Materiały źródłowe

• #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 clinical presentation and diagnosis of pheochromocytoma are important because the tumors have similar clinical presentations and are treated with similar approaches. […] Hereditary catecholamine-secreting tumors typically present at a younger age than sporadic neoplasms. […] Most catecholamine-secreting tumors are sporadic. However, approximately 40 percent of patients have the disease as part of a familial disorder; in these patients, the catecholamine-secreting tumors are more likely to be bilateral adrenal pheochromocytomas or paragangliomas.

• #2 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. This is a result of increased release of the catecholamines epinephrine and norepinephrine. […] 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. […] Levels of epinephrine (adrenaline), norepinephrine (noradrenaline), and their metabolites (breakdown products of epinephrine and norepinephrine) can be measured in either urine or blood.

• #3 Pheochromocytoma: Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/23373-pheochromocytoma

  If youre diagnosed with pheochromocytoma, your provider may recommend genetic counseling to find out your risk for having an inherited syndrome and other related cancers. […] The best treatment option is surgery, when feasible. […] Treatment options for pheochromocytoma depend on several factors, including: The size of the tumor. […] Surgery is the main form of treatment for pheochromocytoma. […] Approximately 90% of pheochromocytomas are successfully removed by surgery. […] If pheochromocytomas are left untreated, they can potentially cause serious, life-threatening complications, including: Heart muscle disease (cardiomyopathy). […] If youve been diagnosed with a pheochromocytoma and are experiencing concerning symptoms, contact your healthcare provider. […] If youve recently found out that one of your first-degree relatives (siblings and parents) has a genetic syndrome, such as multiple endocrine neoplasia 2 syndrome or von Hippel-Lindau (VHL) disease, that puts you at a higher risk of developing pheochromocytoma.

• #4 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. […] In the past, biochemical screening involved measurement of urine excretion of catecholamines and total metanephrines in the 24-hour urine. 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.

• #5 Pheochromocytoma – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pheochromocytoma/diagnosis-treatment/drc-20355372

  To find out if you have a pheochromocytoma, your healthcare professional likely will order various tests. […] These tests measure levels of the hormones adrenaline and noradrenaline, and substances that can come from those hormones called metanephrines. Raised levels of metanephrines are more common when a person has a pheochromocytoma. Metanephrine levels are less likely to be high when a person has symptoms due to something other than pheochromocytoma. […] If the lab test results find signs of a pheochromocytoma, imaging tests are needed. Your healthcare professional likely will order one or more of these tests to find out if you have a tumor. […] Your healthcare professional might recommend genetic tests to see whether a pheochromocytoma is related to a genetic condition. Information about possible genetic factors can be important for many reasons.

• #6 Pheochromocytoma, diagnosis and treatment: Review of the…

  https://sciendo.com/article/10.1515/enr-2017-0018

  Objective. We conducted an extensive review of the literature and tried to cite the most recent recommendations concerning the pheochromocytoma (PHEO). […] Results. More than four times elevation of plasma fractionated metanephrines or elevated 24-h urinary fractionated metanephrines are keys to diagnosing pheochromocytoma. If the results are equivocal then we perform the clonidine test. If we have not done it already, we preferably do a CT scan and/or an MRI scan. The patient needs pre-treatment with 1-blockers at least 1014 days before operation. Alternatives or sometimes adjuncts are Calcium Channels Blockers and/or -Blockers. Several familial syndromes are associated with PHEO and genetic testing should be considered. […] Conclusions. The biggest problem for pheochromocytoma is to suspect it in the first place. Elevated metanephrines establish the diagnosis. With the proper preoperative preparation the risks during operation and the postoperative period are minimal. If there is a risk of the hereditable mutation, it is strongly suggested that all the patients with pheochromocytoma need clinical genetic testing.

• #7 Diagnosis of pheochromocytoma

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3339753/

  Pheochromocytoma is a rare tumor of chromaffin cells accounting for less than 0.3% of all cases of hypertension. Majority of them are benign and are a surgically curable cause of hypertension. Aggressive diagnostics and surgical intervention are recommended because failure to diagnose the tumor can result in uncontrolled catecholamines release leading to catastrophic consequences. […] Many biochemical diagnostic tests and imaging modalities exist for diagnosis and localization of tumor; however, optimum approach remains debatable. […] The diagnosis of pheochromocytoma depends on demonstration of excessive production of catecholamines or its metabolites in urine or plasma. 24 h urine tests are considered superior to plasma tests mainly because tumor often secrete catecholamines intermittently and the short half-life of catecholamines can result in relatively normal plasma catecholamine levels even in the presence of a functional tumor. Measurement of VMA in urine is relatively inexpensive, easy to perform, and although it has acceptable specificity (95%), it has a low sensitivity (64%). Urinary total metanephrines has a similar specificity but higher sensitivity (77%) and several studies have found it to be the most sensitive test for the diagnosis of pheochromocytoma.

• #8 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%.

• #9 Diagnosis of pheochromocytoma

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3339753/

  Measurement of free metanephrines in plasma has emerged as promising and reliable initial test in the diagnosis of pheochromocytoma compared to conventional tests with sensitivity of 99% and specificity of 89%. […] MIBG offers an approach to overcome the limitations of anatomic imaging. The diagnostic specificity of MIBG has been reported close to 100% as it is concentrated, released, and stored in the chromaffin granules; however, it was negative in our case probably due to necrotic lesion suggestive on CT scan. CT and magnetic resonance imaging (MRI) are believed to be the best-available anatomic imaging techniques and choice depends on institutional preference. Both imaging studies have excellent sensitivity but lack adequate specificity for unequivocally confirming a mass such as a pheochromocytoma. The recent introduction of combined positron emission tomography/CT scanning has emerged as a promising test but is not widely available.

• #10 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.

• #11 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 (3) 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.

• #12 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. […] A fractionated plasma free metanephrine level may be measured in a standard venipuncture sample, drawn about 15-20 minutes after intravenous catheter insertion.

• #13 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%.

• #14 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

  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. […] For this reason, 24-hour urine testing remains the gold standard. […] 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.

• #15 Phaeochromocytoma Diagnosis – Endocrinesurgery.net.au

  http://www.endocrinesurgery.net.au/phaeochromocytoma-diagnosis/

  Screening is achieved by either blood testing, urine testing or both. […] Plasma free metanephrines is the most common screening test, and is now freely available. It can be less specific with potential factitious catecholamine elevation due to anxiety, with a false-positive rate of 10-15%, so repeating the test several times will help. […] 24-hour urinary metanephrines or urinary catecholamines are highly specific tests, although it is possible to use nocturnal collection, one hour samples or even spot collection. […] Imaging is a vital tool in localising the tumour. As 90% of the chromaffin tumours occur in the abdomen and pelvis, CT and MRI scanning will usually detect phaeochromocytomas in the adrenals, but also paragangliomas elsewhere in the abdomen, and possible metastases in the case of malignancy.

• #16 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

  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. […] For this reason, 24-hour urine testing remains the gold standard. […] 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.

• #17 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.

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

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

  Perform a 24-hour urine collection for creatinine, total catecholamines, vanillylmandelic acid, and metanephrines. […] 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.

• #19 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

  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). […] 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).

• #20 Pheochromocytoma, diagnosis and treatment: Review of the…

  https://sciendo.com/article/10.1515/enr-2017-0018

  Objective. We conducted an extensive review of the literature and tried to cite the most recent recommendations concerning the pheochromocytoma (PHEO). […] Results. More than four times elevation of plasma fractionated metanephrines or elevated 24-h urinary fractionated metanephrines are keys to diagnosing pheochromocytoma. If the results are equivocal then we perform the clonidine test. If we have not done it already, we preferably do a CT scan and/or an MRI scan. The patient needs pre-treatment with 1-blockers at least 1014 days before operation. Alternatives or sometimes adjuncts are Calcium Channels Blockers and/or -Blockers. Several familial syndromes are associated with PHEO and genetic testing should be considered. […] Conclusions. The biggest problem for pheochromocytoma is to suspect it in the first place. Elevated metanephrines establish the diagnosis. With the proper preoperative preparation the risks during operation and the postoperative period are minimal. If there is a risk of the hereditable mutation, it is strongly suggested that all the patients with pheochromocytoma need clinical genetic testing.

• #21 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. […] A fractionated plasma free metanephrine level may be measured in a standard venipuncture sample, drawn about 15-20 minutes after intravenous catheter insertion.

• #22 Phaeochromocytoma Diagnosis – Endocrinesurgery.net.au

  http://www.endocrinesurgery.net.au/phaeochromocytoma-diagnosis/

  Screening is achieved by either blood testing, urine testing or both. […] Plasma free metanephrines is the most common screening test, and is now freely available. It can be less specific with potential factitious catecholamine elevation due to anxiety, with a false-positive rate of 10-15%, so repeating the test several times will help. […] 24-hour urinary metanephrines or urinary catecholamines are highly specific tests, although it is possible to use nocturnal collection, one hour samples or even spot collection. […] Imaging is a vital tool in localising the tumour. As 90% of the chromaffin tumours occur in the abdomen and pelvis, CT and MRI scanning will usually detect phaeochromocytomas in the adrenals, but also paragangliomas elsewhere in the abdomen, and possible metastases in the case of malignancy.

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

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

  Perform a 24-hour urine collection for creatinine, total catecholamines, vanillylmandelic acid, and metanephrines. […] 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.

• #24 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.

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

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

  Perform a 24-hour urine collection for creatinine, total catecholamines, vanillylmandelic acid, and metanephrines. […] 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.

• #26 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.

• #27 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. […] CT is the first imaging modality to be used, with an overall sensitivity of 89%. This is on account of 98% of tumors being located within the abdomen and 90% limited to the adrenal glands. MRI is the most sensitive modality for the identification of pheochromocytomas and is particularly useful in cases of extra-adrenal location. The overall sensitivity is said to be 98%. […] 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%.

• #28 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

  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). […] 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).

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

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

  Perform a 24-hour urine collection for creatinine, total catecholamines, vanillylmandelic acid, and metanephrines. […] 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.

• #30 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. […] CT is the first imaging modality to be used, with an overall sensitivity of 89%. This is on account of 98% of tumors being located within the abdomen and 90% limited to the adrenal glands. MRI is the most sensitive modality for the identification of pheochromocytomas and is particularly useful in cases of extra-adrenal location. The overall sensitivity is said to be 98%. […] 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%.

• #31 Phaeochromocytoma

  https://dermnetnz.org/topics/phaeochromocytoma

  Iodine-123 metaiodobenzylguanidinescan (I23IMIBG SPECT) has excellent specificity, and sensitivity of 90100% in detection of small tumours 12 cm. It can be used for planning MIBG therapy in metastatic disease. […] Positron emission tomography (PET) as PET-CT or PET-MRI with fluorine-18-L-dihydroxyphenylalanine (18F-DOPA) has excellent specificity and sensitivity of 90100% for 18FDOPA in detection of small tumours 12 cm. This has higher spatial resolution and a more selective, clearer radiotracer accumulation in phaeochromocytomas compared to 123IMIBG SPECT. However, it is less widely available and is more expensive. […] 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.

• #32 Detecting Pheochromocytoma

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

  The objective of the present investigation was to define the most sensitive biochemical tests to establish the diagnosis of pheochromocytoma. […] Total urinary normetanephrine was the most sensitive test (96.9%, 95% confidence interval [CI]), 90.8%100%; rate of false-negativity = 3.1%), followed by platelet norepinephrine (93.8%, 95% CI, 85.4%100%; rate of false-negativity = 6.2%). […] In combination with 131I-MIBG scintigraphy, platelet norepinephrine had a sensitivity of 100% (95% CI, 91.9%100%), plasma norepinephrine/MIBG of 97.1% (95% CI, 91.4%100.0%), total urine normetanephrine/MIBG of 96.6% (95% CI, 89.9%100.0%), and urine norepinephrine/MIBG of 95.3% (95% CI, 90.8%99.8%). […] We found that total urinary normetanephrine is the single most sensitive test in the detection of pheochromocytoma followed by platelet norepinephrine. […] Therefore, if pheochromocytoma is clinically suspected and the patient has normal catecholamine levels, MIBG scan should be performed.

• #33 Phaeochromocytoma

  https://dermnetnz.org/topics/phaeochromocytoma

  Iodine-123 metaiodobenzylguanidinescan (I23IMIBG SPECT) has excellent specificity, and sensitivity of 90100% in detection of small tumours 12 cm. It can be used for planning MIBG therapy in metastatic disease. […] Positron emission tomography (PET) as PET-CT or PET-MRI with fluorine-18-L-dihydroxyphenylalanine (18F-DOPA) has excellent specificity and sensitivity of 90100% for 18FDOPA in detection of small tumours 12 cm. This has higher spatial resolution and a more selective, clearer radiotracer accumulation in phaeochromocytomas compared to 123IMIBG SPECT. However, it is less widely available and is more expensive. […] 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.

• #34 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

  3.3. 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). […] 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).

• #35 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 clinical presentation and diagnosis of pheochromocytoma are important because the tumors have similar clinical presentations and are treated with similar approaches. […] Hereditary catecholamine-secreting tumors typically present at a younger age than sporadic neoplasms. […] Most catecholamine-secreting tumors are sporadic. However, approximately 40 percent of patients have the disease as part of a familial disorder; in these patients, the catecholamine-secreting tumors are more likely to be bilateral adrenal pheochromocytomas or paragangliomas.

• #36 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

  4.2. 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).

• #37 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

  4.2. 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).

• #38 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

  4.2. 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).

• #39 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. […] A fractionated plasma free metanephrine level may be measured in a standard venipuncture sample, drawn about 15-20 minutes after intravenous catheter insertion.

• #40 Pheochromocytoma: Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/23373-pheochromocytoma

  If youre diagnosed with pheochromocytoma, your provider may recommend genetic counseling to find out your risk for having an inherited syndrome and other related cancers. […] The best treatment option is surgery, when feasible. […] Treatment options for pheochromocytoma depend on several factors, including: The size of the tumor. […] Surgery is the main form of treatment for pheochromocytoma. […] Approximately 90% of pheochromocytomas are successfully removed by surgery. […] If pheochromocytomas are left untreated, they can potentially cause serious, life-threatening complications, including: Heart muscle disease (cardiomyopathy). […] If youve been diagnosed with a pheochromocytoma and are experiencing concerning symptoms, contact your healthcare provider. […] If youve recently found out that one of your first-degree relatives (siblings and parents) has a genetic syndrome, such as multiple endocrine neoplasia 2 syndrome or von Hippel-Lindau (VHL) disease, that puts you at a higher risk of developing pheochromocytoma.

• #41 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. Pheochromocytomas are quite rare and occur in about 2 to 8 out of every 1 million people. Approximately 95% of catecholamine-secreting tumors are located within the abdomen, and of these, 85 to 90% are in the adrenal glands. Approximately 10% of patients are found to have pheochromocytomas in both adrenal glands, which is most commonly seen in younger patients with pheochromocytoma-related genetic syndromes. […] 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.

• #42 Pheochromocytoma – Wikipedia

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

  If metanephrine values are equivocal, chromogranin A can be used as an adjunct marker to predict the presence of a tumor. […] The first step is to repeat the labs, taking extra precautions to follow the gold standard diagnosis described above, including the conditions of collection, pharmaceutical interference, and any potential diet and lifestyle habits that could alter the results. […] Plasma methoxytyramine is a breakdown product of the catecholamine, dopamine. […] Anatomic imaging refers to computed tomography (CT) or magnetic resonance imaging (MR) scans. […] If a person has the characteristic signs and symptoms of a pheochromocytoma and the decision is made to pursue additional biochemical evaluation, the differential diagnosis is important as it is more likely to be something other than a pheochromocytoma given the relative frequency of 0.8 per 100,000 person-years.

• #43 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.

• #44 Pheochromocytoma – Wikipedia

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

  If metanephrine values are equivocal, chromogranin A can be used as an adjunct marker to predict the presence of a tumor. […] The first step is to repeat the labs, taking extra precautions to follow the gold standard diagnosis described above, including the conditions of collection, pharmaceutical interference, and any potential diet and lifestyle habits that could alter the results. […] Plasma methoxytyramine is a breakdown product of the catecholamine, dopamine. […] Anatomic imaging refers to computed tomography (CT) or magnetic resonance imaging (MR) scans. […] If a person has the characteristic signs and symptoms of a pheochromocytoma and the decision is made to pursue additional biochemical evaluation, the differential diagnosis is important as it is more likely to be something other than a pheochromocytoma given the relative frequency of 0.8 per 100,000 person-years.

• #45 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. […] 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. […] Pheochromocytomas vary from 2 g to 3 kg but, on average, weigh 100 g (a healthy adrenal gland weighs 4-6 g). These tumors are well encapsulated, highly vascular, and appear reddish brown on cut section.

• #46 UK Vet Companion Animal – Canine phaeochromocytoma: a guide to diagnosis and treatment

  https://www.ukvetcompanionanimal.com/content/clinical/canine-phaeochromocytoma-a-guide-to-diagnosis-and-treatment/

  Phaeochromocytomas are neuroendocrine tumours arising from chromaffin cells of the adrenal medulla. The diagnosis of phaeochromocytoma relies on clinical suspicion, biochemical testing, diagnostic imaging and histopathology. Biochemical testing mainly depends on the measurement of levels of plasma or urinary metanephrines, with normetanephrine demonstrating superior diagnostic performance compared to metanephrine. The diagnosis of a phaeochromocytoma relies on clinical suspicion, biochemical testing and diagnostic imaging findings. The definitive diagnosis relies on histopathology and immunohistochemistry using neuroendocrine markers. Biochemical testing for phaeochromocytomas in dogs has only been carried out for the past 10-15 years and has consisted of evaluation of plasma and urinary levels of catecholamines and metanephrines, as well as urinary vanillylmandelic acid. The current recommendation for biochemical testing for phaeochromocytomas includes measuring plasma or urinary metanephrines. Studies have demonstrated that normetanephrine exhibits better diagnostic performance than metanephrine in both plasma and urine for differentiation between dogs with phaeochromocytomas and those with other diseases, including hypercortisolism. Currently, there is no consensus regarding the preferred use of plasma or urine for metanephrine measurements in terms of diagnostic accuracy. Once the biochemical diagnosis of a phaeochromocytoma is made, patients should undergo diagnostic imaging. Because of its widespread availability and non-invasive nature, abdominal ultrasonography is the primary method for evaluating phaeochromocytomas in dogs. The shape, size, architecture and symmetry of the adrenals can be assessed, as well as local invasion into surrounding structures and the presence of abdominal metastasis. The definitive diagnosis of a phaeochromocytoma is based on histological examination and immunohistochemistry.

• #47 Phaeochromocytoma (Investigations and Treatment)

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

  Histological assessment of tissue removed after surgery using certain criteria (the PASS system) can help to differentiate benign from malignant tumours. A PASS score of 4 is predictive of benign phaeochromocytomas, whereas a score greater than 6 is characteristic of malignant tumours. […] Surgical resection of the tumour is the treatment of choice and usually results in cure of the hypertension. Pre-operative treatment with alpha-blockers and beta-blockers is required to control blood pressure and prevent intraoperative hypertensive crises. […] After surgery, a 24-hour urine collection for total catecholamines, metanephrines and VMA is required two weeks after operation. If results are normal, the prognosis is excellent. It is important to ensure that hypertension is controlled or resolved. Lifelong annual biochemical testing is recommended to detect recurrent or metastatic disease.

• #48 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

  3.3. 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). […] 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).

• #49 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

  4.2. 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).

• #50 Pheochromocytoma – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pheochromocytoma/diagnosis-treatment/drc-20355372

  To find out if you have a pheochromocytoma, your healthcare professional likely will order various tests. […] These tests measure levels of the hormones adrenaline and noradrenaline, and substances that can come from those hormones called metanephrines. Raised levels of metanephrines are more common when a person has a pheochromocytoma. Metanephrine levels are less likely to be high when a person has symptoms due to something other than pheochromocytoma. […] If the lab test results find signs of a pheochromocytoma, imaging tests are needed. Your healthcare professional likely will order one or more of these tests to find out if you have a tumor. […] Your healthcare professional might recommend genetic tests to see whether a pheochromocytoma is related to a genetic condition. Information about possible genetic factors can be important for many reasons.

• #51 Pheochromocytoma, diagnosis and treatment: Review of the…

  https://sciendo.com/article/10.1515/enr-2017-0018

  Objective. We conducted an extensive review of the literature and tried to cite the most recent recommendations concerning the pheochromocytoma (PHEO). […] Results. More than four times elevation of plasma fractionated metanephrines or elevated 24-h urinary fractionated metanephrines are keys to diagnosing pheochromocytoma. If the results are equivocal then we perform the clonidine test. If we have not done it already, we preferably do a CT scan and/or an MRI scan. The patient needs pre-treatment with 1-blockers at least 1014 days before operation. Alternatives or sometimes adjuncts are Calcium Channels Blockers and/or -Blockers. Several familial syndromes are associated with PHEO and genetic testing should be considered. […] Conclusions. The biggest problem for pheochromocytoma is to suspect it in the first place. Elevated metanephrines establish the diagnosis. With the proper preoperative preparation the risks during operation and the postoperative period are minimal. If there is a risk of the hereditable mutation, it is strongly suggested that all the patients with pheochromocytoma need clinical genetic testing.

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

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

  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. […] 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 (i.e. noradrenaline, metanephrines, normetanephrines, and dopamine). […] 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. These tests can also help determine if other organs are involved or if other tumors outside the adrenal glands (i.e. paragangliomas) exist. […] 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. Even then, a biopsy is rarely helpful or necessary.

• #53 Pheochromocytoma | Columbia Surgery

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

  Pheochromocytomas are rare tumors that make too much adrenaline. […] 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. […] 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.

• #54 Phaeochromocytoma (Investigations and Treatment)

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

  Histological assessment of tissue removed after surgery using certain criteria (the PASS system) can help to differentiate benign from malignant tumours. A PASS score of 4 is predictive of benign phaeochromocytomas, whereas a score greater than 6 is characteristic of malignant tumours. […] Surgical resection of the tumour is the treatment of choice and usually results in cure of the hypertension. Pre-operative treatment with alpha-blockers and beta-blockers is required to control blood pressure and prevent intraoperative hypertensive crises. […] After surgery, a 24-hour urine collection for total catecholamines, metanephrines and VMA is required two weeks after operation. If results are normal, the prognosis is excellent. It is important to ensure that hypertension is controlled or resolved. Lifelong annual biochemical testing is recommended to detect recurrent or metastatic disease.

• #55 Pheochromocytoma – National Adrenal Diseases Foundation

  https://www.nadf.us/pheochromocytoma.html

  Pheochromocytomas (PCCs) are tumors of the chromaffin cells that arise within the adrenal medulla. They belong to a group of diseases termed neuroendocrine tumors (NETs). Pheochromocytomas are related to another group of endocrine tumors called paragangliomas which occur outside the adrenal gland and originate at any level of extra-adrenal paraganglia (from the skull base to the pelvic floor). […] The diagnosis of pheochromocytoma is often delayed, impacting patients and their families. In part, these tumors can have an insidious course with non-specific symptoms, and in many cases they can be silent tumors. […] Therefore, the diagnosis and management of these uncommon tumors should be carried out in specialized centers with expertise in the management of these tumors. […] Internists and endocrinologists suspect pheochromocytoma when they see unexplained symptoms suggestive of the disease. Work up should be done in a controlled setting under very strict criteria and protocol. Plasma or urine tests establish elevation of catecholamines or metanephrines (metabolites of catecholamines). Imaging studies can be used to establish the location of the tumors. These include anatomical imaging with CT or MRI and Functional Imaging with DOTATATE and other PET scans. Once diagnosis is established, blood is analyzed for a genetic panel of genes known to cause pheochromocytoma or paraganglioma.

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