare tumors. Reported cases include metastatic tumors without known cases of primary pheochromocytomas.
CLINICAL PRESENTATION
A female patient with a history of a surgically treated adrenal pheochromocytoma presented 23 years later with headache, nausea and blood hypertension. A head CT scan demonstrated a right temporoparietal meningeal heterogeneous lesion with a surrounding hyperdense ring. No other lesions were disclosed.
INTERVENTION
The lesion developed in the inner and outer surface of the dura without brain infiltration and it was totally resected. The patient is free of disease 6 years after brain surgery.
CONCLUSION
To our knowledge this is the first reported case of a primary meningeal pheochromocytoma.
Topics: Adrenal Gland Neoplasms; Diagnosis, Differential; Female; Humans; Meningeal Neoplasms; Middle Aged; Neoplasms, Second Primary; Pheochromocytoma; Radiography; Treatment Outcome
PubMed: 15981108
DOI: 10.1007/s11060-004-4595-5 -
Acta Chirurgica Belgica 2010Phaeochromocytomas are catecholamine-secreting tumours that arise from chromaffin cells of the adrenal medulla and extra-adrenal sites. Extra-adrenal phaeochromocytomas... (Review)
Review
Phaeochromocytomas are catecholamine-secreting tumours that arise from chromaffin cells of the adrenal medulla and extra-adrenal sites. Extra-adrenal phaeochromocytomas are called paragangliomas. A diagnosis of phaeochromocytoma is suspected by typical paroxysmal symptoms, unusual or refractory hypertension, discovery of an adrenal incidentaloma or a family history of phaeochromocytoma or paraganglioma, possibly associated with other genetic syndromes (multiple endocrine neoplasia type 2 A or B, neurofibromatosis type 1 and von Hippel-Lindau disease). It can be confirmed by measurements of urinary or plasma fractionated catecholamines and metanephrines. The best diagnostic performances are achieved by metanephrines. Twenty-four hour urine fractionated metanephrines are still recommended as a screening test but some experts prefer plasma measurements in high-risk patients. Increased serum chromogranin-A levels, combined with high catecholamine or metanephrine in a patient with normal renal function is also a tool, virtually diagnostic of phaeochromocytoma. Recent studies have suggested that 25% of patients with phaeochromocytoma have germline mutations of several genes (NF1, VHL, SDHD, SDHB and RET). Thus, genetic testing should be carried out according to an algorithm of risk factors and specific characteristics. Once a biochemical diagnosis of phaeochromocytoma is made, a CT scan or MRI of the abdomen and pelvis should be performed first. If these investigations remain negative, the chest and neck should be explored. After anatomical imaging, functional imaging by 123I-MIBG should be considered. If the MIBG scan is negative, other imaging modalities have recently proven to be useful (PET, Octreoscan). After localization, the treatment of phaeochromocytoma is a surgical resection, which may be laparoscopic. Preoperative preparation with alpha- and beta-adrenergic blockade and/or calcium channel blockers associated with volume expansion is essential. Malignant phaeochromocytoma is rare and its treatment still unsatisfying. Phaeochromocytoma during pregnancy is also rare and its diagnosis easily missed because of its clinical resemblance to pre-eclampsia.
Topics: Adrenal Gland Neoplasms; Female; Humans; Pheochromocytoma; Pregnancy
PubMed: 20514823
DOI: 10.1080/00015458.2010.11680587 -
The Australian and New Zealand Journal... May 1988
Topics: 3-Iodobenzylguanidine; Adrenal Gland Neoplasms; Humans; Iodine Radioisotopes; Iodobenzenes; Pheochromocytoma; Radiography; Radionuclide Imaging
PubMed: 3178590
DOI: 10.1111/j.1445-2197.1988.tb01080.x -
Lancet (London, England) May 1959
Topics: Humans; Pheochromocytoma
PubMed: 13655679
DOI: No ID Found -
European Heart Journal. Cardiovascular... May 2023
Topics: Humans; Pheochromocytoma; Takotsubo Cardiomyopathy; Syndrome; Adrenal Gland Neoplasms
PubMed: 37014047
DOI: 10.1093/ehjci/jead053 -
Clinical Endocrinology Jan 2014Phaeochromocytoma [corrected] crisis is an endocrine emergency associated with significant mortality. There is little published guidance on the management of...
Phaeochromocytoma [corrected] crisis is an endocrine emergency associated with significant mortality. There is little published guidance on the management of phaeochromocytoma [corrected] crisis. This clinical practice update summarizes the relevant published literature, including a detailed review of cases published in the past 5 years, and a proposed classification system. We review the recommended management of phaeochromocytoma [corrected] crisis including the use of alpha-blockade, which is strongly associated with survival of a crisis. Mechanical circulatory supportive therapy (including intra-aortic balloon pump or extra-corporeal membrane oxygenation) is strongly recommended for patients with sustained hypotension. Surgical intervention should be deferred until medical stabilization is achieved.
Topics: Adrenal Gland Neoplasms; Adrenergic alpha-Antagonists; Humans; Pheochromocytoma; Treatment Outcome
PubMed: 24102156
DOI: 10.1111/cen.12324