Primary Pheochromocytomas

Primary Pheochromocytomas

Primary pheochromocytomas are tumors of the adrenal gland that form catecholamine hormones epinephrine (adrenaline) and norepinephrine (noradrenaline). Primary pheochromocytomas are relatively rare, accounting for only 0.1-0.2% of all cancers. They typically present with signs and symptoms including high blood pressure, headaches, chest pain, sweating, and palpitations.

Pheochromocytomas can have serious complications including sudden heart attacks, stroke and loss of vision, if left untreated. The primary treatment for primary pheochromocytomas is surgical removal. If the tumor is determined to be benign, then the surgery to remove the tumor is usually considered curative.

The diagnosis of pheochromocytoma can be complicated because many of the clinical features are similar to other conditions. Diagnosis is usually made in one of two ways: through laboratory tests which measure levels of catecholamines in the blood and/or urine, or imaging studies which can visualize the tumor and its location.

Risk Factors

The exact cause of primary pheochromocytoma is unknown. However, there are certain risk factors that have been associated with the development of this type of tumor. These include:

  • Family history of the tumor. It is believed to be inherited in some cases.
  • Genetic syndromes such as Multiple Endocrine Neoplasia Type 2 (MEN-2) or Von Hippel-Lindau Syndrome.
  • Exposure to certain chemicals such as asbestos, radiation, and nicotine.
  • Certain medications including chemotherapy drugs for cancer.
  • Obesity.

Prevention

Unfortunately, because the exact cause of primary pheochromocytoma is still unknown, there are no known ways to prevent it. However, it is important to be aware of the risk factors associated with the tumor and to report any symptoms that may suggest the presence of a pheochromocytoma to a healthcare provider.

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