Materiały źródłowe

• #1 Paraganglioma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK549834/

  Paragangliomas are rare neuroendocrine tumors arising from extra-adrenal paraganglia. […] This activity explores paragangliomas, covering etiology, pathophysiology, evaluation, and management. […] It delves into genetic and environmental factors influencing development and offers guidance on diagnostics, including genetic testing and imaging. […] Identify the clinical manifestations and imaging characteristics associated with paragangliomas. […] Select appropriate diagnostic modalities for confirming the diagnosis of paraganglioma. […] Apply evidence-based guidelines for managing paragangliomas, including implementing timely and appropriate surgical interventions. […] A surgical biopsy performed during resection is the gold standard for confirming the diagnosis. However, it does not distinguish between pheochromocytomas and paragangliomas. Typically, clinical correlation suffices for diagnosis, aided by imaging and pathological findings of the lesion.

• #2 Paraganglioma: Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/22394-paraganglioma

  A paraganglioma is a rare but treatable neuroendocrine tumor that usually forms along major blood vessels and nerve pathways in your neck and head. […] Paragangliomas can be benign (not cancer) or malignant (cancer). Approximately 20% of paragangliomas are malignant. […] It can be very challenging for healthcare providers to tell if a paraganglioma is cancerous or not even after they’ve looked at the tumor tissue under a microscope after it’s been removed. […] Since paraganglioma is a rare tumor and is sometimes asymptomatic, it can be difficult to diagnose. Healthcare providers sometimes find paragangliomas when they order a test or procedure for another reason. […] Your healthcare provider may use the following tests and procedures to diagnose paraganglioma: Physical exam, 24-hour urine test, Blood catecholamine tests, PET scan, CT scan, MRI.

• #3 Paraganglioma: Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/22394-paraganglioma

  A paraganglioma is a rare but treatable neuroendocrine tumor that usually forms along major blood vessels and nerve pathways in your neck and head. […] Paragangliomas can be benign (not cancer) or malignant (cancer). Approximately 20% of paragangliomas are malignant. […] It can be very challenging for healthcare providers to tell if a paraganglioma is cancerous or not even after they’ve looked at the tumor tissue under a microscope after it’s been removed. […] Since paraganglioma is a rare tumor and is sometimes asymptomatic, it can be difficult to diagnose. Healthcare providers sometimes find paragangliomas when they order a test or procedure for another reason. […] Your healthcare provider may use the following tests and procedures to diagnose paraganglioma: Physical exam, 24-hour urine test, Blood catecholamine tests, PET scan, CT scan, MRI.

• #4 Epidemiology, clinical presentation, and diagnosis of paragangliomas – UpToDate

  https://www.uptodate.com/contents/paragangliomas-epidemiology-clinical-presentation-diagnosis-and-histology

  Epidemiology, clinical presentation, and diagnosis of paragangliomas […] Paragangliomas are rare neuroendocrine tumors that arise from the extra-adrenal autonomic paraganglia, small organs consisting mainly of neuroendocrine cells that are derived from the embryonic neural crest and have the ability to secrete catecholamines. […] Catecholamine-secreting paragangliomas often present clinically like pheochromocytomas with hypertension, episodic headache, sweating, and tachycardia. However, the distinction between pheochromocytoma and paraganglioma is an important one because of implications for associated neoplasms, risk for malignancy, and genetic testing. […] This topic review will cover the epidemiology, risk factors, molecular pathogenesis, histology, clinical manifestations, diagnosis, and genetic screening issues of paragangliomas arising at a variety of sites in the body.

• #5 Epidemiology, clinical presentation, and diagnosis of paragangliomas – UpToDate

  https://www.uptodate.com/contents/paragangliomas-epidemiology-clinical-presentation-diagnosis-and-histology

  In this article, the term paraganglioma is used to designate both functioning (catecholamine-secreting) and nonfunctioning tumors arising in the paraganglia outside of the adrenal gland, and the term pheochromocytoma is used to designate those tumors that arise in the adrenal glands. […] […] […] Diagnosis: Localizing the tumor, Radioisotope imaging. […] […] […] Clinical presentation and diagnosis of pheochromocytoma are discussed separately.

• #6 Paraganglioma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK549834/

  Paragangliomas are rare neuroendocrine tumors arising from extra-adrenal paraganglia. […] This activity explores paragangliomas, covering etiology, pathophysiology, evaluation, and management. […] It delves into genetic and environmental factors influencing development and offers guidance on diagnostics, including genetic testing and imaging. […] Identify the clinical manifestations and imaging characteristics associated with paragangliomas. […] Select appropriate diagnostic modalities for confirming the diagnosis of paraganglioma. […] Apply evidence-based guidelines for managing paragangliomas, including implementing timely and appropriate surgical interventions. […] A surgical biopsy performed during resection is the gold standard for confirming the diagnosis. However, it does not distinguish between pheochromocytomas and paragangliomas. Typically, clinical correlation suffices for diagnosis, aided by imaging and pathological findings of the lesion.

• #7 Paraganglioma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK549834/

  Evaluation of catecholamine-secreting tumors involves comprehensive biochemical testing, imaging studies, and genetic testing to determine hereditary predispositions and guide appropriate management strategies. […] The initial step in diagnosing a pheochromocytoma or a paraganglioma involves evaluating patients for the excess production of catecholamines, followed by anatomical identification of the tumor. […] The evaluation typically begins with measuring urinary or plasma fractionated metanephrines, as the transformation of catecholamines into metanephrines within the tumor persists regardless of the tumor’s secretion of catecholamines. […] After confirming catecholamine excess, contrast-enhanced computed tomography (CT) scanning or magnetic resonance imaging (MRI) of the abdomen and pelvis are the imaging tests of choice.

• #8 Paraganglioma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK549834/

  Evaluation of catecholamine-secreting tumors involves comprehensive biochemical testing, imaging studies, and genetic testing to determine hereditary predispositions and guide appropriate management strategies. […] The initial step in diagnosing a pheochromocytoma or a paraganglioma involves evaluating patients for the excess production of catecholamines, followed by anatomical identification of the tumor. […] The evaluation typically begins with measuring urinary or plasma fractionated metanephrines, as the transformation of catecholamines into metanephrines within the tumor persists regardless of the tumor’s secretion of catecholamines. […] After confirming catecholamine excess, contrast-enhanced computed tomography (CT) scanning or magnetic resonance imaging (MRI) of the abdomen and pelvis are the imaging tests of choice.

• #9 What is the rationale for the laboratory workup for suspected pheochromocytomas and paragangliomas? | Cleveland Clinic Journal of Medicine

  https://www.ccjm.org/content/90/3/150

  Selection of screening tests for pheochromocytomas and paragangliomas (PPGLs) is best guided by high clinical suspicion. […] Biochemical testing for pheochromocytoma is indicated in patients who have symptoms of catecholamine excess, an adrenal incidentaloma, or a hereditary predisposition to development of PPGLs. […] The diagnostic sensitivity of plasma free metanephrines is above 96% and the specificity is about 89%. […] The sensitivity of 24-hour urinary fractionated metanephrines is 86% and specificity is above 69%. […] For accurate results, the blood sample for plasma free metanephrines must be collected by an indwelling catheter placed 30 minutes prior to the draw, and the patient must be in a supine position for the full 30 minutes. […] A 3-fold to 4-fold rise above the upper limit of normal for plasma free metanephrines or urinary fractionated metanephrines is unlikely to be a false-positive result.

• #10 What is the rationale for the laboratory workup for suspected pheochromocytomas and paragangliomas? | Cleveland Clinic Journal of Medicine

  https://www.ccjm.org/content/90/3/150

  Selection of screening tests for pheochromocytomas and paragangliomas (PPGLs) is best guided by high clinical suspicion. […] Biochemical testing for pheochromocytoma is indicated in patients who have symptoms of catecholamine excess, an adrenal incidentaloma, or a hereditary predisposition to development of PPGLs. […] The diagnostic sensitivity of plasma free metanephrines is above 96% and the specificity is about 89%. […] The sensitivity of 24-hour urinary fractionated metanephrines is 86% and specificity is above 69%. […] For accurate results, the blood sample for plasma free metanephrines must be collected by an indwelling catheter placed 30 minutes prior to the draw, and the patient must be in a supine position for the full 30 minutes. […] A 3-fold to 4-fold rise above the upper limit of normal for plasma free metanephrines or urinary fractionated metanephrines is unlikely to be a false-positive result.

• #11 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. Biochemical tests of pheochromocytoma/paraganglioma […] 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). […] 3. Imaging of pheochromocytoma/paraganglioma […] 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).

• #12 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. Biochemical tests of pheochromocytoma/paraganglioma […] 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). […] 3. Imaging of pheochromocytoma/paraganglioma […] 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).

• #13 What is the rationale for the laboratory workup for suspected pheochromocytomas and paragangliomas? | Cleveland Clinic Journal of Medicine

  https://www.ccjm.org/content/90/3/150

  Selection of screening tests for pheochromocytomas and paragangliomas (PPGLs) is best guided by high clinical suspicion. […] Biochemical testing for pheochromocytoma is indicated in patients who have symptoms of catecholamine excess, an adrenal incidentaloma, or a hereditary predisposition to development of PPGLs. […] The diagnostic sensitivity of plasma free metanephrines is above 96% and the specificity is about 89%. […] The sensitivity of 24-hour urinary fractionated metanephrines is 86% and specificity is above 69%. […] For accurate results, the blood sample for plasma free metanephrines must be collected by an indwelling catheter placed 30 minutes prior to the draw, and the patient must be in a supine position for the full 30 minutes. […] A 3-fold to 4-fold rise above the upper limit of normal for plasma free metanephrines or urinary fractionated metanephrines is unlikely to be a false-positive result.

• #14 What is the rationale for the laboratory workup for suspected pheochromocytomas and paragangliomas? | Cleveland Clinic Journal of Medicine

  https://www.ccjm.org/content/90/3/150

  Fasting blood levels of plasma 3-methoxytyramine should be measured to evaluate for dopamine-secreting PPGLs if initial metanephrine testing is negative but the index of clinical suspicion for PPGL remains high. […] The rationale for timely diagnosis of PPGLs relies on a high index of suspicion and awareness of clinical features.

• #15 Paraganglioma | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/paraganglioma-1?embed_domain=hackmd.io%252f%2540yipuafecsl2jsu8smr5njq%252fbnjhjgjghjghjghfavicon.ico&lang=us

  CT and MRI are the initial imaging modalities for tumor localization. They have excellent sensitivity but lack specificity in unequivocally identifying a mass as a paraganglioma. […] Treatment may include surgical resection or radiotherapy. Malignancy is defined as evidence of metastases. Most common sites for malignancy include lymph nodes, liver, lung, and bone. […] Biopsy (incision or fine needle aspirate) is contraindicated in suspected paragangliomas until biochemical screening is negative for catecholamine excess, due to the risk of catecholamine crisis and severe hypertension.

• #16 Paraganglioma – Diagnosis and Treatment – Patient Education – Endocrine Diseases – AAES

  https://endocrinediseases.org/neuroendocrine-tumor/paraganglioma-diagnosis-and-treatment/

  A paraganglioma is a rare tumor of nerve tissue (located outside of the brain and spinal cord) that often makes and releases too much noradrenaline. […] The diagnosis of a paraganglioma should be suspected in any patient in whom imaging reveals a tumor that is located next to the spine or aorta within the abdomen. When the diagnosis is confirmed with blood or urine tests, the patient should have a CAT scan or MRI to locate the paraganglioma, if not already performed. Paragangliomas usually have a very distinct appearance on CT scan and MRI. […] One common mistake is to perform a biopsy when a tumor is found to be located near the spine or aorta within the abdomen. This can be disastrous if the tumor is a paraganglioma. Suspicious masses should never be biopsied unless a paraganglioma/pheochromocytoma has been ruled out first. Even then, a biopsy is rarely helpful or necessary.

• #17 Paraganglioma Symptoms, Treatment, Diagnosis & More

  https://pheopara.org/education/paraganglioma

  Paragangliomas, if detected early, can be successfully treated and managed in the vast majority of cases. […] If a para 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). […] In rare cases, a paraganglioma, often in the head/neck, may not produce catecholamines or metanephrines. In these latter cases, an imaging test is the preferred method for diagnosis. […] Imaging, taking pictures of the inside of the body, is often used once biochemical tests indicate a pheo or para. Imaging will help to identify where, how many, and size of the tumor(s). […] The method of diagnosing pheo or para is the same as in non-pregnant individuals, using 24-hour urine or plasma metanephrine testing.

• #18 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. Biochemical tests of pheochromocytoma/paraganglioma […] 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). […] 3. Imaging of pheochromocytoma/paraganglioma […] 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).

• #19 SciELO Brazil – The Pheochromocytoma/Paraganglioma syndrome: an overview on mechanisms, diagnosis and management The Pheochromocytoma/Paraganglioma syndrome: an overview on mechanisms, diagnosis and management

  https://www.scielo.br/j/ibju/a/RhwdYQjYSwrsqRSHMNbjcWR/

  The use of MRI for the diagnosis of PPGL has the following advantages: (1) high sensitivity (93-100%) in detecting adrenal disease, (2) presence of a hypersignal in T2 sequence compared to the liver, in at least 75% of PPGL, (3) better sensitivity to localize intracardiac PGL, (4) possibility of visualization and confirmation of bone metastases suggested by mIBG scintigraphy, and (5) can be performed in pregnant women (second trimester on) (without contrast) and in children and carriers of germline variants, since there is no exposure to ionizing radiation. […] CT has a sensitivity of 93-100%, but low specificity (70%). Sensitivity is lower for small adrenal PPGL and for adrenal medullary hyperplasia. It is also less sensitive in the detection of PGL, small metastases and early recurrence of tumors in the adrenal surgical bed. CT is currently recommended as the first choice for topographic diagnosis of PPGL.

• #20 The Multidisciplinary Management of Paragangliomas of the Head and Neck, Part 1

  https://www.cancernetwork.com/view/multidisciplinary-management-paragangliomas-head-and-neck-part-1

  Paragangliomas most commonly occur in the carotid body, jugulotympanic area, and vagus nerve but have also been reported in other areas of the head and neck. […] Part 1 of this two-part article focuses on techniques for diagnosing paraganglioma and the indications for and use of surgery as primary treatment. […] Part 1 of this two-part article addresses the etiology and epidemiology of paragangliomas, their diagnosis, and surgical approaches to treatment. […] Computed tomography (CT) with thin sections (1-3 mm) is an excellent imaging technique by which to identify paragangliomas and document the extent of tumor with precise evaluation of bone invasion. […] Although CT is the study of choice for the evaluation of bone involvement, magnetic resonance imaging (MRI) provides meticulous soft-tissue detail and defines skull-base, intracranial, dural, and neural involvement, especially with the use of gadolinium enhancement.

• #21 SciELO Brazil – The Pheochromocytoma/Paraganglioma syndrome: an overview on mechanisms, diagnosis and management The Pheochromocytoma/Paraganglioma syndrome: an overview on mechanisms, diagnosis and management

  https://www.scielo.br/j/ibju/a/RhwdYQjYSwrsqRSHMNbjcWR/

  The use of MRI for the diagnosis of PPGL has the following advantages: (1) high sensitivity (93-100%) in detecting adrenal disease, (2) presence of a hypersignal in T2 sequence compared to the liver, in at least 75% of PPGL, (3) better sensitivity to localize intracardiac PGL, (4) possibility of visualization and confirmation of bone metastases suggested by mIBG scintigraphy, and (5) can be performed in pregnant women (second trimester on) (without contrast) and in children and carriers of germline variants, since there is no exposure to ionizing radiation. […] CT has a sensitivity of 93-100%, but low specificity (70%). Sensitivity is lower for small adrenal PPGL and for adrenal medullary hyperplasia. It is also less sensitive in the detection of PGL, small metastases and early recurrence of tumors in the adrenal surgical bed. CT is currently recommended as the first choice for topographic diagnosis of PPGL.

• #22 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

  3.2. 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).

• #23 SciELO Brazil – The Pheochromocytoma/Paraganglioma syndrome: an overview on mechanisms, diagnosis and management The Pheochromocytoma/Paraganglioma syndrome: an overview on mechanisms, diagnosis and management

  https://www.scielo.br/j/ibju/a/RhwdYQjYSwrsqRSHMNbjcWR/

  131I-mIBG scintigraphy has diagnostic sensitivity and specificity of 77-90% and 95-100%, respectively. When 123I is used instead, sensitivity reaches higher values: 83-100%, without loss of specificity. Its use should be considered in cases of adrenal Pheo that are suggestive of benignity. […] 18FDG PET-CT is recommended for aggressive metastatic PPGL, lesions greater than 8 cm and those with PV in the SDHB gene. Sensitivity ranges from 74-100%. […] The most used histological classification to aid in establishing malignancy potential is the PASS score (Pheochromocytoma of the Adrenal Gland Scaled Score) which considers the following items. […] Tumor immunohistochemistry for the succinate dehydrogenases, especially the investigation of SDHB is indicated, as the loss of its expression suggests a germline PV in the SDHB gene and implies greater aggressiveness.

• #24 Imaging of Pheochromocytoma and Paraganglioma | Journal of Nuclear Medicine

  https://jnm.snmjournals.org/content/62/8/1033

  The utility of 68Ga-DOTA-SSA in pheochromocytoma/paraganglioma imaging is presented in Tables 1 and 2. 68Ga-DOTA-SSA studies have shown excellent lesion-based sensitivity in detecting pheochromocytoma and paraganglioma, often more than 92%. Recent publications suggest that 68Ga-DOTA-SSA provides a high detection rate across a wide range of mutations. A metaanalysis comparing the sensitivity of 18F-FDG and 68Ga-DOTA-SSA found that the sensitivity of 68Ga-DOTA-SSA (95%) was superior to that of 18F-FDG (85%). Even when germline mutations were considered, the per-lesion sensitivity of 68Ga-DOTA-SSA was 97%, compared with 79% for 18F-FDG. An additional metaanalysis demonstrated that the sensitivity of 68Ga-DOTA-SSA (93%) is superior to that of 18F-FDOPA (80%), 18F-FDG (74%), and 123I/131I-MIBG (38%).

• #25 Imaging of Pheochromocytoma and Paraganglioma | Journal of Nuclear Medicine

  https://jnm.snmjournals.org/content/62/8/1033

  The utility of 68Ga-DOTA-SSA in pheochromocytoma/paraganglioma imaging is presented in Tables 1 and 2. 68Ga-DOTA-SSA studies have shown excellent lesion-based sensitivity in detecting pheochromocytoma and paraganglioma, often more than 92%. Recent publications suggest that 68Ga-DOTA-SSA provides a high detection rate across a wide range of mutations. A metaanalysis comparing the sensitivity of 18F-FDG and 68Ga-DOTA-SSA found that the sensitivity of 68Ga-DOTA-SSA (95%) was superior to that of 18F-FDG (85%). Even when germline mutations were considered, the per-lesion sensitivity of 68Ga-DOTA-SSA was 97%, compared with 79% for 18F-FDG. An additional metaanalysis demonstrated that the sensitivity of 68Ga-DOTA-SSA (93%) is superior to that of 18F-FDOPA (80%), 18F-FDG (74%), and 123I/131I-MIBG (38%).

• #26 SciELO Brazil – The Pheochromocytoma/Paraganglioma syndrome: an overview on mechanisms, diagnosis and management The Pheochromocytoma/Paraganglioma syndrome: an overview on mechanisms, diagnosis and management

  https://www.scielo.br/j/ibju/a/RhwdYQjYSwrsqRSHMNbjcWR/

  131I-mIBG scintigraphy has diagnostic sensitivity and specificity of 77-90% and 95-100%, respectively. When 123I is used instead, sensitivity reaches higher values: 83-100%, without loss of specificity. Its use should be considered in cases of adrenal Pheo that are suggestive of benignity. […] 18FDG PET-CT is recommended for aggressive metastatic PPGL, lesions greater than 8 cm and those with PV in the SDHB gene. Sensitivity ranges from 74-100%. […] The most used histological classification to aid in establishing malignancy potential is the PASS score (Pheochromocytoma of the Adrenal Gland Scaled Score) which considers the following items. […] Tumor immunohistochemistry for the succinate dehydrogenases, especially the investigation of SDHB is indicated, as the loss of its expression suggests a germline PV in the SDHB gene and implies greater aggressiveness.

• #27 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

  3.2. 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).

• #28 Imaging of Pheochromocytoma and Paraganglioma | Journal of Nuclear Medicine

  https://jnm.snmjournals.org/content/62/8/1033

  In summary, in patients with HNPGL or at high risk of developing HNPGL, functional imaging has high sensitivity and should be used in conjunction with MRI, with the latter required for surgical and treatment planning. In almost every situation, 68Ga-DOTA-SSA is considered the agent of choice, 18F-FDOPA the second choice, and 18F-FDG the third choice.

• #29 Paraganglioma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK549834/

  All patients with a paraganglioma should undergo genetic testing. […] Lesional biopsy is the gold standard for confirming the diagnosis of paragangliomas, although it does not differentiate between pheochromocytomas and paragangliomas. […] Surgical resection remains the first-line treatment for paraganglioma and pheochromocytoma.

• #30 Paraganglioma | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/26614

  Paragangliomas are primarily sporadic, although 30% to 50% of cases are familial. […] A surgical biopsy performed during resection is the gold standard for confirming the diagnosis. However, it does not distinguish between pheochromocytomas and paragangliomas. Typically, clinical correlation suffices for diagnosis, aided by imaging and pathological findings of the lesion. […] Evaluation of catecholamine-secreting tumors involves comprehensive biochemical testing, imaging studies, and genetic testing to determine hereditary predispositions and guide appropriate management strategies. Additionally, functional studies like 24-hour urine collection for metanephrines and catecholamines, plasma-free metanephrines, and clonidine suppression tests may be conducted to assess hormonal secretion patterns and aid in diagnosis.

• #31 Paraganglioma | Radiology Reference Article | Radiopaedia.org

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

  Paragangliomas, previously sometimes called glomus tumors, are uncommon neuroendocrine tumors arising from paraganglia and principally encountered in adults. They are divided into those arising from sympathetic paraganglia (primarily in the chest and abdomen, including pheochromocytoma) and parasympathetic paraganglia (primarily in the head and neck). […] Both anatomical and functional imaging of paragangliomas is required for diagnosis and staging. Anatomical imaging includes CT and MRI. Multiple functional imaging modalities exist: 123I-MIBG scintigraphy, 18F-FDA PET, 18F-DOPA PET, 18F-FDG PET and 68Ga-DOTATATE PET. […] CT and MRI are the initial imaging modalities for tumor localization. They have excellent sensitivity but lack specificity in unequivocally identifying a mass as a paraganglioma. […] Biopsy (incision or fine needle aspirate) is contraindicated in suspected paragangliomas until biochemical screening is negative for catecholamine excess, due to the risk of catecholamine crisis and severe hypertension.

• #32 A Case Report of Paraganglioma: A Diagnosis Not to Be Missed

  https://brieflands.com/articles/ijcm-99817

  It is worth noting that biochemical testing should be done in all PGL cases, even if clinically found to be nonfunctional. […] Radiologic imaging also plays an important role in the evaluation of a PGL. […] Histological examination reveals a thin capsule with polygonal or round epithelioid cells arranging in compact cell nests or trabecular patterns known as Zellballen. […] On IHC evaluation, chief cells usually show positivity for neuroendocrine markers such as synaptophysin, chromogranin, and NSE, and sustentacular cells are S-100 positive. […] Due to the catecholamine crisis leading to severe hypertension, an incisional or fine needle biopsy is contraindicated in a patient with suspected PGL unless there are negative biochemical testings or alpha-adrenergic blockers are prescribed.

• #33 A Case Report of Paraganglioma: A Diagnosis Not to Be Missed

  https://brieflands.com/articles/ijcm-99817

  It is worth noting that biochemical testing should be done in all PGL cases, even if clinically found to be nonfunctional. […] Radiologic imaging also plays an important role in the evaluation of a PGL. […] Histological examination reveals a thin capsule with polygonal or round epithelioid cells arranging in compact cell nests or trabecular patterns known as Zellballen. […] On IHC evaluation, chief cells usually show positivity for neuroendocrine markers such as synaptophysin, chromogranin, and NSE, and sustentacular cells are S-100 positive. […] Due to the catecholamine crisis leading to severe hypertension, an incisional or fine needle biopsy is contraindicated in a patient with suspected PGL unless there are negative biochemical testings or alpha-adrenergic blockers are prescribed.

• #34 A Case Report of Paraganglioma: A Diagnosis Not to Be Missed

  https://brieflands.com/articles/ijcm-99817

  It is worth noting that biochemical testing should be done in all PGL cases, even if clinically found to be nonfunctional. […] Radiologic imaging also plays an important role in the evaluation of a PGL. […] Histological examination reveals a thin capsule with polygonal or round epithelioid cells arranging in compact cell nests or trabecular patterns known as Zellballen. […] On IHC evaluation, chief cells usually show positivity for neuroendocrine markers such as synaptophysin, chromogranin, and NSE, and sustentacular cells are S-100 positive. […] Due to the catecholamine crisis leading to severe hypertension, an incisional or fine needle biopsy is contraindicated in a patient with suspected PGL unless there are negative biochemical testings or alpha-adrenergic blockers are prescribed.

• #35 The Diagnosis and Clinical Significance of Paragangliomas in Unusual Locations

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6162705/

  Paragangliomas are neuroendocrine neoplasms, derived from paraganglia of the sympathetic and parasympathetic nervous systems. […] The distinction of these lesions from epithelial neuroendocrine neoplasms is critical for several reasons. Firstly, the determination of clinical and biochemical features is different from that used for epithelial neuroendocrine tumors. […] The histopathologic diagnosis of paraganglioma (PGL) can be simple when the lesion is in an expected location and when the patient presents with classical symptoms related to catecholamine excess. However, the diagnosis is often missed when they occur in unusual locations. […] Immunohistochemistry plays a key role in confirming the diagnosis. […] The importance of the correct diagnosis and the distinction of PGL from NETs has a number of clinical implications.

• #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. Pathological grading system of pheochromocytoma/paraganglioma […] 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. Genetic testing for pheochromocytoma/paraganglioma […] 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).

• #37 Paraganglioma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK549834/

  All patients with a paraganglioma should undergo genetic testing. […] Lesional biopsy is the gold standard for confirming the diagnosis of paragangliomas, although it does not differentiate between pheochromocytomas and paragangliomas. […] Surgical resection remains the first-line treatment for paraganglioma and pheochromocytoma.

• #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. Pathological grading system of pheochromocytoma/paraganglioma […] 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. Genetic testing for pheochromocytoma/paraganglioma […] 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).

• #39 Paraganglioma | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/26614

  Paragangliomas are primarily sporadic, although 30% to 50% of cases are familial. […] A surgical biopsy performed during resection is the gold standard for confirming the diagnosis. However, it does not distinguish between pheochromocytomas and paragangliomas. Typically, clinical correlation suffices for diagnosis, aided by imaging and pathological findings of the lesion. […] Evaluation of catecholamine-secreting tumors involves comprehensive biochemical testing, imaging studies, and genetic testing to determine hereditary predispositions and guide appropriate management strategies. Additionally, functional studies like 24-hour urine collection for metanephrines and catecholamines, plasma-free metanephrines, and clonidine suppression tests may be conducted to assess hormonal secretion patterns and aid in diagnosis.

• #40 Paraganglioma Clinic | Huntsman Cancer Institute | University of Utah Health

  https://healthcare.utah.edu/huntsmancancerinstitute/screening-prevention/paraganglioma-clinic

  Experts recommend all people with paragangliomas get genetic testing, since up to 40% of these tumors are hereditary. […] We recommend all people with a personal diagnosis of paraganglioma or pheochromocytoma get genetic counseling and testing. […] While most people in this clinic have a mutation in the SDHx genes, some people qualify for this clinic if they have a personal and family history of paragangliomas, even without the genetic mutation.

• #41 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. Pathological grading system of pheochromocytoma/paraganglioma […] 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. Genetic testing for pheochromocytoma/paraganglioma […] 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).

• #42 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. Pathological grading system of pheochromocytoma/paraganglioma […] 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. Genetic testing for pheochromocytoma/paraganglioma […] 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).

• #43 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

  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).

• #44 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

  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).

• #45 Paraganglioma: Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/22394-paraganglioma

  If you’re diagnosed with paraganglioma, your provider will likely recommend genetic counseling to find out your risk for having an inherited syndrome and associated cancers. […] Treatment options for paraganglioma depend on several factors, including the size of the tumor, if the tumor is benign (not cancer) or malignant (cancer), if you have symptoms caused by higher-than-normal levels of catecholamines, if the tumor is in one area only or has spread to other places in your body (metastasized), if the tumor has been diagnosed for the first time or has come back (recurred). […] Surgery is the main form of treatment for paragangliomas. […] After surgery, your provider will check your catecholamine levels in your blood or urine. Normal catecholamine levels are a sign that all the paraganglioma cells were removed.

• #46 The Diagnosis and Clinical Significance of Paragangliomas in Unusual Locations

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6162705/

  Patients with PGLs should instead be diagnosed and monitored with more specific measurements of catecholamines unique to their PGL. […] The second issue is the need for genetic testing that is implied by this diagnosis. […] Pathologists can assist in triaging the diagnosis of genetic predisposition by applying immunohistochemistry. […] The diagnosis of PGL requires biochemical assessment to determine the pattern of catecholamine production by the tumor. […] The molecular cluster of a PGL may also impact the selection of imaging studies used for diagnosis and surveillance. […] The results of genetic testing also impact the likelihood of metastatic potential. […] The identification of SDHx-related pathogenesis has also several clinical implications: the increased risk of metastatic and multifocal primary disease.

• #47 Paraganglioma differential diagnosis – wikidoc

  https://www.wikidoc.org/index.php/Paraganglioma_differential_diagnosis

  Paraganglioma must be differentiated from carcinoid tumor, neuroendocrine carcinoma, medullary thyroid carcinoma, middle ear adenoma, and meningioma. […] Paraganglioma should be differentiated from other tumors such as: […] Clinical diagnosis […] In hereditary VHL, disease techniques such as Southern blotting and gene sequencing can be used to analyse DNA and identify mutations. […] Cardinal Clinical Features” are required for positive diagnosis. […] An elevated concentration of serum growth hormone (GH) and insulin-like growth factor 1(IGF-1) levels is diagnostic of acromegaly. […] An elevated concentration of serum calcium with elevated parathyroid hormone level is diagnostic of primary hyperparathyroidism. […] Increased catecholamines and metanephrines in plasma (blood) or through a 24-hour urine collection.

• #48 The Diagnosis and Clinical Significance of Paragangliomas in Unusual Locations

  https://www.mdpi.com/2077-0383/7/9/280

  Paragangliomas are neuroendocrine neoplasms, derived from paraganglia of the sympathetic and parasympathetic nervous systems. […] The distinction of these lesions from epithelial neuroendocrine neoplasms is critical for several reasons. Firstly, the determination of clinical and biochemical features is different from that used for epithelial neuroendocrine tumors. […] The histopathologic diagnosis of paraganglioma (PGL) can be simple when the lesion is in an expected location and when the patient presents with classical symptoms related to catecholamine excess. However, the diagnosis is often missed when they occur in unusual locations. […] Immunohistochemistry plays a key role in confirming the diagnosis. […] The importance of the correct diagnosis and the distinction of PGL from NETs has a number of clinical implications.

• #49 The Diagnosis and Clinical Significance of Paragangliomas in Unusual Locations

  https://www.mdpi.com/2077-0383/7/9/280

  The clinical presentation with sweating, diarrhea, anxiety and intermittent hypertension can resemble the signs and symptoms of carcinoid syndrome. […] Patients with PGLs should instead be diagnosed and monitored with more specific measurements of catecholamines unique to their PGL. […] The second issue is the need for genetic testing that is implied by this diagnosis. […] Pathologists can assist in triaging the diagnosis of genetic predisposition by applying immunohistochemistry. […] The identification of SDHx-related pathogenesis has also several clinical implications: (i) The increased risk of metastatic and multifocal primary disease, (ii) the possibility of synchronous neoplastic manifestations other than PGL or pheochromocytomas. […] The clinical, biochemical, radiologic and morphological features of PGLs are all important features that are required to properly structure a management plan for patients with such tumors.

• #50 The Diagnosis and Clinical Significance of Paragangliomas in Unusual Locations

  https://www.mdpi.com/2077-0383/7/9/280

  The clinical presentation with sweating, diarrhea, anxiety and intermittent hypertension can resemble the signs and symptoms of carcinoid syndrome. […] Patients with PGLs should instead be diagnosed and monitored with more specific measurements of catecholamines unique to their PGL. […] The second issue is the need for genetic testing that is implied by this diagnosis. […] Pathologists can assist in triaging the diagnosis of genetic predisposition by applying immunohistochemistry. […] The identification of SDHx-related pathogenesis has also several clinical implications: (i) The increased risk of metastatic and multifocal primary disease, (ii) the possibility of synchronous neoplastic manifestations other than PGL or pheochromocytomas. […] The clinical, biochemical, radiologic and morphological features of PGLs are all important features that are required to properly structure a management plan for patients with such tumors.

• #51 Paraganglioma: Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/22394-paraganglioma

  If paragangliomas are left untreated, whether benign or malignant, they can potentially cause serious, life-threatening complications due to the excess amounts of adrenaline and noradrenaline they can secrete. […] If you’ve been diagnosed with paraganglioma and experience concerning symptoms, contact your healthcare provider.

• #52 The Diagnosis and Clinical Significance of Paragangliomas in Unusual Locations

  https://www.mdpi.com/2077-0383/7/9/280

  The clinical presentation with sweating, diarrhea, anxiety and intermittent hypertension can resemble the signs and symptoms of carcinoid syndrome. […] Patients with PGLs should instead be diagnosed and monitored with more specific measurements of catecholamines unique to their PGL. […] The second issue is the need for genetic testing that is implied by this diagnosis. […] Pathologists can assist in triaging the diagnosis of genetic predisposition by applying immunohistochemistry. […] The identification of SDHx-related pathogenesis has also several clinical implications: (i) The increased risk of metastatic and multifocal primary disease, (ii) the possibility of synchronous neoplastic manifestations other than PGL or pheochromocytomas. […] The clinical, biochemical, radiologic and morphological features of PGLs are all important features that are required to properly structure a management plan for patients with such tumors.

• #53 Diagnosis and treatment of a rare tumor‑bladder paraganglioma

  https://www.spandidos-publications.com/10.3892/mco.2020.2110

  Adequate medical preparation is very important before surgery, or hypertensive crisis may happen during the operation and the mortality risk will increase correspondingly. […] Imaging examinations are also very important for diagnosis. Ultrasound and CT / Magnetic Resonance Imaging (MRI) are the basic estimated methods for the shapes, sizes and locations of the tumors. […] For the diagnosis of bladder paraganglioma, CT and MRI have a sensitivity as high as 90-100%, but relatively lower specificity of 70-80%. […] Pathologically, the tumor cells usually grow in a characteristic nested pattern, with full and sometimes granular cytoplasm and surrounded by rich blood vessels. […] Therefore, immunohistochemistry is extremely significant for the definitive diagnosis. […] Surgical resection is the most effective way of treatment, including transurethral resection, partial cystectomy and maybe total cystectomy.

• #54 The Diagnosis and Clinical Significance of Paragangliomas in Unusual Locations

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6162705/

  Patients with PGLs should instead be diagnosed and monitored with more specific measurements of catecholamines unique to their PGL. […] The second issue is the need for genetic testing that is implied by this diagnosis. […] Pathologists can assist in triaging the diagnosis of genetic predisposition by applying immunohistochemistry. […] The diagnosis of PGL requires biochemical assessment to determine the pattern of catecholamine production by the tumor. […] The molecular cluster of a PGL may also impact the selection of imaging studies used for diagnosis and surveillance. […] The results of genetic testing also impact the likelihood of metastatic potential. […] The identification of SDHx-related pathogenesis has also several clinical implications: the increased risk of metastatic and multifocal primary disease.

• #55 Paraganglioma – Wikipedia

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

  A paraganglioma is a rare neuroendocrine neoplasm that may develop at various body sites (including the head, neck, thorax and abdomen). […] There is no test that determines benign from malignant tumors; long-term follow-up is therefore recommended for all individuals with paraganglioma. […] Gallium-68 DOTATATE PET/CT imaging modality may be used to confirm the presence of a paraganglioma.

• #56 Paraganglioma: Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/22394-paraganglioma

  If you’re diagnosed with paraganglioma, your provider will likely recommend genetic counseling to find out your risk for having an inherited syndrome and associated cancers. […] Treatment options for paraganglioma depend on several factors, including the size of the tumor, if the tumor is benign (not cancer) or malignant (cancer), if you have symptoms caused by higher-than-normal levels of catecholamines, if the tumor is in one area only or has spread to other places in your body (metastasized), if the tumor has been diagnosed for the first time or has come back (recurred). […] Surgery is the main form of treatment for paragangliomas. […] After surgery, your provider will check your catecholamine levels in your blood or urine. Normal catecholamine levels are a sign that all the paraganglioma cells were removed.

• #57

  https://link.springer.com/article/10.1007/s11154-022-09773-2

  Diagnosis of pheochromocytoma or paraganglioma (PPGL) in pregnancy has been associated historically with high rates of materno-fetal morbidity and mortality. […] Recent evidence suggests outcomes are improved by recognition of PPGL before or during pregnancy and appropriate medical management with alpha-blockade. […] The major challenge remains diagnosis of PPGL as a rare but possibly life-threatening cause of hypertension in pregnancy; when misdiagnosed as pre-eclampsia, maternal mortality can still be as high as 15%. […] Conversely, diagnosis and medical treatment before delivery is today generally associated with good materno-fetal outcomes. […] Clinical suspicion for PPGL in pregnancy should be followed by prompt biochemical testing in the same manner as for nonpregnant patients, although it should be noted normal ranges have not been clearly established for pregnant subjects.

• #58

  https://link.springer.com/article/10.1007/s11154-022-09773-2

  Diagnosis of pheochromocytoma or paraganglioma (PPGL) in pregnancy has been associated historically with high rates of materno-fetal morbidity and mortality. […] Recent evidence suggests outcomes are improved by recognition of PPGL before or during pregnancy and appropriate medical management with alpha-blockade. […] The major challenge remains diagnosis of PPGL as a rare but possibly life-threatening cause of hypertension in pregnancy; when misdiagnosed as pre-eclampsia, maternal mortality can still be as high as 15%. […] Conversely, diagnosis and medical treatment before delivery is today generally associated with good materno-fetal outcomes. […] Clinical suspicion for PPGL in pregnancy should be followed by prompt biochemical testing in the same manner as for nonpregnant patients, although it should be noted normal ranges have not been clearly established for pregnant subjects.

• #59

  https://link.springer.com/article/10.1007/s11154-022-09773-2

  Diagnosis of pheochromocytoma or paraganglioma (PPGL) in pregnancy has been associated historically with high rates of materno-fetal morbidity and mortality. […] Recent evidence suggests outcomes are improved by recognition of PPGL before or during pregnancy and appropriate medical management with alpha-blockade. […] The major challenge remains diagnosis of PPGL as a rare but possibly life-threatening cause of hypertension in pregnancy; when misdiagnosed as pre-eclampsia, maternal mortality can still be as high as 15%. […] Conversely, diagnosis and medical treatment before delivery is today generally associated with good materno-fetal outcomes. […] Clinical suspicion for PPGL in pregnancy should be followed by prompt biochemical testing in the same manner as for nonpregnant patients, although it should be noted normal ranges have not been clearly established for pregnant subjects.

• #60

  https://link.springer.com/article/10.1007/s11154-022-09773-2

  Diagnosis of pheochromocytoma or paraganglioma (PPGL) in pregnancy has been associated historically with high rates of materno-fetal morbidity and mortality. […] Recent evidence suggests outcomes are improved by recognition of PPGL before or during pregnancy and appropriate medical management with alpha-blockade. […] The major challenge remains diagnosis of PPGL as a rare but possibly life-threatening cause of hypertension in pregnancy; when misdiagnosed as pre-eclampsia, maternal mortality can still be as high as 15%. […] Conversely, diagnosis and medical treatment before delivery is today generally associated with good materno-fetal outcomes. […] Clinical suspicion for PPGL in pregnancy should be followed by prompt biochemical testing in the same manner as for nonpregnant patients, although it should be noted normal ranges have not been clearly established for pregnant subjects.

• #61 Paraganglioma Symptoms, Treatment, Diagnosis & More

  https://pheopara.org/education/paraganglioma

  Paragangliomas, if detected early, can be successfully treated and managed in the vast majority of cases. […] If a para 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). […] In rare cases, a paraganglioma, often in the head/neck, may not produce catecholamines or metanephrines. In these latter cases, an imaging test is the preferred method for diagnosis. […] Imaging, taking pictures of the inside of the body, is often used once biochemical tests indicate a pheo or para. Imaging will help to identify where, how many, and size of the tumor(s). […] The method of diagnosing pheo or para is the same as in non-pregnant individuals, using 24-hour urine or plasma metanephrine testing.

• #62

  https://link.springer.com/article/10.1007/s11154-022-09773-2

  Following biochemical confirmation of PPGL, imaging is best performed by MRI in pregnancy, without gadolinium. […] Diagnosis of metastatic PPGL is rare during pregnancy; carefully selected patients with pre-existing metastatic PPGLs may have successful pregnancies with appropriate medical and obstetric care. […] Undiagnosed PPGL increases the risk of maternal and fetal morbidity and mortality. […] Both studies found maternal and fetal mortality were unequivocally higher with PPGL diagnosed postpartum than antepartum, including deaths before delivery could occur; conversely, diagnosis of PPGL antepartum was associated with very low risk of maternal mortality (1%) and lower risk of fetal mortality. […] Once diagnosed, pregnant women with PPGL should ideally be managed in expert centres with multidisciplinary access to high-level obstetric and neonatal care, endocrinologists and anaesthesiologists experienced in the management of catecholaminergic crises.

• #63 International consensus statement on the diagnosis and management of phaeochromocytoma and paraganglioma in children and adolescents | Nature Reviews Endocrinology

  https://www.nature.com/articles/s41574-024-01024-5

  Phaeochromocytomas and paragangliomas (PPGL) are rare neuroendocrine tumours that arise not only in adulthood but also in childhood and adolescence. Up to 70-80% of childhood PPGL are hereditary, accounting for a higher incidence of metastatic and/or multifocal PPGL in paediatric patients than in adult patients. […] The management of PPGL in childhood is complicated by the high incidence of multifocality and/or recurrence and metastatic disease, together with the limited evidence base and paucity of international guidance and the lack of clinical trials. […] Thus, germline genetic testing has high priority in the diagnostic work-up and guides personalized diagnostic, management, therapeutic and surveillance strategies in children and adolescents with PPGL. […] Clinical suspicion of PPGL should be raised in paediatric patients with (a) signs and symptoms of catecholamine excess and (b) an incidentally discovered adrenal or extra-adrenal mass. The risk of PPGL is higher in children with pathogenic variants in tumour susceptibility genes and/or a previous history of PPGL compared with those without.

• #64 International consensus statement on the diagnosis and management of phaeochromocytoma and paraganglioma in children and adolescents | Nature Reviews Endocrinology

  https://www.nature.com/articles/s41574-024-01024-5

  Phaeochromocytomas and paragangliomas (PPGL) are rare neuroendocrine tumours that arise not only in adulthood but also in childhood and adolescence. Up to 70-80% of childhood PPGL are hereditary, accounting for a higher incidence of metastatic and/or multifocal PPGL in paediatric patients than in adult patients. […] The management of PPGL in childhood is complicated by the high incidence of multifocality and/or recurrence and metastatic disease, together with the limited evidence base and paucity of international guidance and the lack of clinical trials. […] Thus, germline genetic testing has high priority in the diagnostic work-up and guides personalized diagnostic, management, therapeutic and surveillance strategies in children and adolescents with PPGL. […] Clinical suspicion of PPGL should be raised in paediatric patients with (a) signs and symptoms of catecholamine excess and (b) an incidentally discovered adrenal or extra-adrenal mass. The risk of PPGL is higher in children with pathogenic variants in tumour susceptibility genes and/or a previous history of PPGL compared with those without.

• #65 International consensus statement on the diagnosis and management of phaeochromocytoma and paraganglioma in children and adolescents | Nature Reviews Endocrinology

  https://www.nature.com/articles/s41574-024-01024-5

  Phaeochromocytomas and paragangliomas (PPGL) are rare neuroendocrine tumours that arise not only in adulthood but also in childhood and adolescence. Up to 70-80% of childhood PPGL are hereditary, accounting for a higher incidence of metastatic and/or multifocal PPGL in paediatric patients than in adult patients. […] The management of PPGL in childhood is complicated by the high incidence of multifocality and/or recurrence and metastatic disease, together with the limited evidence base and paucity of international guidance and the lack of clinical trials. […] Thus, germline genetic testing has high priority in the diagnostic work-up and guides personalized diagnostic, management, therapeutic and surveillance strategies in children and adolescents with PPGL. […] Clinical suspicion of PPGL should be raised in paediatric patients with (a) signs and symptoms of catecholamine excess and (b) an incidentally discovered adrenal or extra-adrenal mass. The risk of PPGL is higher in children with pathogenic variants in tumour susceptibility genes and/or a previous history of PPGL compared with those without.

• #66 International consensus statement on the diagnosis and management of phaeochromocytoma and paraganglioma in children and adolescents | Nature Reviews Endocrinology

  https://www.nature.com/articles/s41574-024-01024-5

  Biochemical testing for patients with suspicion of PPGL should include plasma-free or urine (spot or 24-h) levels of normetanephrine and metanephrine, and should be performed using liquid chromatography assay. […] Blood sampling for measurements of plasma levels of metanephrines and methoxytyramine should be carried out with appropriate consideration of pre-analytical conditions. […] In situations of acute physical illness or intense emotional stress, biochemical test results should be interpreted with caution. […] For plasma or urine normetanephrine and metanephrine testing, laboratories should employ appropriate reference intervals. […] In case of borderline elevations (increase less than twofold above the upper cut-off value) of only one catecholamine metabolite in a paediatric patient with a low degree of suspicion of PPGL, biochemical testing should be repeated.

• #67 Image-Guided Precision Medicine in the Diagnosis and Treatment of Pheochromocytomas and Paragangliomas

  https://www.mdpi.com/2072-6694/15/18/4666

  Pheochromocytomas and paragangliomas are rare neural-crest-derived tumors with variable prognosis. In an era of tailored treatment strategies, medical imaging plays a crucial role in the surgeries, image-guided procedures, chemotherapies, immunotherapies, and radionuclide therapies involved in the diagnosis and treatment of these tumors. Medical imaging helps to confirm the diagnosis, guide surgical resection, assess metastatic staging, and select patients for specific therapies. […] Despite major medical improvements, PPGLs, as with other neuroendocrine tumors (NETs), leave clinicians facing several challenges; their inherent particularities and their diagnosis and treatment pose several challenges for clinicians due to their inherent complexity, and they require management by multidisciplinary teams. The conventional concepts of medical imaging are currently undergoing a paradigm shift, thanks to developments in radiomic and metabolic imaging.

• #68 Image-Guided Precision Medicine in the Diagnosis and Treatment of Pheochromocytomas and Paragangliomas

  https://www.mdpi.com/2072-6694/15/18/4666

  Use of AI in PPGLs may detect changes in tumor phenotype that precede classical medical imaging biomarkers, such as shape, texture, and size. […] Therefore, screening for catecholamine secretion is critical before any biopsy in case of suspected PPGL, to prevent life-threatening outcomes. […] Genetic testing is an essential component of not only patient management, follow-up, and prognostication, but also for election of an optimal imaging modality. […] The majority of histological criteria are applied to postoperative histological samples. However, preoperative imaging sheds light on poor prognostic factors. […] Accurate preoperative staging, performed with both anatomical and molecular imaging, is crucial to identify anatomical risks, assess extent of disease, and discuss surgical options.

• #69 A Case Report of Paraganglioma: A Diagnosis Not to Be Missed

  https://brieflands.com/articles/ijcm-99817

  Furthermore, a biopsy can lead to severe hemorrhage or fibrosis with subsequent difficulty with definitive surgery. […] Locoregional PGLs can be managed by surgical resection or radiation therapy. […] Generally, the preferred approach for localized PGLs below the neck and all catecholamine-secreting PGLs at any site is resection. […] The diversity of manifestations and rarity of PGL can delay the diagnosis, which may lead to potential complications.

• #70 The Diagnosis and Clinical Significance of Paragangliomas in Unusual Locations

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6162705/

  Awareness of the existence of PGLs in unusual locations and the likelihood of multifocal primary tumors in patients with germline genetic predisposition to this disease will reduce errors in diagnosis and provide more accurate data collection to allow progress in the understanding of these fascinating tumors.

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