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Endocrine Regulations Jul 2019Pheochromocytomas are rare tumors originating in the adrenal medulla. They may be sporadic or in the context of a hereditary syndrome. A considerable number of... (Review)
Review
Pheochromocytomas are rare tumors originating in the adrenal medulla. They may be sporadic or in the context of a hereditary syndrome. A considerable number of pheochromocytomas carry germline or somatic gene mutations, which are inherited in the autosomal dominant way. All patients should undergo genetic testing. Symptoms are due to catecholamines over production or to a mass effect. Diagnosis is confirmed by raised plasma or urine metanephrines or normetanephrines. Radiology assists in the tumor location and any local invasion or metastasis. All the patients should have preoperative preparation with α-blockers and/or other medications to control hypertension, arrhythmia, and volume expansion. Surgery is the definitive treatment. Follow up should be life-long.
Topics: Adrenal Gland Neoplasms; Adult; Child; Female; Genetic Predisposition to Disease; History, 20th Century; History, 21st Century; Humans; Male; Pheochromocytoma; Pregnancy
PubMed: 31517632
DOI: 10.2478/enr-2019-0020 -
Journal of Hypertension Dec 2006Pheochromocytomas are rare, mostly benign catecholamine-producing tumors of chromaffin cells of the adrenal medulla or of a paraganglion. Typical clinical manifestations... (Review)
Review
Pheochromocytomas are rare, mostly benign catecholamine-producing tumors of chromaffin cells of the adrenal medulla or of a paraganglion. 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. If remaining unrecognized or untreated, they can be a life-threatening condition. Therefore, the most important message of this review is to think of them. The diagnosis of pheochromocytomas depends mainly upon the demonstration of catecholamine excess by 24-h urinary catecholamines and metanephrines or plasma metanephrines. They are localized by a computed tomography scan and magnetic resonance imaging of the adrenal glands and abdomen; complementary 123I-metaiodobenzylguanidine scintigraphy and 18F-dihydroxyphenylalanine-positron emission tomography are available. Because approximately one out of four pheochromocytomas turn out to be hereditary entities, screening for genetic alterations is important. Laparoscopic and adrenal sparing surgical intervention following preoperative alpha-blockade is the treatment of choice and usually curative. In malignant pheochromocytomas, radiotherapy and chemotherapy are palliative treatment options. This review provides an update on identification and management of pheochromocytomas, emphasizing current developments in diagnosis, including genetic testing, pathophysiology and treatment of pheochromocytomas.
Topics: Adrenal Gland Neoplasms; Humans; Pheochromocytoma
PubMed: 17082709
DOI: 10.1097/01.hjh.0000251887.01885.54 -
Endocrinology and Metabolism Clinics of... Jun 1993Pheochromocytomas cause the most dramatic life-threatening crises in all of endocrinology. Pheochromocytoma is an explosive clinical syndrome that may be characterized... (Review)
Review
Pheochromocytomas cause the most dramatic life-threatening crises in all of endocrinology. Pheochromocytoma is an explosive clinical syndrome that may be characterized by either severe hypertension associated with cerebral, cardiac, and renal complications or hypotension, or even shock and sudden death. Other emergencies include lactic acidosis, hypoglycemia, hypercalcemic crisis, severe hypokalemic alkalosis, and acute bowel obstruction due to bowel ischemia, necessitating prompt surgical intervention. Better understanding of the mechanisms of catecholamine action and the pathophysiology of pheochromocytoma and the availability of various treatment modalities have made successful management more promising than ever before.
Topics: Adrenal Gland Neoplasms; Emergencies; Humans; Pheochromocytoma
PubMed: 8325290
DOI: No ID Found -
Journal of Clinical Hypertension... 2002Pheochromocytoma, a relatively rare (<0.05% of hypertensives), catecholamine-secreting tumor, is almost always lethal unless recognized and appropriately treated.... (Review)
Review
Pheochromocytoma, a relatively rare (<0.05% of hypertensives), catecholamine-secreting tumor, is almost always lethal unless recognized and appropriately treated. Clinical and biochemical manifestations are mainly caused by excess circulating catecholamines and hypertension. Manifestations mimic many conditions, which may result in erroneous diagnoses and improper treatment. Sustained or paroxysmal hypertension associated with headaches, sweating, or palpitations, occurs in 95% of patients, but at least 5% are normotensive. All patients with manifestations of hypercatecholaminemia or coexisting neoplasms should be investigated for pheochromocytoma. Plasma free metanephrines and fractionated urinary metanephrines are the most sensitive (about 100%) chemical tests for diagnosing sporadic and familial pheochromocytomas; plasma and urinary catecholamines and total metanephrines are fairly sensitive for identifying sporadic cases but are less sensitive for familial tumors. The clonidine suppression test helps exclude other conditions that may elevate plasma and urinary catecholamines and their metabolites. Magnetic resonance imaging is more sensitive than computed tomography for localizing pheochromocytomas; iodine-131-metaiodobenzylguanidine (131I-MIBG) tumor uptake confers specificity. Surgical resection is successful in 90% of cases, but 10% of tumors are malignant. Pheochromocytomas <5 cm in diameter can be removed laparoscopically; larger tumors should be removed by open surgery. Drug treatment prior to and during surgery is mandatory; drug treatment, chemotherapy, and radiation therapy are used to treat malignant lesions.
Topics: Adrenal Gland Neoplasms; Algorithms; Diagnosis, Differential; Humans; Hypertension; Pheochromocytoma
PubMed: 11821644
DOI: 10.1111/j.1524-6175.2002.01452.x -
American Journal of Surgery May 2011Pheochromocytomas are rare catecholamine-secreting tumors. Approximately 10 percent of pheochromocytomas are malignant. Traditionally, there has been no reliable method... (Review)
Review
BACKGROUND
Pheochromocytomas are rare catecholamine-secreting tumors. Approximately 10 percent of pheochromocytomas are malignant. Traditionally, there has been no reliable method available to predict the malignant potential of pheochromocytoma. However, recent research has increased focus on differentiating at the time of surgery/diagnosis those pheochromocytoma tumors which have malignant potential. In this review, we discuss the current information known of malignant pheochromocytomas.
DATA SOURCES
The PubMed database was searched for articles on malignant pheochromocytoma published between 1993 and 2010.
CONCLUSIONS
The difficult task of predicting the malignant potential of a pheochromocytoma has yet to be answered definitively. However, all the studies presented give an idea of what we may look for in these tumors at the time of diagnosis. We have provided an algorithm based on the most current information known. A much larger study should be performed to test many of these theories with enough power to determine a standard of care.
Topics: Adrenal Gland Neoplasms; Diagnosis, Differential; Diagnostic Imaging; Humans; Morbidity; Pheochromocytoma; Prognosis; Survival Rate; United States
PubMed: 20870212
DOI: 10.1016/j.amjsurg.2010.04.012 -
The Journal of Urology Jan 1992Extra-adrenal pheochromocytomas may arise in any portion of the paraganglion system, although they most commonly occur below the diaphragm. The most common site of... (Review)
Review
Extra-adrenal pheochromocytomas may arise in any portion of the paraganglion system, although they most commonly occur below the diaphragm. The most common site of occurrence of extra-adrenal pheochromocytoma is the superior para-aortic region between the diaphragm and lower renal poles. Although the traditional teaching has been that 10% of all pheochromocytomas are at extra-adrenal sites, this may be an underestimation. Extra-adrenal pheochromocytomas probably represent at least 15% of adult and 30% of childhood pheochromocytomas. They may be malignant in up to 40% of the cases, although conflicting data add to the uncertainty of this point. Patients with tumors arising at extra-adrenal sites commonly present with headache, palpitations, sweating and hypertension. The diagnosis is most often confirmed by demonstrating increased catecholamine production, usually by measurement of urinary catecholamines and/or their metabolites. CT scanning is presently the imaging procedure of choice for localization. The roles of MRI and 131I-MIBG scintigraphy in the localization process are still being determined. Thorough preoperative pharmacological preparation, attentive intraoperative monitoring and aggressive surgical therapy all have an important role in achieving the safest and most successful outcome. Complete surgical excision is the treatment of choice for primary extra-adrenal pheochromocytoma as well as recurrent or metastatic disease. When residual tumor cannot be resected, medical therapy for symptomatic relief is preferred, since radiotherapy and chemotherapy have limited effectiveness. Extra-adrenal pheochromocytomas are more likely to recur and to metastasize than their adrenal counterparts, making lifelong followup with annual determinations of catecholamine production essential.
Topics: Humans; Neoplasm Recurrence, Local; Pheochromocytoma; Prognosis; Urinary Bladder Neoplasms
PubMed: 1729490
DOI: 10.1016/s0022-5347(17)37119-7 -
Lancet (London, England)Phaeochromocytomas are rare neuroendocrine tumours with a highly variable clinical presentation but most commonly presenting with episodes of headaches, sweating,... (Review)
Review
Phaeochromocytomas are rare neuroendocrine tumours with a highly variable clinical presentation but most commonly presenting with episodes of headaches, sweating, palpitations, and hypertension. The serious and potentially lethal cardiovascular complications of these tumours are due to the potent effects of secreted catecholamines. Biochemical testing for phaeochromocytoma is indicated not only in symptomatic patients, but also in patients with adrenal incidentalomas or identified genetic predispositions (eg, multiple endocrine neoplasia type 2, von Hippel-Lindau syndrome, neurofibromatosis type 1, and mutations of the succinate dehydrogenase genes). Imaging techniques such as CT or MRI and functional ligands such as (123)I-MIBG are used to localise biochemically proven tumours. After the use of appropriate preoperative treatment to block the effects of secreted catecholamines, laparoscopic tumour removal is the preferred procedure. If removal of phaeochromocytoma is timely, prognosis is excellent. However, prognosis is poor in patients with metastases, which especially occur in patients with large, extra-adrenal tumours.
Topics: Humans; Pheochromocytoma
PubMed: 16112304
DOI: 10.1016/S0140-6736(05)67139-5 -
Current Hypertension Reports Dec 2004Pheochromocytoma is a rare but extremely treacherous neuroendocrine tumor causing manifestations by secreting catecholamines into the circulation. It is usually fatal if... (Review)
Review
Pheochromocytoma is a rare but extremely treacherous neuroendocrine tumor causing manifestations by secreting catecholamines into the circulation. It is usually fatal if unrecognized. Manifestations can mimic many diseases and cause erroneous diagnoses. Approximately 15% are malignant, 18% extra-adrenal, and 20% familial. Plasma or urinary metanephrines are approximately 98% sensitive for detecting pheochromocytomas. They can be localized by MRI and CT; (131)I- and (123)I-metaiodobenzylguanidine (MIBG) are highly specific and 81% to 90% sensitive, respectively. Suspect pheochromocytoma in patients with sustained or paroxysmal hypertension or in normotensives having manifestations of hypercatecholaminemia. Surgical removal is usually curative; chemotherapy and radiotherapy are palliative for malignant pheochromocytomas.
Topics: Adrenal Gland Neoplasms; Combined Modality Therapy; Decision Trees; Humans; Magnetic Resonance Imaging; Pheochromocytoma; Tomography, X-Ray Computed
PubMed: 15527694
DOI: 10.1007/s11906-004-0044-2 -
Radiographics : a Review Publication of... Oct 2004Pheochromocytomas are rare catecholamine-secreting tumors with many clinical and imaging manifestations. They may produce overwhelming cardiovascular crises if the... (Review)
Review
Pheochromocytomas are rare catecholamine-secreting tumors with many clinical and imaging manifestations. They may produce overwhelming cardiovascular crises if the diagnosis is not made or if appropriate treatment is delayed. It is thus important to recognize both their characteristic and atypical imaging appearances. Pheochromocytomas are encountered, sometimes unexpectedly, across a range of imaging modalities. They are characteristically solid, hypervascular masses with high signal intensity on T2-weighted magnetic resonance (MR) images. A wide spectrum of imaging appearances is seen, however, and pheochromocytomas may mimic other adrenal lesions, both benign and malignant. They may be dark on T2-weighted MR images, in contrast to their more classic bright T2-weighted appearance. Other atypical features include fatty, hemorrhagic, cystic, and calcific changes. Pheochromocytomas may contain sufficient fat to be mistaken for an adenoma at computed tomography (CT) or MR imaging. They may also demonstrate rapid contrast material washout and be mistaken for an adenoma owing to their deenhancement profile; however, their washout pattern can be inconsistent. The appearance of pheochromocytomas at radionuclide imaging is also unpredictable. These characteristics at CT, MR imaging, and scintigraphy pose diagnostic challenges, since they allow pheochromocytomas to mimic many other adrenal masses. Pheochromocytoma is an important, often clinically occult neoplasm with devastating consequences if overlooked. Radiologists must be aware of the various forms that pheochromocytomas can assume at imaging.
Topics: Adrenal Gland Neoplasms; Adult; Aged; Female; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Pheochromocytoma; Radiography
PubMed: 15486252
DOI: 10.1148/rg.24si045506 -
Veterinary Pathology May 2019Pheochromocytoma is frequent in dogs and carries a guarded prognosis. Current histological criteria may not predict malignant behavior in dogs, similar to humans. In...
Pheochromocytoma is frequent in dogs and carries a guarded prognosis. Current histological criteria may not predict malignant behavior in dogs, similar to humans. In humans, characterization of tumors has been refined using the pheochromocytoma of the adrenal gland scaled score (PASS) and by immunohistochemistry. The study aim was to investigate PASS and immunohistochemical markers used in humans in 24 dogs with pheochromocytoma that underwent adrenalectomy. Dogs with pheochromocytomas were reviewed and tumors collected. Histological sections were evaluated to apply the PASS and were single-labeled for chromogranin A, Ki-67, COX-2, p53, BCL-2, c-erbB-2, vascular endothelial growth factor, and S100. Survival, age, and vascular and capsular invasion were compared for PASS and immunohistochemical markers; results of PASS were also compared for each marker. Associations between markers were tested. PASS and immunohistochemical markers did not differ for survival, age, and vascular and capsular invasion. Tumors showing BCL-2 expression in >50% cells had lower PASS than those with lower expression (PASS: 7 ± 2 vs 9 ± 2; P = .011). Tumors positive for S100 had higher PASS than those that were negative (PASS: 10 ± 2 vs 7 ± 2; P = .001). Results of the different markers were not associated. In conclusion, in the context of canine pheochromocytoma, PASS and the selected immunohistochemical markers are not associated with survival, age, or vascular or capsular invasion. The higher PASS in S100-positive tumors may indicate that pheochromocytomas developing morphologic changes acquire S100 expression. The significance of lower PASS in tumors with elevated BCL-2 expression is uncertain. Overall, the use of PASS and the present immunohistochemical markers may not be useful in dogs with pheochromocytoma.
Topics: Adrenal Gland Neoplasms; Adrenal Glands; Adrenalectomy; Animals; Biomarkers, Tumor; Dog Diseases; Dogs; Female; Male; Pheochromocytoma; Prognosis
PubMed: 30595108
DOI: 10.1177/0300985818819174