Clinical Practice Guideline on Rare Adrenal Tumors Released

Clinical Practice Guideline (CPG) for the diagnosis and treatment of pheochromocytomas paragangliomas was released by The Endocrine Society. The CPG appears in the June 2014 issue of the Journal of Clinical Endocrinology and Metabolism.

The guideline highlights the increased risk of cardiovascular disease and possible death if left untreated. Initial testing through blood or urine tests for metanephrines are recommended by the Society. These methods have shown to be more effective at identifying patients who have pheochromocytomas or paragangliomas than other testing methods.

Some of the other recommendations include:

  • People who are diagnosed with pheochromocytomas or paragangliomas should be involved in a shared decision-making process with their physicians to evaluate the need for genetic testing.
  • A diagnostic algorithm that takes into account risk factors such as age at tumor presentation and family history should be used to establish which patients would benefit most from genetic testing and which specific gene mutations to test for.
  • People with paragangliomas and those diagnosed with metastatic tumors should be tested for specific gene mutations associated with those conditions.
  • Computed tomography can be used as the first choice imaging technology for determining the location of pheochromocytomas or paragangliomas for surgical treatment.
  • MRI imaging technology is to be used in specific situations, including for patients who have metastatic tumors, for detecting head and neck paragangliomas and for patients in whom radiation exposure should be limited.
  • 18F-fluorodeoxyglucose-positron emission tomography/computed tomography can be used in patients with metastatic tumors.

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