Materiały źródłowe

• #1 Pheochromocytoma FAQs: Treatment & Diagnosis – Endocrine Surgery | UCLA Health

  https://www.uclahealth.org/medical-services/surgery/endocrine-surgery/conditions-treated/adrenal/adrenal-tumors/pheochromocytoma/pheochromocytoma-treatment-diagnosis

  Pheochromocytoma is a rare and potentially dangerous tumor that affects approximately 1 in 500,000 people. […] High blood pressure (hypertension) is the most common problem attributed to pheochromocytomas. […] Patients with pheochromocytomas may experience either consistently high blood pressure (due to constant hormone release) or episodic peaks in blood pressure (due to random bursts of hormone release). […] Pheochromocytomas are regarded as quite likely the single most high risk tumor that physicians treat. […] Establishing the diagnosis of pheochromocytoma is dependent on the demonstration of significant catecholamine excess. […] A 24-hour urine test for pheochromocytoma is considered positive if the catecholamine levels exceed two times the upper limit of normal. […] The most commonly ordered blood test for pheochromocytoma is the plasma free metanephrine test.

• #1 Pheochromocytoma – Endocrine and Metabolic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/endocrine-and-metabolic-disorders/adrenal-disorders/pheochromocytoma

  Pheochromocytoma is suspected in patients with typical symptoms or particularly sudden, severe, or intermittent unexplained hypertension, especially in younger patients and those with resistant hypertension. Diagnosis involves demonstrating high levels of catecholamine products in the serum or urine. […] Plasma free metanephrines are up to 99% sensitive with either metanephrine or more frequently normetanephrine being elevated. This test has superior sensitivity to measurement of circulating epinephrine and norepinephrine because plasma metanephrines are elevated continuously, unlike epinephrine and norepinephrine, which are secreted intermittently. […] Urinary metanephrine and normetanephrine levels are slightly less specific for pheochromocytoma than plasma free metanephrine, but elevation of either metabolite has approximately 95% sensitivity.

• #1 Pheochromocytoma – Endocrinology Advisor

  https://www.endocrinologyadvisor.com/ddi/pheochromocytoma/

  Pheochromocytoma diagnosis is confirmed by a combination of lab tests and imaging. Elevated levels of catecholamines or their metabolites on blood or urine analysis are highly indicative of pheochromocytomas or paragangliomas, while imaging tests such as computed tomography (CT) and magnetic resonance imaging (MRI) confirm the presence and determine the size and specific location of the tumor. The results from both lab and imaging tests are essential in making a proper diagnosis and developing a sound pheochromocytoma treatment plan. […] A diagnostic pheochromocytoma workup begins with a high degree of suspicion by the clinician based on a detailed patient history, a thorough clinical evaluation, and identification of characteristic findings, such as headaches, perspiration, and palpitations in patients with hypertension, hypertension that is unresponsive to standard treatment, and paroxysmal attacks. Once the clinician suspects a pheochromocytoma, the first step is biochemical laboratory testing. Plasma metanephrines are the recommended test in patients with high suspicion for pheochromocytoma, while 24-hour urine metanephrine testing is recommended in those with a low pretest probability.

• #1 Azthena logo with the word Azthena

  https://www.news-medical.net/health/Pheochromocytoma-Diagnosis.aspx

  Pheochromocytoma is a catecholamine-producing tumor that originates from chromaffin cells of the adrenergic system, most commonly in the adrenal medulla. There are two main steps in the diagnosis of pheochromocytoma: biochemical demonstration of excessive catecholamine secretion and radiological localization of the causative tumor. […] In any case, the diagnosis should be suspected in patients with one or more of the following: The onset of hypertension at a young age, Resistant or labile hypertension, Hypertensive response during anesthesia, surgery, or angiography, Paroxysmal and self-limiting episodes of typical adrenergic symptoms, Incidentally discovered adrenal mass, Family history of pheochromocytoma, Familial syndrome predisposing to catecholamine-secreting tumors. […] Today, the diagnosis of pheochromocytoma has been made simple by significant developments in the assays that are used to both detect and quantify catecholamine levels in the blood and urine.

• #1 Diagnosis and Treatment of Pheochromocytoma

  https://www.ekjm.org/journal/view.php?number=22892

  Pheochromocytomas are rare neuroendocrine tumours with a highly variable and heterogeneous clinical manifestation. […] Diagnosis of pheochromocytoma depends on biochemical evidence of catecholamine production by the tumor. Plasma free metanephrines provide the best test for excluding or confirming pheochromocytoma and should be used as an initial screening tool, but if it is not available, urinary fractionated metanephrines could be used as an alternative method. […] Different anatomical and functional imaging modalities are used to localize biochemically proven pheochromocytomas. These include computed tomography, magnetic resonance imaging, single-photon emission computed tomography (SPECT) using 123/131I-metaiodobenzylguanidine (MIBG) or 111In-DTPA-pentetreotide and positron emission tomography (PET).

• #1 Pheochromocytoma Workup: Approach Considerations, Metanephrine and Catecholamine Testing, Additional Laboratory Tests

  https://emedicine.medscape.com/article/124059-workup

  Catecholamines produced by pheochromocytomas are metabolized within chromaffin cells. Norepinephrine is metabolized to normetanephrine and epinephrine is metabolized to metanephrine. Because this process occurs within the tumor, independently of catecholamine release, pheochromocytomas are best diagnosed by measurement of these metabolites rather than by measurement of the parent catecholamines. […] Guidelines from the North American NeuroEndocrine Tumor Society (NANETS) recommend biochemical testing for pheochromocytoma in the following cases: symptomatic patients, patients with an adrenal incidentaloma, patients who have a hereditary risk for developing a pheochromocytoma or paraganglioma (extra-adrenal pheochromocytoma). […] The choice of diagnostic test should be based on the clinical suspicion of a pheochromocytoma. Plasma metanephrine testing has the highest sensitivity (96%) for detecting a pheochromocytoma, but it has a lower specificity (85%). In comparison, a 24-hour urinary collection for catecholamines and metanephrines has a sensitivity of 87.5% and a specificity of 99.7%.

• #1 Clinical presentation and diagnosis of pheochromocytoma – UpToDate

  https://www.uptodate.com/contents/clinical-presentation-and-diagnosis-of-pheochromocytoma

  Clinical presentation and diagnosis of pheochromocytoma […] The clinical presentation and approach to diagnosis of pheochromocytoma are reviewed here. […] Sporadic pheochromocytoma is usually diagnosed on the basis of symptoms or an incidental discovery on computed imaging, whereas syndromic pheochromocytoma is frequently diagnosed earlier in the course of disease because of biochemical surveillance or genetic testing. […] The initial biochemical tests for pheochromocytoma include 24-hour urine fractionated metanephrines and catecholamines for low-risk patients and plasma fractionated metanephrines for high-risk patients. […] Imaging techniques such as CT and MRI are used for additional evaluation after biochemical diagnosis.

• #1 Pheochromocytoma: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/124059-overview

  Diagnostic tests for pheochromocytoma include the following: Plasma metanephrine testing: 96% sensitivity, 85% specificity. 24-hour urinary collection for catecholamines and metanephrines: 87.5% sensitivity, 99.7% specificity. […] Test selection criteria include the following: Use plasma metanephrine testing in patients at high risk (ie, those with predisposing genetic syndromes or a family or personal history of pheochromocytoma). Use 24-hour urinary collection for catecholamines and metanephrines in patients at lower risk. […] Imaging studies should be performed only after biochemical studies have confirmed the diagnosis of pheochromocytoma. Studies are as follows: Abdominal CT scanning: Has accuracy of 85-95% for detecting adrenal masses with a spatial resolution of 1 cm or greater. MRI: Preferred over CT scanning in children and pregnant or lactating women; has reported sensitivity of up to 100% in detecting adrenal pheochromocytomas. Scintigraphy: Reserved for biochemically confirmed cases in which CT scanning or MRI does not show a tumor. PET scanning: A promising technique for detection and localization of pheochromocytomas.

• #1 Pheochromocytoma – Endocrinology Advisor

  https://www.endocrinologyadvisor.com/ddi/pheochromocytoma/

  Computed tomography (CT) scan or magnetic resonance imaging (MRI) should be used to localize the tumor and confirm a diagnosis of pheochromocytoma. MRI is indicated over a CT scan in certain conditions, such as for patients with contrast allergy, the pediatric population, and pregnant patients. It should be noted that these imaging modalities are sensitive but not specific; a very specific test for catecholamine-secreting tumors is the 123I-labeled metaiodobenzylguanidine (MIBG) scintigraphy. MIBG can help to differentiate between adrenal and paraganglionic tumors and can also help reveal multiple lesions and metastases. […] If metanephrine levels are four times higher than the normal reference value, the next step is to localize the hormone-secreting tumor. If metanephrine levels are only minimally elevated or ambiguous and the clinician still suspects pheochromocytoma, the clonidine suppression test can help confirm a diagnosis.

• #1 Diagnosis and Treatment of Pheochromocytoma

  https://www.ekjm.org/journal/view.php?number=22892

  Sensitivity and specificity of biochemical tests for diagnosis of pheochromocytoma: Plasma-free metanephrines 99% sensitivity, 89% specificity; Plasma catecholamines 84% sensitivity, 81% specificity; Urinary catecholamines 86% sensitivity, 88% specificity; Urinary-fractionated metanephrines 97% sensitivity, 69% specificity; Urinary total metanephrines 77% sensitivity, 93% specificity; VMA 64% sensitivity, 95% specificity. […] Sensitivity and specificity of biochemical and imaging methods for diagnosis of pheochromocytoma: 24-hr urinary tests VMA ++ ++++; Cathecholamines +++ +++ ; Fractionated metanephrines ++++ ++; Total metanephrines +++ ++++; Plasma tests Cathecholamines +++ ++; Free metanephrines ++++ +++ ; Chromogranin A +++ ++; CT ++++ +++ ; MRI ++++ +++ ; MIBG scintigraphy +++ ++++; Somatostatin receptor scintigraphy ++ ++; dopa PET ++++ ++++.

• #1 Diagnosis for Pheochromocytoma and Paraganglioma: A Joint Position Statement of the Korean Pheochromocytoma and Paraganglioma Task Force

  https://www.e-enm.org/journal/view.php?doi=10.3803/EnM.2020.908

  Pheochromocytoma and paraganglioma (PPGLs) are rare catecholamine-secreting neuroendocrine tumors but can be life-threatening. […] Therefore, timely and accurate diagnosis of PPGLs is crucial. […] However, performing diagnostic studies to localize lesions and detect metastatic potential can be still challenging and complicated. […] The Korean PPGL task force team has developed this position statement focusing on the comprehensive and updated diagnosis for PPGLs. […] 1.1. All pheochromocytoma and paraganglioma (PPGLs) are considered to have metastatic potential. The terms benign and malignant should not be used to distinguish non-metastatic PPGLs from metastatic PPGLs (A). […] 1.2. The tumor-node-metastasis (TNM) staging system should be evaluated in diagnosing PPGLs (A). […] 2.1. Initial biochemical tests of PPGL should include measurements of plasma free metanephrines or urinary fractionated metanephrines (A). […] 2.2. Measurements of urinary dopamine and plasma 3-methoxytyramine are useful for the biochemical diagnosis of PPGLs with predominantly dopamine secretion and/or high risk for metastases (C). […] 2.3. Chromogranin A can be used as a biomarker for biochemically silent PPGL (PPGL with normal metanephrine, normetanephrine, and 3-methoxytyramine) (C).

• #1 Pheochromocytoma Workup: Approach Considerations, Metanephrine and Catecholamine Testing, Additional Laboratory Tests

  https://emedicine.medscape.com/article/124059-workup

  Suppression tests using phentolamine and clonidine can also be used for diagnostic purposes. NANETS guidelines recommend clonidine suppression testing when plasma metanephrine values are less than 4-fold above the upper reference limit. […] Chromogranin A is an acidic monomeric protein that is stored with and secreted with catecholamines. Plasma levels of chromogranin A reportedly are 83% sensitive and 96% specific for identifying a pheochromocytoma. […] Abdominal CT scanning has an accuracy of 85-95% for detecting adrenal masses with a spatial resolution of 1 cm or greater but is less accurate for lesions smaller than 1 cm. […] MRI is preferred for detection of pheochromocytoma in children and in pregnant or lactating women. MRI has a reported sensitivity of up to 100% in detecting adrenal pheochromocytomas, does not require contrast, and does not expose the patient to ionizing radiation.

• #1 Pheochromocytoma – WikiAnesthesia

  https://wikianesthesia.org/wiki/Pheochromocytoma

  Diagnosis of pheochromocytoma relies on measurement of excessive levels of catecholamines and its metabolites followed by imaging studies. Catecholamine hypersecretion can be confirmed by measurement of urinary and plasma catecholamines; and/or metanephrine and normetanephrine, metabolites of epinephrine and norepinephrine. […] 24 hour urine levels of these compounds have classically been used to diagnose pheochromocytoma and is the most reliable test. […] Plasma metanephrine and normetanephrine is also commonly used for diagnosis but notably has a lower specificity (89%) than 24-hour urine levels. […] When equivocal results occur, a clonidine suppression test can be used. Clonidine is an alpha-2 adrenergic antagonist and decreases catecholamine levels. In patients with pheochromocytoma, clonidine will fail to suppress the catecholamine levels.

• #1 Pheochromocytoma FAQs: Treatment & Diagnosis – Endocrine Surgery | UCLA Health

  https://www.uclahealth.org/medical-services/surgery/endocrine-surgery/conditions-treated/adrenal/adrenal-tumors/pheochromocytoma/pheochromocytoma-treatment-diagnosis

  Imaging should only be performed after the diagnosis of pheochromocytoma has been established with 24-hour urine testing. […] The great majority of pheochromocytomas are successfully treated with surgery. […] Surgery can only be performed safely after the careful administration of alpha-blockers for at least two to three weeks prior to surgery. […] The key to successful surgery is effective teamwork between the surgeon and anesthesiologist.

• #1 Diagnosis for Pheochromocytoma and Paraganglioma: A Joint Position Statement of the Korean Pheochromocytoma and Paraganglioma Task Force

  https://www.e-enm.org/journal/view.php?doi=10.3803/EnM.2020.908

  3.1. Once there is clear biochemical evidence of a PPGL, anatomic imaging by computed tomography (CT) is the first-choice imaging modality to locate PPGLs. Magnetic resonance imaging is the second-choice imaging method when CT findings are inconclusive or when patients are poor candidates to undergo contrast-enhanced CT (A). […] 3.2. Functional imaging is recommended for evaluating disease characteristics and detecting metastases, particularly in patients with a high-risk for metastases and multifocal diseases (e.g., larger tumor size 5.0 cm, extra-adrenal, bilateral, or hereditary) (A). […] 3.3. We suggest 123I-metaiodobenzylguanidine (MIBG) scintigraphy/single-photon emission computed tomography (SPECT), gallium 68 (68Ga) 1,4,7,10-tetraazacyclododecane-1,4,7,10-tetraacetic acid (DOTA)-somatostatin receptor analogs (SSA) positron emission tomography (PET)/CT, or fluorine-18-L-dihydroxyphenylalanine (18F-DOPA) PET/CT as the functional imaging modality according to the genotype, location, availability of radiopharmaceuticals, and clinical situation (B). […] 3.4. In PPGL patients planning for 131I-MIBG treatment, 123I-MIBG is necessary for treatment decision and response monitoring. 68Ga-DOTA-SSA PET/CT is necessary in patients with metastatic PPGLs when peptide receptor radionuclide therapy (PRRT) is planned (B).

• #1 Imaging and Tests for Pheochromocytoma Adrenal Tumors

  https://www.adrenal.com/pheochromocytoma/imaging-scans

  Doctors have a number of options when they order scans or x-rays to look for the presence of a pheochromocytoma within the adrenal gland. […] The gold standard of adrenal imaging is a CT scan (CAT scan). An adrenal-protocol, contrast enhancement CT scan is best. Thus a CT Scan of the adrenal with and without contrast should always be the first scan ordered, and in more than 90% of cases the ONLY scan a patient will need. […] MRI has similar diagnostic accuracy to CT allowing characterizing adenomas regardless of their CT enhancement. […] The MIBG Scan is used only to detect the presence and location of pheochromocytomas and paragangliomas. […] DOTATATE PET/CT should now be considered the ideal first line investigation for imaging pheochromocytoma and paraganglioma. […] Pheochromocytomas on Contrast-Enhanced CT Scans. Pheos can present with solid, cystic, calcific, and/or necrotic components–quite variable. Smaller pheos tend to display a more uniform attenuation, with a density of 40-50 HU. After contrast administration, pheochromocytomas enhance avidly (meaning turn very bright) with some of them showing higher enhancement on the portal venous phase and other on the arterial phase; nevertheless, their APW and RPW are similar to those of adenomas. Therefore, pheochromocytomas often cannot be reliably differentiated from adenomas using CT washout protocols.

• #1 Adrenal pheochromocytoma: Keys to radiologic diagnosis | Radiología (English Edition)

  https://www.elsevier.es/en-revista-radiologia-english-edition–419-articulo-adrenal-pheochromocytoma-keys-radiologic-diagnosis-S217351072200091X?newsletter=true

  Pheochromocytomas are adrenal paragangliomas. Potentially malignant, these tumors have a low incidence but clear importance. They can appear in various hereditary syndromes, especially in von Hippel-Lindau syndrome, multiple endocrine neoplasia-2 (MEN2), and familial paraganglioma syndromes. […] Although these tumors can manifest with a characteristic clinical presentation, in 13.1%57.6% of cases, it is the radiologist who first suggests the diagnosis, indicating analyses for catecholamines or nuclear medicine examinations. […] Radiologists should suspect a pheochromocytoma on detection of a well-delimited adrenal mass with rapid, intense enhancement that typically shows cystic and hemorrhagic phenomena, high T2 signal intensity, and the absence of macroscopic or microscopic lipids. […] Approximately one-third of lesions show late washout similar to that seen with adenomas on CT.

• #1 Phaeochromocytoma

  https://dermnetnz.org/topics/phaeochromocytoma

  Phaeochromocytoma may be suspected from typical history and presence of hypertension on examination. The following investigations are performed. […] Plasma or 24-hour urinary catecholamines and metanephrines if strongly elevated (34 times the normal range), the diagnosis of phaeochromocytoma is likely. […] Chromogranin A elevated in phaeochromocytoma. […] Genetic testing if there are multiple tumours or a family history of phaeochromocytoma. For solitary tumours in patients over 40 years of age without a family history, genetic testing is probably not necessary. […] Magnetic resonance imaging (MRI) with gadolinium contrast shows a hyperintense mass in T2 phase. Infiltration of local organs and vessels can be better evaluated with MRI than with CT. […] Computed tomography (CT) with contrast. Benefits of CT are low cost, availability and high sensitivity (detection of lesion 0.51 cm). CT has low specificity for phaeochromocytoma. […] The criteria for malignancy are excessive hormone production and tumour size 46 cm. Currently, there are no histological criteria for distinguishing benign and malignant tumours.

• #1 How do health care providers diagnose pheochromocytoma? | NICHD – Eunice Kennedy Shriver National Institute of Child Health and Human Development

  https://www.nichd.nih.gov/health/topics/pheochrom/conditioninfo/diagnosed

  A health care provider uses blood and urine tests that measure catecholamines and/or their metabolites to diagnose pheochromocytoma. Higher-than-typical amounts of these biochemical substances in the blood and/or urine can be an indication of the presence of a pheochromocytoma/paraganglioma. […] Multiple studies at the National Institutes of Health (NIH) have demonstrated the utility of measuring metanephrines in the blood for the diagnosis of pheochromocytoma/paraganglioma. This method is often recommended as the first diagnostic test when there is suspicion of a pheochromocytoma/paraganglioma. […] The location of a pheochromocytoma can be determined by using several imaging methods, including computed tomography (CT) and magnetic resonance imaging (MRI). A third imaging method that can be used to detect pheochromocytomas is MIBG (metaiodobenzylguanidine) scintigraphy. During this procedure, MIBG, a compound containing a small amount of radioactivity, is injected into a vein and is picked up by pheochromocytoma cells but not healthy cells. Any MIBG that is seen with the scanner can indicate the presence of pheochromocytoma cells.

• #1 Detecting Pheochromocytoma

  https://pmc.ncbi.nlm.nih.gov/articles/PMC1449983/

  The sensitivity of 131I-MIBG in other studies ranged from 77% to 94.7%, similar to the result found in our investigation (83.7%). […] We thus propose that 131I-MIBG scanning should be performed in patients with clinically suspected pheochromocytoma if the catecholamine measurements are normal. […] MIBG scanning is not only a complementary approach to improve the sensitivity of the urine, plasma, and platelets tests, but also critically important to the surgeon in the preoperative localization of the pheochromocytoma.

• #1 Diagnosis for Pheochromocytoma and Paraganglioma: A Joint Position Statement of the Korean Pheochromocytoma and Paraganglioma Task Force

  https://www.e-enm.org/journal/view.php?doi=10.3803/EnM.2020.908

  4.1. The Pheochromocytoma of the Adrenal Gland Scaled Score (PASS) and the Grading of Adrenal Pheochromocytoma and Paraganglioma (GAPP) cannot be used to confirm the diagnosis of malignancy (B). […] 4.2. The loss of succinate dehydrogenase B (SDHB) protein by immunohistochemistry staining in tumor cells is suggested to detect the presence of germline mutations in one of the SDHx genes. PPGLs associated with SDHB mutation have a high risk of metastases (B). […] 5.1. Genetic testing is recommended in all patients diagnosed with PPGLs (A). […] 5.2. Genetic testing should be also considered for first-degree relatives of patients with hereditary PPGLs (B). […] 5.3. Validated targeted next-generation sequencing (NGS) is a preferred method for the genetic diagnosis of PPGLs (B). […] 5.4. We recommend targeted NGS panels of gene sets based on the current level of evidence of their pathogenic driver status: 10 basic panel (fumarate hydratase [FH], myc-associated protein X [MAX], neurofibromatosis 1 [NF1], rearranged during transfection [RET], succinate dehydrogenase A [SDHA], SDHB, SDHC, SDHD, transmembrane protein 127 [TMEM127], von Hippel-Lindau [VHL]) and five extended panel (egl-9 family hypoxia inducible factor 1/prolyl hydroxylase domain 2 [EGLN1/PHD2], endothelial PAS domain-containing protein 1 [EPAS1], kinesin family member 1B [KIF1B], receptor tyrosine kinase [MET], succinate dehydrogenase complex assembly factor 2 [SDHAF2]) (C).

• #1 Pheochromocytoma: Diagnosis and Treatment – Patient Education – Endocrine Diseases – AAES

  https://endocrinediseases.org/adrenal/pheochromocytoma-diagnosis-and-treatment/

  A pheochromocytoma is an adrenal tumor that makes and releases excess catecholamines. These tumors can cause serious health problems including stroke, heart attack, and even death. […] For patients with pheochromocytoma, genetic testing should be considered if a patient has one or more of the following: Diagnosis at younger than 30 years old, Bilateral pheochromocytomas, Paraganglioma, Family history of pheochromocytoma or paraganglioma, Family history of the syndromes previously described, Other signs or symptoms of the syndromes previously described. […] About 10% of pheochromocytomas are cancerous. Telling the difference between a benign and a cancerous pheochromocytoma may be difficult. The best predictor of cancer is whether or not there is spread to other organs (metastases), invasion of other organs, or recurrence after surgery.

• #1 Pheochromocytoma: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/124059-overview

  Additional studies to rule out a familial syndrome in patients with confirmed pheochromocytoma include the following: Serum intact parathyroid hormone level and a simultaneous serum calcium level to rule out primary hyperparathyroidism (which occurs in MEN 2A). Screening for mutations in the ret proto-oncogene (which give rise to MEN 2A and 2B). Genetic testing for mutations causing the MEN 2A and 2B syndromes. Consultation with an ophthalmologist to rule out retinal angiomas (VHL disease).

• #1 Pheochromocytoma Evaluation | Test Detail | Quest Diagnostics

  https://testdirectory.questdiagnostics.com/test/test-detail/10520/pheochromocytoma-evaluation?p=r&cc=MASTER

  Pheochromocytoma Evaluation – Pheochromocytoma is a tumor of the adrenal gland associated with headaches, cyclic changes in blood pressure, sweating, and other symptoms. Pheochromocytomas produce catecholamines and metanephrine. […] It is preferable for the patient to be off medications for three days prior to collection. Patient should avoid tobacco, tea, coffee, for three days prior to specimen collection. Common antihypertensives (diuretics, ACE inhibitors, calcium channel blockers, alpha and beta blockers) cause minimal or no interference. Medications which are alpha agonists (aldomet), alpha blockers (dibenzyline) should be avoided 18-24 hours prior to specimen collection. […] This test was developed and its analytical performance characteristics have been determined by Quest Diagnostics. It has not been cleared or approved by FDA. This assay has been validated pursuant to the CLIA regulations and is used for clinical purposes.

• #1 Pheochromocytoma: Diagnosis and Treatment – Patient Education – Endocrine Diseases – AAES

  https://endocrinediseases.org/adrenal/pheochromocytoma-diagnosis-and-treatment/

  In patients who are thought to have a pheochromocytoma, laboratory tests (blood and/or urine) should be done to measure the levels of adrenaline, as well as other hormones that are produced during the normal production and processing of adrenaline. […] When the diagnosis is confirmed with blood or urine tests, the patient should have a CAT scan or MRI to locate the pheochromocytoma. Pheochromocytomas usually have a very distinct appearance on CAT scan and MRI. […] One common mistake is to perform a biopsy when an adrenal tumor is discovered. This can be disastrous if the tumor is a pheochromocytoma. Adrenal masses should never be biopsied unless a pheochromocytoma/paraganglioma has been ruled out first. […] Not treating a pheochromocytoma is incredibly dangerous and life-threatening. The best treatment for pheochromocytoma is to surgically remove the adrenal gland(s) with the tumor.

• #1 Treatment of pheochromocytoma in adults – UpToDate

  https://www.uptodate.com/contents/treatment-of-pheochromocytoma-in-adults

  Pheochromocytoma is a rare neuroendocrine tumor, occurring in less than 0.2 percent of patients with hypertension. In approximately 60 percent of patients, the tumor is discovered incidentally during computed tomography (CT) or magnetic resonance imaging (MRI) of the abdomen for unrelated symptoms. […] The clinical manifestations, diagnosis, and genetics of pheochromocytoma and management of metastatic pheochromocytoma are discussed separately. […] Once a pheochromocytoma is diagnosed, all patients should undergo a resection of the pheochromocytoma following appropriate medical preparation. […] Resecting a pheochromocytoma is a high-risk surgical procedure and an experienced surgeon/anesthesiologist team is required.

• #1 Pheochromocytoma: Diagnosis and Treatment – Patient Education – Endocrine Diseases – AAES

  https://endocrinediseases.org/adrenal/pheochromocytoma-diagnosis-and-treatment/

  Before surgery, you must be appropriately prepared with medications that will block the effects of adrenaline during the start of anesthesia and during the operation. […] Surgery is performed under general anesthesia with you completely asleep and relaxed. Blood pressure swings, abnormal heart rhythms, and bleeding can occur during pheochromocytoma surgery, so it is important that the surgical and anesthesia team are experienced in treating this disease.

• #1 Pheochromocytoma: Not your everyday diagnosis

  https://www.myamericannurse.com/pheochromocytoma-not-your-everyday-diagnosis/

  Analysis of Ms. Wares 24-hour urine specimen revealed excessive catecholamine levels. […] The MRI revealed a mass on her left adrenal gland, and the MIBG confirmed that it was a pheochromocytoma and revealed no extra-adrenal tumors. […] Surgical removal of a pheochromocytoma is a high-risk procedure. […] Today, most tumors are removed laparoscopically, unless they are large, or there are lots of them. […] One to two weeks after surgery, a patient has follow-up biochemical assays to ensure that all catecholamine-producing tumor cells have been removed. […] Complete surgical resection of a benign pheochromocytoma usually means a normal life expectancy. […] For some types of pheochromocytoma, care includes genetic counseling. […] About one-half of paragangliomas are malignant. Be sure to encourage any patient with a paraganglioma to follow the genetic counseling protocol.

• #1 Pheochromocytoma > Fact Sheets > Yale Medicine

  https://www.yalemedicine.org/conditions/pheochromocytoma

  With treatment, the outlook is better. In many cases, surgical removal of the tumor can cure people of the disease. […] Because of this risk, people who have been treated for pheochromocytoma should get regular check-ups to monitor the possible return of the disease. […] At Yale, we provide a robust multidisciplinary approach to the treatment of pheochromocytomas, with involvement of experienced clinicians in the fields of medical endocrinology, endocrine surgery, radiology, and anesthesiology, says Dr. Gibson.

• #1 Diagnosis for Pheochromocytoma and Paraganglioma: A Joint Position Statement of the Korean Pheochromocytoma and Paraganglioma Task Force

  https://www.e-enm.org/journal/view.php?number=2149

  As a result of overarching research, PPGLs has been known as the representative hereditary tumor and newly classified as a malignant tumor. Also, great progress has been made regarding biochemical tests, functional imaging, and genetic testing. Accordingly, we can diagnose PPGLs more appropriately, detect multifocal or metastatic lesions, and predict prognosis better.

• #2 How do health care providers diagnose pheochromocytoma? | NICHD – Eunice Kennedy Shriver National Institute of Child Health and Human Development

  https://www.nichd.nih.gov/health/topics/pheochrom/conditioninfo/diagnosed

  A health care provider uses blood and urine tests that measure catecholamines and/or their metabolites to diagnose pheochromocytoma. Higher-than-typical amounts of these biochemical substances in the blood and/or urine can be an indication of the presence of a pheochromocytoma/paraganglioma. […] Multiple studies at the National Institutes of Health (NIH) have demonstrated the utility of measuring metanephrines in the blood for the diagnosis of pheochromocytoma/paraganglioma. This method is often recommended as the first diagnostic test when there is suspicion of a pheochromocytoma/paraganglioma. […] The location of a pheochromocytoma can be determined by using several imaging methods, including computed tomography (CT) and magnetic resonance imaging (MRI). A third imaging method that can be used to detect pheochromocytomas is MIBG (metaiodobenzylguanidine) scintigraphy. During this procedure, MIBG, a compound containing a small amount of radioactivity, is injected into a vein and is picked up by pheochromocytoma cells but not healthy cells. Any MIBG that is seen with the scanner can indicate the presence of pheochromocytoma cells.

• #2 Azthena logo with the word Azthena

  https://www.news-medical.net/health/Pheochromocytoma-Diagnosis.aspx

  Pheochromocytoma is a catecholamine-producing tumor that originates from chromaffin cells of the adrenergic system, most commonly in the adrenal medulla. There are two main steps in the diagnosis of pheochromocytoma: biochemical demonstration of excessive catecholamine secretion and radiological localization of the causative tumor. […] In any case, the diagnosis should be suspected in patients with one or more of the following: The onset of hypertension at a young age, Resistant or labile hypertension, Hypertensive response during anesthesia, surgery, or angiography, Paroxysmal and self-limiting episodes of typical adrenergic symptoms, Incidentally discovered adrenal mass, Family history of pheochromocytoma, Familial syndrome predisposing to catecholamine-secreting tumors. […] Today, the diagnosis of pheochromocytoma has been made simple by significant developments in the assays that are used to both detect and quantify catecholamine levels in the blood and urine.

• #2 Pheochromocytoma Workup: Approach Considerations, Metanephrine and Catecholamine Testing, Additional Laboratory Tests

  https://emedicine.medscape.com/article/124059-workup

  Imaging studies should be performed only after biochemical studies have confirmed the diagnosis of pheochromocytoma. Computed tomography (CT) scanning or magnetic resonance imaging (MRI) can be used for detection of the disorder. Scintigraphy may be used when these techniques fail to localize the tumor. […] High-risk patients, including those who have a genetic syndrome that predisposes them to pheochromocytoma (eg, multiple endocrine neoplasia [MEN] types 2A or 2B, von Hippel-Lindau [VHL] disease, neurofibromatosis, a prior history of a pheochromocytoma, a family history of a pheochromocytoma), should be screened with plasma metanephrine testing. […] Although dopamine is a major catecholamine, measurement of dopamine levels in 24-hour urine is not useful, because most urinary dopamine is derived from renal extraction.

• #2 Pheochromocytoma Workup: Approach Considerations, Metanephrine and Catecholamine Testing, Additional Laboratory Tests

  https://emedicine.medscape.com/article/124059-workup

  Catecholamines produced by pheochromocytomas are metabolized within chromaffin cells. Norepinephrine is metabolized to normetanephrine and epinephrine is metabolized to metanephrine. Because this process occurs within the tumor, independently of catecholamine release, pheochromocytomas are best diagnosed by measurement of these metabolites rather than by measurement of the parent catecholamines. […] Guidelines from the North American NeuroEndocrine Tumor Society (NANETS) recommend biochemical testing for pheochromocytoma in the following cases: symptomatic patients, patients with an adrenal incidentaloma, patients who have a hereditary risk for developing a pheochromocytoma or paraganglioma (extra-adrenal pheochromocytoma). […] The choice of diagnostic test should be based on the clinical suspicion of a pheochromocytoma. Plasma metanephrine testing has the highest sensitivity (96%) for detecting a pheochromocytoma, but it has a lower specificity (85%). In comparison, a 24-hour urinary collection for catecholamines and metanephrines has a sensitivity of 87.5% and a specificity of 99.7%.

• #2 Diagnosis and Treatment of Pheochromocytoma

  https://www.ekjm.org/journal/view.php?number=22892

  Sensitivity and specificity of biochemical tests for diagnosis of pheochromocytoma: Plasma-free metanephrines 99% sensitivity, 89% specificity; Plasma catecholamines 84% sensitivity, 81% specificity; Urinary catecholamines 86% sensitivity, 88% specificity; Urinary-fractionated metanephrines 97% sensitivity, 69% specificity; Urinary total metanephrines 77% sensitivity, 93% specificity; VMA 64% sensitivity, 95% specificity. […] Sensitivity and specificity of biochemical and imaging methods for diagnosis of pheochromocytoma: 24-hr urinary tests VMA ++ ++++; Cathecholamines +++ +++ ; Fractionated metanephrines ++++ ++; Total metanephrines +++ ++++; Plasma tests Cathecholamines +++ ++; Free metanephrines ++++ +++ ; Chromogranin A +++ ++; CT ++++ +++ ; MRI ++++ +++ ; MIBG scintigraphy +++ ++++; Somatostatin receptor scintigraphy ++ ++; dopa PET ++++ ++++.

• #2 Pheochromocytoma Symptoms, Treatment, Diagnosis & More

  https://pheopara.org/education/pheochromocytoma

  If a pheo is suspected, you should be referred to a doctor who specializes in hormonal disorders (an endocrinologist). […] The twenty-four hour urine and blood (plasma) tests are commonly used if pheochromocytoma or paraganglioma is suspected. […] The 24-hour urine test and the blood plasma test both measure catecholamines and metanephrines (which are metabolites of catecholamines). […] Metanephrines are released continuously from the tumor, as opposed to catecholamines, which are usually released intermittently. This continuous release of metanephrines from the tumor explains why testing for metanephrines is preferred over testing for catecholamines. […] The 24-hr urine metanephrine test measures the free metanephrines in your urine. […] Plasma free metanephrines test is a blood test that measures the amount of metanephrines in the blood.

• #2 Pheochromocytoma – Wikipedia

  https://en.wikipedia.org/wiki/Pheochromocytoma

  The diagnostic method for pheochromocytoma includes elevated plasma free metanephrines, plasma catecholamines, urinary catecholamines, MRI, and PET Scan. […] A diagnosis of pheochromocytoma should be suspected when the patient simultaneously presents with hypertension and the classic triad of heart palpitations, headaches, and profuse sweating. […] Elevated plasma free metanephrines is considered the gold standard diagnosis for pheochromocytoma. […] While the above conditions are likely to contribute to false-positive results if not controlled for, any value greater than 3 to 4 times the upper reference limit of normal should be considered diagnostic for a pheochromocytoma. […] Twenty-four hour urinary metanephrines are an acceptable alternative if the plasma test is unavailable. […] If metanephrine values are equivocal, chromogranin A can be used as an adjunct marker to predict the presence of a tumor.

• #2 Pheochromocytoma Symptoms, Treatment, Diagnosis & More

  https://pheopara.org/education/pheochromocytoma

  3-methoxytyramine (3MT), which is the breakdown product of dopamine, is sometimes used for testing. […] Sometimes test results are false negative (normal test result in a patient with a pheo), a false positive (abnormal test result in patient with no pheo) or indeterminate (levels may be slightly higher than the upper limit of normal). […] Imaging, taking pictures of the inside of the body, is often used once biochemical tests indicate a pheo or para. […] A CT scan takes a series of detailed pictures inside the body in places such as the neck, chest, abdomen, and pelvis. […] An MRI uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body such as the neck, chest, abdomen, and pelvis. […] Functional imaging allows physicians to see how the body is functioning and to measure its chemical and biological processes.

• #2 Pheochromocytoma Workup: Approach Considerations, Metanephrine and Catecholamine Testing, Additional Laboratory Tests

  https://emedicine.medscape.com/article/124059-workup

  Suppression tests using phentolamine and clonidine can also be used for diagnostic purposes. NANETS guidelines recommend clonidine suppression testing when plasma metanephrine values are less than 4-fold above the upper reference limit. […] Chromogranin A is an acidic monomeric protein that is stored with and secreted with catecholamines. Plasma levels of chromogranin A reportedly are 83% sensitive and 96% specific for identifying a pheochromocytoma. […] Abdominal CT scanning has an accuracy of 85-95% for detecting adrenal masses with a spatial resolution of 1 cm or greater but is less accurate for lesions smaller than 1 cm. […] MRI is preferred for detection of pheochromocytoma in children and in pregnant or lactating women. MRI has a reported sensitivity of up to 100% in detecting adrenal pheochromocytomas, does not require contrast, and does not expose the patient to ionizing radiation.

• #2 Pheochromocytoma – WikiAnesthesia

  https://wikianesthesia.org/wiki/Pheochromocytoma

  Diagnosis of pheochromocytoma relies on measurement of excessive levels of catecholamines and its metabolites followed by imaging studies. Catecholamine hypersecretion can be confirmed by measurement of urinary and plasma catecholamines; and/or metanephrine and normetanephrine, metabolites of epinephrine and norepinephrine. […] 24 hour urine levels of these compounds have classically been used to diagnose pheochromocytoma and is the most reliable test. […] Plasma metanephrine and normetanephrine is also commonly used for diagnosis but notably has a lower specificity (89%) than 24-hour urine levels. […] When equivocal results occur, a clonidine suppression test can be used. Clonidine is an alpha-2 adrenergic antagonist and decreases catecholamine levels. In patients with pheochromocytoma, clonidine will fail to suppress the catecholamine levels.

• #2 Pheochromocytoma: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/124059-overview

  Diagnostic tests for pheochromocytoma include the following: Plasma metanephrine testing: 96% sensitivity, 85% specificity. 24-hour urinary collection for catecholamines and metanephrines: 87.5% sensitivity, 99.7% specificity. […] Test selection criteria include the following: Use plasma metanephrine testing in patients at high risk (ie, those with predisposing genetic syndromes or a family or personal history of pheochromocytoma). Use 24-hour urinary collection for catecholamines and metanephrines in patients at lower risk. […] Imaging studies should be performed only after biochemical studies have confirmed the diagnosis of pheochromocytoma. Studies are as follows: Abdominal CT scanning: Has accuracy of 85-95% for detecting adrenal masses with a spatial resolution of 1 cm or greater. MRI: Preferred over CT scanning in children and pregnant or lactating women; has reported sensitivity of up to 100% in detecting adrenal pheochromocytomas. Scintigraphy: Reserved for biochemically confirmed cases in which CT scanning or MRI does not show a tumor. PET scanning: A promising technique for detection and localization of pheochromocytomas.

• #2 Imaging and Tests for Pheochromocytoma Adrenal Tumors

  https://www.adrenal.com/pheochromocytoma/imaging-scans

  Doctors have a number of options when they order scans or x-rays to look for the presence of a pheochromocytoma within the adrenal gland. […] The gold standard of adrenal imaging is a CT scan (CAT scan). An adrenal-protocol, contrast enhancement CT scan is best. Thus a CT Scan of the adrenal with and without contrast should always be the first scan ordered, and in more than 90% of cases the ONLY scan a patient will need. […] MRI has similar diagnostic accuracy to CT allowing characterizing adenomas regardless of their CT enhancement. […] The MIBG Scan is used only to detect the presence and location of pheochromocytomas and paragangliomas. […] DOTATATE PET/CT should now be considered the ideal first line investigation for imaging pheochromocytoma and paraganglioma. […] Pheochromocytomas on Contrast-Enhanced CT Scans. Pheos can present with solid, cystic, calcific, and/or necrotic components–quite variable. Smaller pheos tend to display a more uniform attenuation, with a density of 40-50 HU. After contrast administration, pheochromocytomas enhance avidly (meaning turn very bright) with some of them showing higher enhancement on the portal venous phase and other on the arterial phase; nevertheless, their APW and RPW are similar to those of adenomas. Therefore, pheochromocytomas often cannot be reliably differentiated from adenomas using CT washout protocols.

• #2 How is pheochromocytoma diagnosed?

  https://www.adrenal.com/blog/how-is-pheochromocytoma-diagnosed

  Genetic testing can identify these mutations and help diagnose the condition. […] A glucose test can be performed to see if these spikes occur after the administration of glucose. […] If you are experiencing any symptoms associated with pheochromocytoma, such as high blood pressure, headaches, or heart palpitations, it’s important to seek an expert evaluation. Your doctor can perform the necessary tests to diagnose or rule out pheochromocytoma. […] In 95% of cases all you need is a CT scan. The best CT scan is an adrenal-protocol CT scan which with and without contrast. If the CT scan shows an adrenal tumor and your adrenaline levels are high, you will need adrenal surgery for a likely pheochromocytoma. […] If you belong to any of the following individuals, you need to be screened (tested) to ensure you do not have a pheochromocytoma or paraganglioma. […] If you have been diagnosed with pheochromocytoma, adrenal surgery may be recommended to remove the tumor. […] Adrenal surgery is considered the most effective treatment for pheochromocytoma.

• #2 Adrenal pheochromocytoma: Keys to radiologic diagnosis | Radiología (English Edition)

  https://www.elsevier.es/en-revista-radiologia-english-edition–419-articulo-adrenal-pheochromocytoma-keys-radiologic-diagnosis-S217351072200091X?newsletter=true

  Although the clinical presentation and laboratory abnormalities can guide the diagnosis of pheochromocytoma, it is not uncommon for the radiologist to be the first to suggest it from the findings in an imaging test. […] We need to remember that the characteristic presentation is a well-defined adrenal nodular lesion which has a high signal intensity on T2-weighted images, rapid and intense enhancement and, frequently, cystic and hemorrhagic features. […] In uncertain cases, measurement of catecholamines or additional Nuclear Medicine tests may be recommended.

• #2 Pheochromocytoma Workup: Approach Considerations, Metanephrine and Catecholamine Testing, Additional Laboratory Tests

  https://emedicine.medscape.com/article/124059-workup

  A scan with iodine-123 (123I) labeled metaiodobenzylguanidine (MIBG) is reserved for cases in which a pheochromocytoma is confirmed biochemically but CT scanning or MRI does not show a tumor. […] Once the diagnosis of pheochromocytoma is made, additional studies to rule out a familial syndrome may be indicated. Testing for every possible gene would be inappropriate and expensive; however, the following information can suggest which genetic tests to select: biochemical profile of catecholamine secretion, age of the patient, site of the primary tumor, family history. […] Because of the high sensitivity of MRI and CT scanning, procedures are rarely indicated for localization of pheochromocytomas. Selective venous sampling is seldom performed to localize pheochromocytomas but has occasionally been used to detect extra-adrenal pheochromocytomas that were not found at surgery.

• #2 Diagnosing phaeochromocytomas | Adrenal gland cancer | Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/adrenal-gland-cancer/phaeochromocytoma/diagnosing

  A CT scanner takes x-rays from different angles to form a detailed image of the inside of your body. This can show doctors where a tumour is, how big it is and whether it has spread anywhere else. […] An MRI scanner uses magnetism rather than x-rays to build up a picture of the inside of your body. MRI scans are especially good at showing up soft tissue in the body. […] This is a specialised scan that doctors might use to diagnose phaeochromocytoma. They can also see if it has spread to other parts of your body. […] In some hospitals, instead of an MIBG scan, you may have a DOTATATE PET scan. This helps to diagnose phaeochromocytoma and to see if it has spread. […] This is the most common type of PET scan. The doctors use a different radioactive drug (tracer) called fluorodeoxyglucose (FDG). You might have this type of PET scan before your operation to remove the tumour.

• #2 Detecting Pheochromocytoma

  https://pmc.ncbi.nlm.nih.gov/articles/PMC1449983/

  To define the most sensitive biochemical test to establish the diagnosis of pheochromocytoma and also to assess the potential role of iodine 131-labeled metaiodobenzylguanidine scintigraphy (131I-MIBG) in the diagnosis of this tumor. […] The most sensitive tests were total urinary normetanephrine (96.9%), platelet norepinephrine (93.8%), and 131I-MIBG scintigraphy (83.7%). […] The tests of choice to establish the diagnosis of pheochromocytoma are urinary normetanephrine and platelet norepinephrine. […] We thus advocate performing an MIBG scan if the diagnosis of pheochromocytoma is clinically suspected and catecholamine measurements are within the normal range. […] Based on prospectively collected data on 152 patients with pheochromocytoma, we found that the most sensitive tests were total urinary normetanephrine (96.9%), platelet norepinephrine (93.8%), and 131I-MIBG scintigraphy (83.7%).

• #2 Pheochromocytoma | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/phaeochromocytoma-1?lang=us

  Pheochromocytomas are an uncommon tumor of the adrenal gland, with characteristic clinical, and to a lesser degree, imaging features. The first investigation in cases where pheochromocytoma is suspected is usually 24-hour urinary or plasma metanephrines, metabolites of norepinephrine and epinephrine. When results are positive, imaging is then performed to try and localize the tumor or tumors. Approximately 10% of all pheochromocytomas are not located in the adrenal glands. Extra-adrenal tumors are more likely to be malignant and metastasize. It is also important to note that it is not possible to distinguish malignant from benign pheochromocytomas merely on the direct appearance of the mass. Rather, the distinction is made on demonstrating evidence of direct tumor invasion into adjacent organs/structures or the presence of metastases. CT is the first imaging modality to be used, with an overall sensitivity of 89%. MRI is the most sensitive modality for the identification of pheochromocytomas and is particularly useful in cases of extra-adrenal location. Some agents can be used to attempt to image pheochromocytomas and are especially useful in trying to locate an extra-adrenal tumor (when CT of the abdomen is negative) or metastatic deposits. I-123 MIBG uptake in an adrenal nodule is strong supporting evidence for a pheochromocytoma. Overall sensitivity is ~80%. 18F-DOPA PET is thought to be highly sensitive according to initial results. Gallium-68 DOTATATE PET-CT imaging has also been advocated due to their higher lesion to background tissue contrast and high specificity for pheochromocytoma.

• #2 Pheochromocytoma: Diagnosis and Treatment – Patient Education – Endocrine Diseases – AAES

  https://endocrinediseases.org/adrenal/pheochromocytoma-diagnosis-and-treatment/

  A pheochromocytoma is an adrenal tumor that makes and releases excess catecholamines. These tumors can cause serious health problems including stroke, heart attack, and even death. […] For patients with pheochromocytoma, genetic testing should be considered if a patient has one or more of the following: Diagnosis at younger than 30 years old, Bilateral pheochromocytomas, Paraganglioma, Family history of pheochromocytoma or paraganglioma, Family history of the syndromes previously described, Other signs or symptoms of the syndromes previously described. […] About 10% of pheochromocytomas are cancerous. Telling the difference between a benign and a cancerous pheochromocytoma may be difficult. The best predictor of cancer is whether or not there is spread to other organs (metastases), invasion of other organs, or recurrence after surgery.

• #2 Pheochromocytoma Diagnosis & Treatment – Cancer Therapy Advisor

  https://www.cancertherapyadvisor.com/ddi/pheochromocytoma/

  Computed tomography (CT) scan or magnetic resonance imaging (MRI) should be used to localize the tumor and confirm a diagnosis of pheochromocytoma. MRI is indicated over a CT scan in certain conditions, such as for patients with contrast allergy, the pediatric population, and pregnant patients. It should be noted that these imaging modalities are sensitive but not specific; a very specific test for catecholamine-secreting tumors is the 123I-labeled metaiodobenzylguanidine (MIBG) scintigraphy. MIBG can help to differentiate between adrenal and paraganglionic tumors and can also help reveal multiple lesions and metastases. […] Genetic testing should be determined on a case-by-case basis, with potential indications including a family history of catecholamine-producing tumor, multiple tumors at different sites, metastases, patient age of less than 50 years, or bilateral tumors.

• #2 Azthena logo with the word Azthena

  https://www.news-medical.net/health/Pheochromocytoma-Diagnosis.aspx

  Moreover, as levels of these metabolites evince a universal feature of pheochromocytoma a continuous metabolism of catecholamines within the tumor the measurement of plasma metanephrines is thought to be diagnostically superior to measuring only serum catecholamines. […] Chromogranin A is a major secretory protein present in the chromaffin granules and a very effective tumor marker that can improve the sensitivity of diagnostic testing and long-term follow-up for pheochromocytoma. […] Upon establishing the biochemical diagnosis of catecholamine excess, radiographic studies are undertaken in order to pinpoint the exact location of the tumor. The test of choice is cross-sectional imaging (either by using computed tomography or magnetic resonance imaging) of the abdomen and pelvis, which is followed by imaging studies of the neck and chest. […] Nuclear scintigraphy with radioiodine is then used to confirm the catecholamine-secreting nature of the tumor, but it can also be utilized to localize tumors that were not seen with computed tomography or magnetic resonance imaging.

• #2 Pheochromocytoma | Columbia Surgery

  https://columbiasurgery.org/conditions-and-treatments/pheochromocytoma

  Pheochromocytomas are rare tumors that make too much adrenaline. […] Even after removing the tumor it is often impossible to tell the difference between a malignant and benign pheochromocytoma under the microscope. The only definite way to tell if it is a malignant pheochromocytoma is if there is spread into nearby organs (i.e. local invasion), spread to distant sites like the lung and bone (i.e. metastases), or cancer that comes back (i.e. recurrence). […] Patients who are suspected of having a pheochromocytoma should be tested to see if they have too much catecholamines in their system. […] After confirming the diagnosis of pheochromocytoma, imaging tests will be done to find the location of the tumor(s). […] Once an adrenal tumor is found, it must NOT be biopsied. Doing a biopsy of a pheochromocytoma or paraganglioma can lead to a pheo crisis and possibly even death.

• #2 Pheochromocytoma: Diagnosis and Treatment – Patient Education – Endocrine Diseases – AAES

  https://endocrinediseases.org/adrenal/pheochromocytoma-diagnosis-and-treatment/

  In patients who are thought to have a pheochromocytoma, laboratory tests (blood and/or urine) should be done to measure the levels of adrenaline, as well as other hormones that are produced during the normal production and processing of adrenaline. […] When the diagnosis is confirmed with blood or urine tests, the patient should have a CAT scan or MRI to locate the pheochromocytoma. Pheochromocytomas usually have a very distinct appearance on CAT scan and MRI. […] One common mistake is to perform a biopsy when an adrenal tumor is discovered. This can be disastrous if the tumor is a pheochromocytoma. Adrenal masses should never be biopsied unless a pheochromocytoma/paraganglioma has been ruled out first. […] Not treating a pheochromocytoma is incredibly dangerous and life-threatening. The best treatment for pheochromocytoma is to surgically remove the adrenal gland(s) with the tumor.

• #2 Pheochromocytoma: Not your everyday diagnosis

  https://www.myamericannurse.com/pheochromocytoma-not-your-everyday-diagnosis/

  Analysis of Ms. Wares 24-hour urine specimen revealed excessive catecholamine levels. […] The MRI revealed a mass on her left adrenal gland, and the MIBG confirmed that it was a pheochromocytoma and revealed no extra-adrenal tumors. […] Surgical removal of a pheochromocytoma is a high-risk procedure. […] Today, most tumors are removed laparoscopically, unless they are large, or there are lots of them. […] One to two weeks after surgery, a patient has follow-up biochemical assays to ensure that all catecholamine-producing tumor cells have been removed. […] Complete surgical resection of a benign pheochromocytoma usually means a normal life expectancy. […] For some types of pheochromocytoma, care includes genetic counseling. […] About one-half of paragangliomas are malignant. Be sure to encourage any patient with a paraganglioma to follow the genetic counseling protocol.

• #2 Pheochromocytoma | Columbia Surgery

  https://columbiasurgery.org/conditions-and-treatments/pheochromocytoma

  The best treatment for pheochromocytoma is to remove it surgically. […] Before an operation to remove a pheochromocytoma, the patient must be properly prepared with certain medications to block the effects of adrenaline that will be released during the operation. […] Even with the best pre-operative preparation, the patient may still have wide blood pressure changes, irregular heart rhythms, and bleeding.

• #2 Phaeochromocytoma (Investigations and Treatment)

  https://patient.info/doctor/phaeochromocytoma-pro

  CT is the initial imaging modality of choice – it is sensitive and detects around 85-95% of tumours in excess of 1 cm in diameter. […] All patients should be involved in shared decision-making for genetic testing. […] Histological assessment of tissue removed after surgery using certain criteria (the PASS system) can help to differentiate benign from malignant tumours. […] Surgical resection of the tumour is the treatment of choice and usually results in cure of the hypertension. […] After surgery, a 24-hour urine collection for total catecholamines, metanephrines and VMA is required two weeks after operation. […] If the urine tests for total catecholamines, VMA and metanephrines are positive, the main differential diagnosis is to decide if is it is part of a familial condition.

• #2 Pheochromocytoma – Hormonal and Metabolic Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/hormonal-and-metabolic-disorders/adrenal-gland-disorders/pheochromocytoma

  A pheochromocytoma is a tumor that usually originates from the chromaffin cells of the adrenal glands, causing overproduction of catecholamines, powerful hormones that induce high blood pressure and other symptoms. […] Doctors measure the blood levels of catecholamines or products created when catecholamines are broken down by the body and use imaging tests to try to find the tumor. […] Doctors may not suspect a pheochromocytoma, because almost half of the people have no symptoms other than persistent high blood pressure. However, when high blood pressure occurs in a young adult, comes and goes, or accompanies other symptoms of pheochromocytoma, doctors may request certain laboratory tests. For example, the level of certain catecholamines or products created when these catecholamines are broken down may be measured in blood or urine samples. […] If the level of catecholamines is high, computed tomography (CT), magnetic resonance imaging (MRI), or another imaging test can help locate the pheochromocytoma. […] Once pheochromocytoma has been confirmed, doctors typically do genetic tests on the person.

• #3 Pheochromocytoma – Endocrinology Advisor

  https://www.endocrinologyadvisor.com/ddi/pheochromocytoma/

  Computed tomography (CT) scan or magnetic resonance imaging (MRI) should be used to localize the tumor and confirm a diagnosis of pheochromocytoma. MRI is indicated over a CT scan in certain conditions, such as for patients with contrast allergy, the pediatric population, and pregnant patients. It should be noted that these imaging modalities are sensitive but not specific; a very specific test for catecholamine-secreting tumors is the 123I-labeled metaiodobenzylguanidine (MIBG) scintigraphy. MIBG can help to differentiate between adrenal and paraganglionic tumors and can also help reveal multiple lesions and metastases. […] If metanephrine levels are four times higher than the normal reference value, the next step is to localize the hormone-secreting tumor. If metanephrine levels are only minimally elevated or ambiguous and the clinician still suspects pheochromocytoma, the clonidine suppression test can help confirm a diagnosis.

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