Experts recommend blood, urine testing to diagnose rare adrenal tumors
The Endocrine Society today issued a Clinical Practice Guideline (CPG) for the diagnosis and treatment of two types of rare adrenal tumors - pheochromocytomas and paragangliomas - that can raise the risk of cardiovascular disease and even death if left untreated.
The CPG, entitled “Pheochromocytoma and Paraganglioma: An Endocrine Society Clinical Practice Guideline,” appeared in the June 2014 issue of the Journal of Clinical Endocrinology and Metabolism (JCEM), a publication of the Endocrine Society.
Pheochromocytomas are rare, usually noncancerous tumors that form inside the adrenal glands, while paragangliomas are similar tumors that develop outside the glands. The tumors cause the body to produce excess amounts of the hormones epinephrine - commonly known as adrenaline - and norepinephrine, which is involved in regulating blood pressure. Symptoms of these tumors include high blood pressure, episodic severe headaches, excess sweating, racing heart, feelings of anxiety and trembling.
Experts estimate between 0.1 and 1 percent of patients treated for high blood pressure have pheochromocytomas, according to the National Institutes of Health’s National Cancer Institute.
“Correctly diagnosing pheochromocytomas and paragangliomas is extremely important,” said Jacques W.M. Lenders, MD, PhD, FRCP, of Radboud University in Nijmegen, the Netherlands, and chair of the task force that authored the guideline. “In addition to the strain these tumors put on the cardiovascular system, between 10 and 17 percent of the tumors can become malignant. Researchers have discovered that at least a third of people with these conditions have a disease-causing genetic mutation, so early detection can benefit family members who may be at risk.”
In the CPG, the Endocrine Society recommends that initial testing for pheochromocytomas and paragangliomas include blood or urine tests for metanephrines - the products left behind when the body metabolizes epinephrine and norepinephrine. Research has shown blood and urine testing for metanephrines are more effective at identifying patients who have pheochromocytomas or paragangliomas than other testing techniques.
About the adrenal glands
The adrenals are small glands that sit above each of the kidneys. The kidneys are located deep inside the upper part of the abdomen.
The adrenal gland has 2 parts. The outer part, called the cortex, is where most tumors develop. The function of the cortex is to make certain hormones for the body. These hormones all have a similar chemical structure and are called steroids. They include:
Cortisol causes changes in metabolism that help the body to handle stress.
Aldosterone helps the kidneys regulate the amount of salt in the blood and helps regulate blood pressure.
Adrenal androgens are hormones which can be converted to more common forms of the sex hormones estrogen and testosterone in other parts of the body. The amount of these hormones that result from conversion of adrenal androgens is small compared to what is made in other parts of the body. The testicles produce most of the androgens (male hormones) in men. The ovaries produce most of the estrogens (female hormones) in women.
Other recommendations from the CPG 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; and
Another imaging technology, 18F-fluorodeoxyglucose-positron emission tomography/computed tomography, can be used in patients with metastatic tumors.
Other members of the Endocrine Society task force that developed this CPG include: Quan-Yang Duh of the VA Medical Center and University of California, San Francisco; Graeme Eisenhofer of the University Hospital Dresden in Dresden, Germany; Anne-Paule Gimenez-Roqueplo of Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou and Université Paris Descartes in Paris, France; Stefan K.G. Grebe, Mohammad Hassan Murad and William F. Young, Jr. of the Mayo Clinic in Rochester, MN; Mitsuhide Naruse of National Hospital Organisation Kyoto Medical Center in Kyoto, Japan; and Karel Pacak of the Eunice Kennedy Shriver National Institute of Child Health & Human Development in Bethesda, MD.
Types of adrenal gland tumors
A tumor begins when normal cells change and grow uncontrollably, forming a mass. A tumor can be cancerous or benign. A cancerous tumor is malignant, meaning it can spread to other parts of the body. A benign tumor means the tumor will not spread.
A tumor can start in an adrenal gland, called a primary adrenal tumor, or it can begin in another organ, such as the lungs, and then spread to the adrenal glands. An adrenal gland tumor can sometimes overproduce hormones. When it does, the tumor is called a functioning tumor. An adrenal gland tumor that does not produce hormones is called a nonfunctioning tumor. The symptoms and treatment of an adrenal gland tumor depend on whether the tumor is functioning or nonfunctioning, what hormone(s) is overproduced, and whether the tumor is a primary adrenal gland tumor or if the cancer has spread from another organ.
This section focuses on primary adrenal gland tumors, which include the following:
Adenoma. Also called an adrenocortical adenoma, this is the most common type of adrenal gland tumor. It is a noncancerous, nonfunctioning tumor of the adrenal cortex. An adenoma usually does not cause symptoms and, if it is small, often does not need treatment.
Adrenocortical carcinoma. Although rare, adrenocortical carcinoma is the most common type of cancerous adrenal gland tumor. It is also known as adrenal cortical carcinoma. Approximately four to 12 out of one million people develop this type of tumor, which begins in the adrenal cortex. Adrenocortical carcinoma can be a functioning or nonfunctioning tumor. If the tumor is functioning, it may produce more than one hormone.
Neuroblastoma. This is a type of childhood cancer that can begin in the adrenal medulla. Learn more about childhood neuroblastoma.
Pheochromocytoma. This type of neuroendocrine tumor most often begins in the adrenal medulla. Learn more about pheochromocytoma.
The Society established the CPG Program to provide endocrinologists and other clinicians with evidence-based recommendations in the diagnosis and treatment of endocrine-related conditions. Each CPG is created by a task force of topic-related experts in the field. Task forces rely on scientific reviews of the literature in the development of CPG recommendations. The Endocrine Society does not solicit or accept corporate support for its CPGs. All CPGs are supported entirely by Society funds.
The CPG was co-sponsored by the American Association for Clinical Chemistry and the European Society of Endocrinology.
Founded in 1916, the Endocrine Society is the world’s oldest, largest and most active organization devoted to research on hormones and the clinical practice of endocrinology. Today, the Endocrine Society’s membership consists of over 17,000 scientists, physicians, educators, nurses and students in more than 100 countries. Society members represent all basic, applied and clinical interests in endocrinology. The Endocrine Society is based in Washington, DC.
Jenni Glenn Gingery
The Endocrine Society
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