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Regulatory Peptides Nov 2010Pheochromocytomas are rare catecholamine-secreting tumors that arise from chromaffin tissue within the adrenal medulla and extra-adrenal sites. Typical clinical... (Review)
Review
Pheochromocytomas are rare catecholamine-secreting tumors that arise from chromaffin tissue within the adrenal medulla and extra-adrenal sites. Typical clinical manifestations are sustained or paroxysmal hypertension, severe headaches, palpitations and sweating resulting from hormone excess. However, their presentation is highly variable and can mimic many other diseases. The diagnosis of pheochromocytomas depends mainly upon the demonstration of catecholamine excess by 24-h urinary catecholamines and metanephrines or plasma metanephrines. Occurrence of malignant pheochromocytomas can only be asserted by imaging of metastatic lesions, which are associated with a poor survival rate. The characterization of tissue, circulating or genetic markers is therefore crucial for the management of these tumors. Proteins of the granin family and their derived peptides are present in dense-core secretory vesicles and secreted into the bloodstream, making them useful markers for the identification of neuroendocrine cells and neoplasms. In this context, we will focus here on reviewing the distribution and characterization of granins and their processing products in normal and tumoral chromaffin cells, and their clinical usefulness for the diagnosis and prognosis of pheochromocytomas. It appears that, except SgIII, all members of the granin family i.e. CgA, CgB, SgII, SgIV-SgVII and proSAAS, and most of their derived peptides are present in adrenomedullary chromaffin cells and in pheochromocytes. Moreover, besides the routinely used CgA test assays, other assays have been developed to measure concentrations of tissue and/or circulating granins or their derived peptides in order to detect the occurrence of pheochromocytomas. In most cases, elevated levels of these entities were found, in correlation with tumor occurrence, while rarely discriminating between benign and malignant neoplasms. Nevertheless, measurement of the levels of granins and derived peptides improves the diagnostic sensitivity and may therefore provide a complementary tool for the management of pheochromocytomas. However, the existing data need to be substantiated in larger groups of patients, particularly in the case of malignant disease.
Topics: Adrenal Gland Neoplasms; Chromaffin Cells; Chromogranins; Humans; Pheochromocytoma
PubMed: 20600356
DOI: 10.1016/j.regpep.2010.06.003 -
Advances in Experimental Medicine and... 2017Phaeochromocytomas and paragangliomas are relatively uncommon tumours which may be manifest in many ways, specifically as sustained or paroxysmal hypertension, episodes... (Review)
Review
Phaeochromocytomas and paragangliomas are relatively uncommon tumours which may be manifest in many ways, specifically as sustained or paroxysmal hypertension, episodes of palpitations, sweating, headache and anxiety, or increasingly as an incidental finding. Recent studies have shown that an increasing number are due to germline mutations. This review concentrates on the diagnosis, biochemistry and treatment of these fascinating tumours.
Topics: Adrenal Gland Neoplasms; Blood Pressure; Catecholamines; Genetic Predisposition to Disease; Humans; Hypertension; Paraganglioma; Phenotype; Pheochromocytoma; Predictive Value of Tests; Risk Factors; Treatment Outcome
PubMed: 27888488
DOI: 10.1007/5584_2016_76 -
Schweizerische Medizinische... Sep 2000Phaeochromocytoma is a rare tumour which produces a variety of symptoms. The most important factor is to think of the diagnosis, and there are many biochemical and... (Review)
Review
Phaeochromocytoma is a rare tumour which produces a variety of symptoms. The most important factor is to think of the diagnosis, and there are many biochemical and pharmacological tests as well as radiological procedures to confirm it. Once the phaeochromocytoma is localised, it should, if possible, be removed. Surgery is the treatment of choice. In 1972 Ross described the diagnosis and therapy as "think of it, confirm it, find it and remove it". Today, 28 years later, this paper reviews the diagnosis and therapy of phaeochromocytoma under these key headings.
Topics: Adrenal Gland Neoplasms; Humans; Hypertension; Pheochromocytoma
PubMed: 11045034
DOI: No ID Found -
The Journal of Urology Oct 1989From 1955 to 1985, 20 patients presented with a total of 22 extra-adrenal pheochromocytomas (2 had multiple tumors and 2 had a malignant extra-adrenal pheochromocytoma)....
From 1955 to 1985, 20 patients presented with a total of 22 extra-adrenal pheochromocytomas (2 had multiple tumors and 2 had a malignant extra-adrenal pheochromocytoma). There were 13 male and 7 female patients, and the highest incidence was in the second decade. Although most patients presented with symptoms typical of pheochromocytoma, several presented with unusual features related to the anatomical location, such as mediastinal mass (chest tumor), upper airway obstruction (neck tumor) or gross hematuria (bladder tumor). In 5 of 6 patients in whom plasma catecholamine levels were fractionated epinephrine levels were elevated. The most common tumor location was the superior para-aortic region (13 patients). In 16 patients the location of tumors was established before treatment. Computerized tomography (9 patients) was the most accurate imaging study for tumor localization. A total of 19 patients underwent complete excision of all pheochromocytomas. Postoperative followup information (median interval 120 months) was available for 15 of these patients. Three patients had recurrent pheochromocytoma that was treated successfully. One patient had essential hypertension. No patient had metastatic disease. The low incidence of malignancy suggests a benign course for extra-adrenal pheochromocytoma and represents a departure from the previously reported higher incidence of malignancy with extra-adrenal pheochromocytoma.
Topics: Adolescent; Adult; Aged; Angiography; Child; Female; Follow-Up Studies; Humans; Male; Middle Aged; Neoplasm Recurrence, Local; Pheochromocytoma; Tomography, X-Ray Computed
PubMed: 2795745
DOI: 10.1016/s0022-5347(17)38945-0 -
Clinics in Endocrinology and Metabolism Feb 1985Phaeochromocytomas are uncommon among patients with hypertension, and sometimes occur in persons without known hypertension, but are important to detect because they are... (Review)
Review
Phaeochromocytomas are uncommon among patients with hypertension, and sometimes occur in persons without known hypertension, but are important to detect because they are often lethal but commonly curable, and because they are a clue to the presence of associated conditions. Paroxysmal symptoms (especially headache, palpitations, diaphoresis and anxiety), hypertension that is intermittent, unusually labile or resistant to conventional therapy, and conditions known to be associated raise the clinical suspicion of phaeochromocytoma. Biochemical confirmation is commonly achieved by measurement of urinary catecholamines, metanephrines or VMA. Plasma noradrenaline and adrenaline measurements may be superior to measurements of urinary catecholamine metabolites, but strict attention to the details of sample collection, handling and storage, the many sources of possible biological variation and the effects of drugs is critical if diagnostic error is to be avoided. Patients should be evaluated in the drug-free state if at all possible. Anatomical localization, in the abdomen in the vast majority of cases and usually in the adrenal medullae, can generally be accomplished with computed tomographic scans. Bilateral adrenomedullary tumours are the rule in familial phaeochromocytoma. Most phaeochromocytomas are benign and can be excised totally after medical preparation with an alpha-adrenergic antagonist.
Topics: Adrenal Gland Neoplasms; Adrenal Glands; Blood Pressure; Chromaffin Granules; Chromatography, High Pressure Liquid; Clonidine; Electrochemistry; Epinephrine; False Negative Reactions; Glucose Tolerance Test; Humans; Metanephrine; Norepinephrine; Normetanephrine; Phentolamine; Pheochromocytoma; Sympathetic Nervous System; Tomography, X-Ray Computed; Vanilmandelic Acid
PubMed: 3893809
DOI: 10.1016/s0300-595x(85)80070-0 -
Clinics in Endocrinology and Metabolism Nov 1977Factors involved in the evaluation and care of patients with phaeochromocytoma have been discussed with respect to important considerations for the consulting or... (Review)
Review
Factors involved in the evaluation and care of patients with phaeochromocytoma have been discussed with respect to important considerations for the consulting or practising physician. Historical, physical, biochemical and other diagnostic procedures, as well as therapeutic manoeuvres have been adequately documented so that the clinician requiring additional information in depth may seek out the pertinent literature. Utilizing this manner of approach should significantly improve the care of patients with phaeochromocytoma in the hands of physicians who have not themselves had extensive experience with this disease. However, it must be emphasized that because of the potential gravity of this condition, if the physician feels insecure in the care of a patient or has further questions, he should not hesitate to seek expert advice which will benefit both the patient and himself.
Topics: Adrenergic alpha-Antagonists; Basal Metabolism; Blood Pressure; Catecholamines; Diagnosis, Differential; Humans; Pheochromocytoma; Thyroid Neoplasms
PubMed: 22415
DOI: 10.1016/s0300-595x(77)80079-0 -
Surgery Dec 1983During a 15-year period, 24 patients were treated operatively for pheochromocytoma at this medical center. In this group a father and three daughters were recognized to...
During a 15-year period, 24 patients were treated operatively for pheochromocytoma at this medical center. In this group a father and three daughters were recognized to have the familial variety of this disease. Malignant hypertension, which began at an early age, was the most prominent characteristic. Operative treatment of the three sisters occurred at the ages of 9, 15, and 17 years. All pheochromocytomas were bilateral and were confined to the adrenal glands except for hepatic extension in the father. In contrast to most recent reports of familial pheochromocytoma, there was no evidence of multiple endocrine adenomatosis in this family. Total adrenalectomy was performed in only the youngest patient and, although normotensive, she requires close observation and steroid replacement. Three patients underwent a controversial partial adrenalectomy after excision of bilateral tumors. The father has remained hypertensive for 8 years and has evidence of a residual tumor in the liver. The two daughters who have had subtotal adrenalectomies remain normotensive, require no medication, and have normal urine catecholamine values 7 and 3 years after operation. These results show that removal of bilateral pheochromocytomas without total adrenalectomy may allow normal adrenal function free of recurrence for several years in patients without multiple endocrine adenomatosis.
Topics: Adolescent; Adrenal Gland Neoplasms; Adult; Female; Humans; Male; Pedigree; Pheochromocytoma
PubMed: 6648808
DOI: No ID Found -
Medicine Jan 2021Phaeochromocytomas are catecholamine-producing neuroendocrine tumors that may manifest in many ways, specifically as sustained or paroxysmal hypertension. Data,...
Phaeochromocytomas are catecholamine-producing neuroendocrine tumors that may manifest in many ways, specifically as sustained or paroxysmal hypertension. Data, including data from mental status screening, were prospectively collected from suspected patients. The Hospital Anxiety and Depression Scale was used as a screening tool to identify abnormal mental status. Results showed phaeochromocytoma patients were more likely to experience anxiety and depression. For future phaeochromocytoma treatment, early screening for anxiety and depression should be recommended.
Topics: Adrenal Gland Neoplasms; Adult; Aged; Anxiety; Case-Control Studies; China; Depression; Humans; Logistic Models; Middle Aged; Pheochromocytoma; Psychometrics; Translating
PubMed: 33546066
DOI: 10.1097/MD.0000000000024335 -
Journal D'urologie 1989Between 1971 and 1987, 60 patients presenting 68 pheochromocytomas were operated on in the University Teaching Hospital of Lille. Diagnosis has been transformed by the... (Review)
Review
Between 1971 and 1987, 60 patients presenting 68 pheochromocytomas were operated on in the University Teaching Hospital of Lille. Diagnosis has been transformed by the use of CT scan, which for the last 10 years has enabled the lesion to be more reliably localized than by MIBG scintigraphic scanning. Combination of the two investigations enables the diagnosis to be confirmed in 100% of cases. The following topics are covered: preparation for surgery with the indications for alpha and beta blockers, route of approach--preferably median laparotomy--and operative sequelae as a function of the benign or malignant nature of the tumor.
Topics: Adrenal Gland Neoplasms; Female; Humans; Male; Methods; Pheochromocytoma; Postoperative Complications; Premedication; Tomography, X-Ray Computed
PubMed: 2677154
DOI: No ID Found -
Journal of the National Medical... Feb 2021Dopamine-secreting pheochromocytomas are exceedingly rare.
BACKGROUND
Dopamine-secreting pheochromocytomas are exceedingly rare.
CASE PRESENTATION
A 28-year-old woman, who was admitted due to 4 hours of acute-onset abdominal pain, detected an adrenal mass incidentally. She was almost asymptomatic without a known family history. Laboratory assessments showed significant increases in dopamine levels of serum and 24-h urinary. By using preoperative a-adrenergic receptor blockers, she developed orthostatic hypotension and palpitations. When she underwent laparoscopic left adrenalectomy, she experienced rapid cyclic fluctuations in systolic blood pressure from 90 mmHg to 200 mmHg. Postoperatively, she exhibited prolonged hypotension, requiring vasopressor therapy and fluid replacement. According to histopathological diagnosis, it was a pheochromocytoma. Dopamine levels in 24-h urine and serum decreased to normal after operation. Analysis of specific gene SDHB, SDHD, RET, VHL and NF1 detected no pathogenic mutations.
CONCLUSION
Patients with dopamine-secreting pheochromocytomas are mostly asymptomatic, leading to a significant delay in diagnosis. There is a large possibility for dopamine-secreting pheochromocytomas to show a malignant tendency than the adrenergic and noradrenergic phenotypes. The a-adrenergic receptor blocker is not indicated for preoperative medical treatment because it can cause hypotension and cardiovascular failure. Calcium channel blockers or metyrosine may be better alternatives. All patients with pheochromocytomas should receive targeted genetic testing based on specific clinical features. SDHB, SDHD, RET, VHL and NF1 mutations are suggested for genetic testing of adrenal dopamine-secreting pheochromocytomas.
Topics: Adrenal Gland Neoplasms; Adrenalectomy; Adult; Dopamine; Female; Humans; Pheochromocytoma
PubMed: 32741578
DOI: 10.1016/j.jnma.2020.07.005