June 09, 2014
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Endocrine Society publishes practice guidelines to detect rare adrenal tumors

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The Endocrine Society published clinical practice guidelines to aid in earlier detection of pheochromocytomas and paragangliomas, two kinds of rare adrenal tumors that increase cardiovascular symptoms and risk for cardiovascular disease and death.

Experts at the Endocrine Society said blood and urine tests for metanephrines can help identify patients with these adrenal tumors.

Metanephrine is a byproduct of epinephrine and norepinephrine metabolism, according to a press release from the Endocrine Society. Research has shown that testing for metanephrine levels in the blood and urine is the most effective method to identify current pheochromocytoma and paraganglioma (PPGL) status, the release said.

The experts recommended that initial biochemical testing for PPGLs should include measurements of plasma-free or urinary-fractionated metanephrines.

The recently released clinical practice guidelines also included recommendations for patient care during the treatment of adrenal tumors. The guidelines advised that shared decision-making should be used in determining whether a patient should undergo genetic testing for pheochromocytomas and/or paragangliomas, and genetic testing should be a consideration in all PPGLs.

The society also recommended that patients with paragangliomas and/or metastatic tumors should be tested for gene mutations that accompany those conditions. Patients with paraganglioma should be tested for SDHx mutations; patients with metastatic tumors should be tested for SDHB mutations.

Use of MRI was indicated in patients who have current metastatic tumors to detect head and neck paragangliomas and for patients who require limited radiation exposure. The guidelines also note that an alternative imagine process, (123)I-metaiodobenzylguanidine scintigraphy, can be used for patients with metastatic tumors.

Adverse effects of PPGL tumors are also commonly seen in CVD, including high blood pressure, severe headaches, sweating, racing heart, trembling and general anxiety. The Endocrine Society cited NIH data showing that between 0.1% and 1% of patients treated for high BP have pheochromocytomas.

For more information and additional practice guidelines, visit www.endocrine.org.