Pheochromocytoma Questions & Answers

Updated: Aug 10, 2018
  • Author: Michael A Blake, MBBCh, MRCPI, FRCR; Chief Editor: George T Griffing, MD  more...
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Questions & Answers

Overview

What is a pheochromocytoma?

What are the characteristics of pheochromocytomas spells?

What are the typical progression of pheochromocytomas spells?

What are symptoms associated with pheochromocytomas spells?

What are the clinical signs of pheochromocytomas?

Which tests are used to diagnose pheochromocytomas?

What is the basis for diagnostic test selection for pheochromocytomas?

What is the role of imaging in the diagnosis of pheochromocytomas?

Which studies are used to rule out a familial syndrome in confirmed pheochromocytoma?

What is the treatment of choice for pheochromocytomas?

What is preoperative medical stabilization for pheochromocytoma?

What is a pheochromocytoma?

What is the role of genetics in the etiology of pheochromocytomas?

What are life-threatening manifestations of pheochromocytomas?

Where are pheochromocytomas located most commonly?

What is the likelihood of malignancy for pheochromocytomas?

How are pheochromocytomas diagnosed?

What are the clinical manifestations of pheochromocytomas?

What is the role of catecholamine in the pathogenesis of pheochromocytomas?

What myocardial changes have been revealed on cardiac MRI following surgical removal of pheochromocytoma?

What are the clinical manifestations of hereditary forms of pheochromocytomas?

What are the precipitants of hypertensive crisis in pheochromocytomas?

How is multiple endocrine neoplasia type 2A (MEN 2A) characterized?

What is the genetic etiology of pheochromocytomas?

What is the role of multiple endocrine neoplasia type 2 (MEN 2) in the etiology of pheochromocytomas?

Which mutations are associated with multiple endocrine neoplasia type 2A (MEN 2A) in pheochromocytomas?

How is multiple endocrine neoplasia type 2A (MEN 2A) diagnosed and what is the risk of developing pheochromocytomas?

How is multiple endocrine neoplasia type 2B (MEN 2B) characterized?

Which mutations are associated with multiple endocrine neoplasia type 2B (MEN 2B)?

How is multiple endocrine neoplasia type 2B (MEN 2B) diagnosed and what is the risk for pheochromocytomas?

What are novel mutations that cause hereditary pheochromocytoma?

Which medical conditions are associated with von Hippel-Lindau (VHL) disease?

What is the prevalence of von Hippel-Lindau (VHL) disease in patients with pheochromocytoma?

What is the cause of von Hippel-Lindau (VHL) disease?

What are characteristics of neurofibromatosis (von Recklinghausen disease) and what is the prevalence in patients with pheochromocytomas?

Which neuroectodermal disorders are associated with pheochromocytomas?

Which hormones may be produced by pheochromocytomas?

What is the role of the SDHD gene in the etiology of pheochromocytomas?

Which conditions are caused by mutations in the SDHD gene?

What is the inheritance pattern for succinate dehydrogenase complex?

Which succinate dehydrogenase subunit genes are associated with pheochromocytomas?

What is the role of hemihyperplasia in the etiology of pheochromocytomas?

What is the prevalence of pheochromocytomas?

What are the racial predilections of pheochromocytoma?

How does the prevalence of pheochromocytoma vary by age?

What are the survival rates pheochromocytoma?

Which factors increase the likelihood of malignancy for pheochromocytomas?

What are the possible complications of pheochromocytomas?

What are neurologic complications of pheochromocytoma?

What is the prognosis of pheochromocytoma in pregnant patients?

Presentation

What are the symptoms and signs of pheochromocytoma?

What history findings suggest pheochromocytoma?

How do pheochromocytoma spells vary in frequency and duration?

Which familial syndromes are characterized by pheochromocytomas?

What are the clinical signs of pheochromocytomas?

What is the most common cardiac rhythm abnormality in pheochromocytoma?

What physical findings are characteristic of pheochromocytoma in patients with neurofibromatosis?

DDX

What conditions should be included in the differential diagnoses of pheochromocytoma?

What are the differential diagnoses for Pheochromocytoma?

Workup

What are Endocrine Society, the American Association for Clinical Chemistry, and the European Society of Endocrinology joint diagnostic guidelines for pheochromocytoma?

How are catecholamines measured in the workup of pheochromocytomas?

What are the North American NeuroEndocrine Tumor Society (NANETS) guidelines for biochemical testing in the evaluation of pheochromocytomas?

What is the basis for selecting diagnostic tests for pheochromocytoma?

What are the general lab features of pheochromocytoma?

When are imaging studies indicated for the diagnosis of pheochromocytoma?

Which patients should be screened for pheochromocytoma and what test should be used?

How are plasma metanephrine and catecholamine testing used to diagnose pheochromocytoma?

What is the efficacy of plasma metanephrine and catecholamine testing to screen for pheochromocytomas?

Which factors may affect the accuracy of screening tests for pheochromocytomas?

Which compounds decrease 24-hour urine levels of metanephrines in screening tests for pheochromocytomas?

How is provocative testing used for the diagnosis of pheochromocytomas?

How are suppression tests used for the diagnosis of pheochromocytomas?

How are chromogranin A levels used for the diagnosis of pheochromocytoma?

What is the role of CT scanning in the diagnosis of pheochromocytoma?

What is the role of MRI for the detection of pheochromocytoma?

What is the role of scintigraphy in the diagnosis of pheochromocytoma?

What is the efficacy of scintigraphy for the diagnosis of pheochromocytoma?

What is the role of PET scanning for the diagnosis of pheochromocytoma?

What is the role of FDOPA-PET scanning in the diagnosis of pheochromocytoma?

What is the efficacy of FDOPA-PET scanning for the diagnosis of pheochromocytomas?

What is the role of HED-PET scanning in the diagnosis of pheochromocytoma?

How are genetic tests selected in the evaluation of pheochromocytoma?

Which profiles of plasma catecholamine metabolites guide genetic testing for pheochromocytoma?

How are normetanephrine and metanephrine measures used to differentiate hereditary etiologies of pheochromocytoma?

What does the locations of pheochromocytomas vary among hereditary etiologies?

When is screening for mutations in the ret proto-oncogene indicated in the diagnosis of pheochromocytoma?

What should be the focus of genetic testing for MEN 2A and 2B in the diagnosis of pheochromocytoma?

What is the role of selective venous sampling in the diagnosis of pheochromocytoma?

What is the role of arteriography in the diagnosis of pheochromocytoma?

Which histologic findings are characteristic of pheochromocytomas?

Treatment

What are the Endocrine Society, the American Association for Clinical Chemistry, and the European Society of Endocrinology joint treatment guidelines for pheochromocytoma?

What is the treatment of choice for pheochromocytomas?

Which medications are used in the treatment of pheochromocytomas?

What is the role of beta blockers in the treatment of pheochromocytomas?

Why are plasma free metanephrines monitored following surgical resection of pheochromocytomas?

What is included in the follow-up of surgery for pheochromocytoma?

What are the surgical mortality rates for pheochromocytomas?

What is needed for a laparoscopic adrenalectomy in patients with pheochromocytoma?

What is the role of the anterior midline abdominal approach in the surgical treatment of pheochromocytoma?

When is unilateral subtotal adrenalectomy indicated in the surgical treatment of pheochromocytoma?

How does hypertension affect the surgical treatment of pheochromocytoma?

What are the treatments options for pheochromocytomas during pregnancy?

Guidelines

Which organizations have released treatment guidelines for pheochromocytoma?

Which biochemical tests are recommended for the diagnosis of pheochromocytoma?

What are the North American Neuroendocrine Tumor Society (NANETS) guidelines for biochemical testing for pheochromocytoma?

What are the ES/AACC/ESE guidelines recommendations for the use of imaging to diagnose pheochromocytoma?

What are the NCCN guidelines for evaluation of pheochromocytoma?

What are the NCCN guidelines for evaluation of suspected metastatic pheochromocytoma?

What are the ES/AACC/ESE recommendations for genetic testing in patients with pheochromocytomas?

What are the ES/AACC/ESE recommendations for preoperative care in patients with pheochromocytomas?

When is minimally invasive adrenalectomy indicated for the surgical treatment of pheochromocytoma?

What are the ES/AACC/ESE recommendations for immediate postoperative care following surgery for pheochromocytoma?

What are the NANETS recommendations for the treatment of advanced pheochromocytoma?

Medications

What is the role of medical therapy in the treatment of pheochromocytoma?

What is the role of beta blockers in the treatment of pheochromocytoma?

What is the role of selective alpha1 blocking agents in the treatment of pheochromocytomas?

What is the role of Iobenguane I 131 in the treatment of pheochromocytomas?

Which medications in the drug class Antihypertensives, Other are used in the treatment of Pheochromocytoma?

Which medications in the drug class Beta Blockers, Beta1 Selective are used in the treatment of Pheochromocytoma?

Which medications in the drug class Beta Blockers, Nonselective are used in the treatment of Pheochromocytoma?

Which medications in the drug class Vasodilators are used in the treatment of Pheochromocytoma?

Which medications in the drug class BPH, Alpha Blocker are used in the treatment of Pheochromocytoma?

Which medications in the drug class Alpha Blockers, Antihypertensives are used in the treatment of Pheochromocytoma?

Which medications in the drug class Radiopharmaceuticals are used in the treatment of Pheochromocytoma?