Pheochromocytoma

Screen patients with resistant hypertension; paroxysmal hypertension or hypertensive crisis superimposed on sustained hypertension; episodic headache, sweating, palpitations, pallor, or marked blood-pressure lability; an adrenal incidentaloma; or a first-degree family history of pheochromocytoma or paraganglioma. Orthostatic hypotension and cutaneous neurofibromatosis findings, including café-au-lait macules and neurofibromas, further increase suspicion. jacc+1jacc2017 ACC/AHA/AAPA/ABC/ACPM/AGS/APhA/ASH/ASPC ... - JACCjacc2017 ACC/AHA/AAPA/ABC/ACPM/AGS/APhA/ASH/ASPC/NMA/PCNA Guideline for the Prevention, Detection, Evaluation, and Management of High Blood Pressure in Adults: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines

In adults with cyanotic congenital heart disease, episodic tachycardia or hypertension should trigger screening with plasma fractionated metanephrines and 24-hour urinary fractionated metanephrines because chronic hypoxemia is associated with increased pheochromocytoma/paraganglioma prevalence in this population. jaccjacc2025 ACC/AHA/HRS/ISACHD/SCAI Guideline for the ... - JACC

During hypertensive emergency or an urgent preoperative assessment, proceed promptly with adrenal or paraganglionic imaging when rapid tumor identification is clinically necessary, while obtaining biochemical testing because metanephrine results may not be immediately available. NatureNatureThe Japanese Society of Hypertension Guidelines for the ... - Nature

Use either plasma metanephrines obtained under standardized conditions or 24-hour urinary fractionated metanephrines as the initial biochemical evaluation. For plasma testing, collect with the patient supine and an indwelling intravenous cannula in place; this is the specified standard-condition approach in hypertension guidance. jacc+2jacc2017 ACC/AHA/AAPA/ABC/ACPM/AGS/APhA/ASH/ASPC ... - JACCjacc2025 AHA/ACC/AANP/AAPA/ABC/ACCP/ACPM/AGS/AMA ... - JACCjacc2017 ACC/AHA/AAPA/ABC/ACPM/AGS/APhA/ASH/ASPC/NMA/PCNA Guideline for the Prevention, Detection, Evaluation, and Management of High Blood Pressure in Adults: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines

A positive biochemical result should lead to localization with CT or MRI of the abdomen and pelvis. In patients whose disease distribution requires broader staging or localization, Ga-DOTATATE PET/CT is included as an imaging option in contemporary hypertension guidance. jaccjacc2025 AHA/ACC/AANP/AAPA/ABC/ACCP/ACPM/AGS/AMA ... - JACC

Interpret testing in the clinical context rather than using imaging alone to establish functional disease. Pheochromocytomas and sympathetic paragangliomas can produce catecholamines causing episodic tachycardia and hypertension, whereas some paragangliomas may be detected as masses or through hereditary surveillance. jacc+1jacc2025 ACC/AHA/HRS/ISACHD/SCAI Guideline for the ... - JACCWolters KluwerAbstract : Tropical Gastroenterology - Ovid

Pheochromocytoma may occur as part of RET-associated multiple endocrine neoplasia type 2, von Hippel-Lindau disease, neurofibromatosis type 1, and hereditary paraganglioma syndromes. Multiple head-and-neck paragangliomas, multiple pheochromocytomas, or a family history should prompt hereditary-risk assessment rather than being labeled nonsyndromic. NEJM+1NEJMGerm-Line Mutations in Nonsyndromic PheochromocytomacellFocus on hereditary endocrine neoplasia - Cell Press

Germline VHL mutation can underlie familial pheochromocytoma, as illustrated by affected first-degree relatives and VHL c.298T→C in a reported family. NEJMNEJMPheochromocytoma and Paraganglioma SDHB pathogenic-variant carriers require structured surveillance planning; tumor size and age at first diagnosis have been reported as independent predictors of metastatic behavior and survival in SDHB-related pheochromocytoma/paraganglioma. NatureNatureManagement of phaeochromocytoma and paraganglioma in patients with germline SDHB pathogenic variants: an international expert Consensus statement | Nature Reviews Endocrinology

Metastatic pheochromocytoma/paraganglioma is defined by metastasis to non-chromaffin tissue or local invasion, not by primary-tumor histology alone. This distinction should redirect care toward disease mapping, germline assessment, and multidisciplinary treatment planning. NatureNatureThe Japanese Society of Hypertension Guidelines for the ... - Nature

Refer patients with functional pheochromocytoma or sympathetic paraganglioma for planned resection after preoperative alpha-receptor blockade. Endocrine Society guidance recommends preoperative blockade for all functional tumors to prevent perioperative complications, with a high-sodium diet as part of preparation. Oxford AcademicOxford AcademicPheochromocytoma and Paraganglioma: An Endocrine Society ... A perioperative review similarly states that all pheochromocytoma and sympathetic paraganglioma patients should receive alpha-receptor blockade before surgery. Oxford AcademicOxford AcademicPerioperative Management of Pheochromocytomas and ...

Begin alpha-adrenergic blockade 1 to 2 weeks before surgery when elective resection is planned. WileyWileyPerioperative considerations in patients with adrenal tumors Do not proceed directly to tumor manipulation or nonurgent surgery without catecholamine-directed preparation, because intraoperative hemodynamic instability is the central preventable risk. Oxford Academic+2Oxford AcademicPerioperative Management of Pheochromocytomas and ...Oxford AcademicPheochromocytoma and Paraganglioma: An Endocrine Society ...WileyMalignant Pheochromocytomas and Paragangliomas - FITZGERALD

Metyrosine is an adjunct for selected surgical candidates with troublesome or persistent catecholamine symptoms despite blockade. A cited preoperative regimen starts metyrosine at 250 mg orally every 8 to 12 hours; dosing and duration are individualized to catecholamine burden. Oxford Academic+2Oxford AcademicPreoperative Management of the Pheochromocytoma PatientWileyPheochromocytoma/Paraganglioma: Review of Perioperative ...Oxford Academic54. Pheochromocytoma - Oxford Academic Use it as an adjunct rather than a substitute for alpha blockade. Oxford Academic+1Oxford AcademicPheochromocytoma and Paraganglioma: An Endocrine Society ...Oxford Academic54. Pheochromocytoma - Oxford Academic

Classify pheochromocytoma/paraganglioma as malignant when metastasis is present in non-chromaffin tissue or when there is local invasion. NatureNatureThe Japanese Society of Hypertension Guidelines for the ... - Nature This finding warrants comprehensive anatomic and functional disease mapping, germline testing, and referral to a multidisciplinary endocrine oncology center. The Lancet+2The LancetSupplementary appendix - The Lancetjacc2025 AHA/ACC/AANP/AAPA/ABC/ACCP/ACPM/AGS/AMA ... - JACCNatureManagement of phaeochromocytoma and paraganglioma in patients with germline SDHB pathogenic variants: an international expert Consensus statement | Nature Reviews Endocrinology

Molecular subtypes include pseudohypoxia, kinase-signaling, and Wnt-altered groups. Cluster 1 alterations involving SDHx, VHL, FH, EPAS1, and EglN/PHD2 activate hypoxia-inducible pathways; this biology has therapeutic relevance because HIF2alpha inhibition with belzutifan has been described as recently approved monotherapy for metastatic pheochromocytoma/paraganglioma after phase 2 trials. NatureNatureMetastatic progression of pheochromocytoma and paraganglioma occurs via parallel evolution | npj Precision Oncology

SDHx alterations have been associated with temozolomide response in small retrospective metastatic-disease series. NatureNatureMetastatic progression of pheochromocytoma and paraganglioma occurs via parallel evolution | npj Precision Oncology Treatment selection for metastatic disease should therefore incorporate germline and tumor molecular findings rather than rely on primary location alone. Nature+1NatureMetastatic progression of pheochromocytoma and paraganglioma occurs via parallel evolution | npj Precision OncologyNatureManagement of phaeochromocytoma and paraganglioma in patients with germline SDHB pathogenic variants: an international expert Consensus statement | Nature Reviews Endocrinology