Adrenal Cancer

Overview: Your adrenal glands

The adrenal glands are two small organs located just above your left and right kidney. Each gland measures approximately 5 cm x 3 cm x 1 cm and weighs approximately 4-5 grams. The glands consist of two parts: the inner portion, the adrenal medulla, and an outer portion, the adrenal cortex. Each part of the gland functions independent of the other part.

Your body relies on the adrenal glands to produce a variety hormones. The adrenal medulla produces two hormones, epinephrine and norepinephrine, otherwise known as adrenaline. These hormones are important in regulating many functions in the body including your heart rate and blood pressure. The adrenal cortex produces three major hormones: aldosterone, cortisol, and DHEA. Aldosterone regulates salt and water absorption by the kidneys as well as your blood pressure. Cortisol acts to regulate a variety of bodily functions including glucose metabolism, protein metabolism, bone growth, blood pressure, and immune system activity.

Adrenal tumors

Adrenal tumors. Tumors can arise from both parts of the adrenal gland, the adrenal cortex and the medulla. Most adrenal tumors are benign and generally are asymptomatic. Occasionally, the tumors are active, in that they overproduce certain adrenal hormones resulting in symptoms and usually requiring treatment. Although rare, malignant forms of these tumors may develop.

Benign tumors of the adrenal cortex:

Benign adrenal adenoma. This is a relatively common benign tumor of the adrenal gland. The tumor results from an overgrowth of adrenal gland tissue which can occasionally result in symptoms of excess hormone activity. The type of symptoms that may develop depends on which hormone is being produced in excess. Cushing's syndrome develops if an adrenal tumor produces excess cortisol hormone. The symptoms may include weight gain, high blood pressure, diabetes, muscle atrophy, hirsuitism, menstrual abnormalities, easy bruising, and headaches. If the tumor produces excess aldosterone, Conn's syndrome develops. The primary symptoms of Conn's syndrome include high blood pressure, electrolyte abnormalities, nocturia, polydipsia, weakness, and cramps.

Adrenal oncytoma, adrenal cysts, and myelolipoma. These are all benign tumors/lesions of the adrenal gland that do not produce hormones. These tumors do not spread and do not overproduce hormones. Occasionally, these tumors may grow to a large size which may result in symptoms requiring treatment.

Tumors of the adrenal medulla

Pheochromocytoma is a relatively rare benign tumor of the adrenal gland that arises from the tissue in the adrenal medulla. Very rarely, this tumor may become cancerous. Because this tumor comes from the adrenal medulla, patients with pheochromocytoma experience symptoms related to excess adrenaline production. The excess adrenaline results in high blood pressure that doesn't respond to medication treatment, palpitations, headaches, facial flushing, and dizziness. Occasionally, patients with pheochromocytoma can have life threatening complications from the excess adrenaline such as stroke and heart attack. The tumors most commonly occur in individuals between the ages of 30 and 50 years old. Certain rare genetic conditions, such as von Hippel Lindau syndrome and Multiple Endocrine Neoplasia syndrome, are associated with the development of pheochromocytoma.

Neuroblastoma is a rare tumor of the adrenal gland that arises from the nerves in the adrenal medulla. It occurs in infancy and early childhood. Unfortunately, the tumor is quite aggressive and the patients often have metastasis at the time of diagnosis.

Adrenal Cancer (Adrenal Cortical Carcinoma)

Adrenal cancer is a very rare but aggressive tumor that arises from the adrenal cortex. It occurs in approximately 1 person per 1.7 million people, accounting for 0.02% of all cancer cases and 0.2% of cancer deaths in the United States. It is most commonly diagnosed in people between the age of 40-60 years old, and occurs more commonly in men. There are no known risk factors.

Adrenal cancer can be divided into nonfunctioning and functioning tumors. Nonfunctioning tumors do not produce excess hormones and generally become symptomatic when they are large enough to cause symptoms. These may include abdominal pain, abdominal fullness, weight loss, and fever. Functioning tumors produce excess amounts of one or more of the adrenal hormones. In addition to the symptoms listed for nonfunctioning tumors, functioning tumors can cause symptoms related to the excess hormone(s). Cortisol secreting tumors can result in Cushing's syndrome and aldosterone secreting tumors can result in Conn's syndrome. In addition, these tumors may over produce the sex hormones, testosterone and estrogen. In men, these tumors can cause gynecomastia, testicular atrophy, decreased libido, and erectile dysfunction. In women, these tumors can cause symptoms related to virilization, such as hirsuitism, acne, menstrual abnormalities, and deepening of the voice.