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| | __NOTOC__ |
| | {{Infobox_Disease |
| | | Name = {{PAGENAME}} |
| | | Image = Head.jpg |
| | | Caption = Gross pathology of pheochromocytoma, source: wikipedia.org |
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| | {{Pheochromocytoma}} |
| | {{CMG}}; {{AE}} {{AAM}} {{MAD}} |
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| | {{SK}} Phaeochromocytoma; Pheochromocytoma; Chromaffin paraganglioma; Chromaffin tumor; Chromaffinoma |
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| | '''For patient information click [[{{PAGENAME}} (patient information)|here]]''' |
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| {{Infobox_Disease | | | {{Infobox_Disease | |
| Name = Pheochromocytoma | | | Name = Pheochromocytoma | |
| Image = Pheochromocytoma high mag.jpg | | | Image = Pheochromocytoma high mag.jpg | |
| Caption = Micrograph of a pheochromocytoma. | | | Caption = Micrograph of a pheochromocytoma | |
| DiseasesDB = 9912 |
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| ICD10 = {{ICD10|C|74|1|c|73}} |
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| ICD9 = {{ICD9|255.6}} |
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| ICDO = {{ICDO|8700|0}} |
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| OMIM = 171300 |
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| MedlinePlus = |
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| eMedicineSubj = |
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| eMedicineTopic = |
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| eMedicine_mult ={|
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| MeshID = D010673 |
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| }} | | }} |
| {{Pheochromocytoma}}
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| {{CMG}}
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| ==[[Pheochromocytoma overview|Overview]]== | | ==[[Pheochromocytoma overview|Overview]]== |
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| ==[[Pheochromocytoma differential diagnosis|Differentiating Pheochromocytoma from other Diseases]]== | | ==[[Pheochromocytoma historical perspective|Historical Perspective]]== |
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| ==[[Pheochromocytoma causes|Causes of Pheochromocytoma]]== | | ==[[Pheochromocytoma classification|Classification]]== |
| ==[[Pheochromocytoma history and symptoms|History & Symptoms]]==
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| ==Tumor Location== | | ==[[Pheochromocytoma pathophysiology|Pathophysiology]]== |
| In adults, 90% tumors are located unilaterally and are solitary, and 10% are located outside the adrenal gland. In children 50% are adrenal, while 25% are bilateral and 25% are extraadrenal. The common extradrenal locations are the abdomen, thorax and urinary bladder.
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| ==Diagnosis== | | ==[[Pheochromocytoma causes|Causes]]== |
| [[Image:Adrenal pheochromocytoma (3) histopathology.jpg|thumb|left|150px|Histopathology of adrenal pheochromocytoma. Adrenectomy specimen. ]]
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| [[Image:Adrenaline.svg|thumb|150px|left|[[Epinephrine]]]]
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| [[Image:Norepinephrine.png|thumb|100px|left|[[Norepinephrine]]]]
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| The diagnosis can be established by measuring [[catecholamine]]s and [[metanephrine]]s in plasma or through a 24-hour urine collection. Care should be taken to rule out other causes of adrenergic (adrenalin-like) excess like hypoglycemia, stress, exercise, and drugs affecting the catecholamines like [[stimulant]]s, [[methyldopa]], [[dopamine]] [[agonist]]s, or ganglion blocking [[antihypertensive]]s. Various foodstuffs (e.g. vanilla ice cream) can also affect the levels of urinary [[metanephrine]] and VMA ([[vanillyl mandelic acid]]). Imaging by [[computed tomography]] or a [[Spin-spin relaxation time|T2]] weighted [[magnetic resonance imaging|MRI]] of the [[head]], [[neck]], and [[chest]], and [[abdomen]] can help localize the tumor. Tumors can also be located using [[Iodine-131]] meta-iodobenzylguanidine (I131 MIBG) imaging.
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| One diagnostic test used in the past for a pheochromocytoma is to administer [[clonidine]], a centrally-acting alpha-2 agonist used to treat high blood pressure. Clonidine mimics catecholamines in the brain, causing it to reduce the activity of the sympathetic nerves controlling the adrenal medulla. A healthy adrenal medulla will respond to the [[Clonidine#Clonidine suppression test|Clonidine suppression test]] by reducing catecholamine production; the lack of a response is evidence of pheochromocytoma.
| | ==[[Pheochromocytoma differential diagnosis|Differentiating Pheochromocytoma from other Diseases]]== |
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| Another test is for the clinician to press gently on the [[adrenal gland]]. A pheochromocytoma will often release a burst of catecholamines, with the associated signs and symptoms quickly following. This method is not recommended because of possible complications arising from a potentially massive release of catecholamines.
| | ==[[Pheochromocytoma epidemiology and demographics|Epidemiology and Demographics]]== |
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| Pheochromocytomas occur most often during young-adult to mid-adult life. Less than 10% of pheochromocytomas are [[malignant]] (cancerous), bilateral or pediatric.
| | ==[[Pheochromocytoma risk factors|Risk Factors]]== |
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| These tumors can form a pattern with other endocrine gland cancers which is labeled [[multiple endocrine neoplasia]] (MEN). Pheochromocytoma may occur in patients with MEN 2 and MEN 3. [[VHL]] (Von Hippel Lindau) patients may also develop these tumors.
| | ==[[Pheochromocytoma screening|Screening]]== |
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| Patients experiencing symptoms associated with pheochromocytoma should be aware that it is rare. However, it often goes undiagnosed until autopsy; therefore patients might wisely choose to take steps to provide a physician with important clues, such as recording whether blood pressure changes significantly during episodes of apparent anxiety.
| | ==[[Pheochromocytoma natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
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| ==[[Pheochromocytoma medical therapy|Medical Therapy]]== | | ==Diagnosis== |
| Surgical [[resection]] of the tumor is the treatment of first choice. Given the complexity of [[perioperative]] management, and the potential for catastrophic intra and postoperative complications, such surgery should be performed only at centers experienced in the area. In addition to the surgical expertise that such centers can provide, they will also have the necessary endocrine and anesthesia resources as well. It may also be nescessary to carry out adrenalectomy, a complete surgical removal of the affected adrenal gland(s).
| | [[Pheochromocytoma history and symptoms|History and Symptoms]] | [[Pheochromocytoma physical examination|Physical Examination]] | [[Pheochromocytoma laboratory findings|Laboratory Findings]] | [[Pheochromocytoma electrocardiogram|Electrocardiogram]] | [[Pheochromocytoma x ray|X Ray]] | [[Pheochromocytoma CT|CT]] | [[Pheochromocytoma MRI|MRI]] | [[Pheochromocytoma echocardiography or ultrasound|Echocardiography or Ultrasound]] | [[Pheochromocytoma other imaging findings|Other Imaging Findings]] | [[Pheochromocytoma other diagnostic studies|Other Diagnostic Studies]] |
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| Either surgical option requires prior treatment with both the non-specific alpha adrenoceptor blocker [[Phenoxybenzamine]] to counteract hypertension and the beta-1 adrenoceptor antagonist [[Atenolol]] to reduce cardiac output. Given before surgery, these can also block the effect of a sudden release of adrenaline during tumour removal, which would otherwise endanger the anaethetised patient.
| | ==Treatment== |
| | [[Pheochromocytoma medical therapy|Medical Therapy]] | [[Pheochromocytoma surgery|Surgery]] | [[Pheochromocytoma primary prevention|Primary Prevention]] | [[Pheochromocytoma secondary prevention|Secondary Prevention]] | [[Pheochromocytoma cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Pheochromocytoma future or investigational therapies|Future or Investigational Therapies]] |
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| ==[[Pheochromocytoma historical perspective|Historical perspective]]== | | ==Case Studies== |
| In 1886, Fränkel made the first description of a patient with pheochromocytoma, however the term was first coined by Pick, a pathologist, in 1912. In 1926, Roux (in Switzerland) and Mayo (in U.S.A.) were the first surgeons to remove pheochromocytomas.<br />
| | [[Pheochromocytoma case study one|Case #1]] |
| Jaroszewski DE, Tessier DJ, Schlinkert RT, et al. Laparoscopic adrenalectomy for pheochromocytoma. Mayo Clin Proc. 2003; 78: 1501-1504.
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| ==Additional images==
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| <gallery>
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| Image:Adrenal pheochromocytoma (1) histopathology.jpg|[[Micrograph]] of pheochromocytoma.
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| Image:Adrenal pheochromocytoma (2) histopathology.jpg|Micrograph of pheochromocytoma.
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| Image:Adrenal pheochromocytoma (3) histopathology.jpg|Micrograph of pheochromocytoma.
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| Image:Bilateral pheo MEN2.jpg|Bilateral pheochromocytoma in [[Multiple_endocrine_neoplasia_type_2|MEN2]]. Gross image.
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| Image:Pheochromocytoma.jpg|Pheochromocytoma. CT abdomen.
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| Image:Pheochromocytoma2.jpg|Pheochromocytoma. CT abdomen.
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| </gallery>
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| ==References== | | ==References== |
| {{Reflist|2}} | | {{reflist|2}} |
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| ==External links==
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| * [http://clinicaltrials.gov/show/NCT00458952 Pheochromocytoma clinical trial currently recruiting patients]
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| * {{MedlinePlusOverview|pheochromocytoma}}
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| * [http://www.cancer.gov/cancertopics/pdq/treatment/pheochromocytoma/healthprofessional overview] from [[National Cancer Institute]]
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| * [http://www.pressor.org/members.html Pheochromocytoma Research Support Organization at pressor.org]
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| * [http://pub1.ezboard.com/bpheochromocytomasupportboard Pheochromocytoma Support Worldwide at pub1.ezboard.com]
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| * [http://www.pheochromocytoma.org/sys-tmpl/door/ pheochromocytoma.org]
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| {{Epithelial neoplasms}}
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| {{SIB}}
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| [[Category:Endocrinology]] | | [[Category:Endocrinology]] |
| [[Category:Oncology]]
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| [[de:Phäochromozytom]]
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| [[es:Feocromocitoma]]
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| [[fr:Phéochromocytome]]
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| [[it:Feocromocitoma]]
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| [[he:פאוכרומוציטומה]]
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| [[nl:Feochromocytoom]]
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| [[ja:褐色細胞腫]]
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| [[pl:Guz chromochłonny nadnerczy]]
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| [[sv:Feokromocytom]]
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