Adrenocortical carcinoma

Adrenocortical carcinoma is a rare and violent cancer that originates in the outer layer of the adrenal gland, also known as adrenal cortical carcinoma (ACC). Two adrenal glands are present. On top of each kidney, one sits. The adrenal cortex produces essential hormones, including those that maintain the balance of water and salt, regulate blood pressure, and help the body use energy.

Typically, adrenocortical tumors contain extra levels of one or more adrenal hormones that can cause symptoms. The risk of adrenocortical cancer is increased by some hereditary conditions, including Li-Fraumeni syndrome, Beckwith-Wiedemann syndrome, and the Carney complex.

Before, during, and after treatment, learn about adrenocortical carcinoma and find information on how we help and care for adults with adrenocortical carcinoma.

The uncommon condition is adrenal cortical carcinoma (ACC). It causes cancerous development in the adrenal cortex, the outer layer of the adrenal glands. On top of the kidneys lie the adrenal glands. In the endocrine system, which is the system that generates and controls hormones, they play a significant role. As an adrenocortical carcinoma, ACC is also identified.

Hormones that control metabolism and blood pressure are released by the adrenal cortex. Cortisol and the male hormones called androgens, including testosterone, are also made. ACC may cause excessive development of these hormones to occur.

Types of Adrenal Cortical Carcinomas

There are two types of adrenal cortical carcinomas.

Functioning tumors increase the production of adrenal hormones. With this type of tumor, large amounts of cortisol, testosterone, and aldosterone are usually found in the body. (Aldosterone is a hormone that regulates blood pressure.)

Nonfunctioning tumors do not increase the adrenal glands’ hormonal production. Most tumors on the adrenal glands are not cancerous. Only 5 to 10 percent of adrenal tumors are malignant.

The reasons are unclear for primary ACC. ACC, however, may be a secondary cancer as well. What occurs when another type of cancer spreads to the adrenal glands is secondary cancer.

Risk factors for Adrenal Cortical Carcinoma

Scientists have identified several risk factors for ACC. You may be at higher risk if you:

• are female
• are between the ages of 40 and 50
• have a hereditary disease that affects the adrenal glands
• have another form of cancer that is aggressive

These inherited syndromes include:

• Beckwith-Wiedemann syndrome
• Carney complex
• Li-Fraumeni syndrome
• Lynch syndrome
• Multiple endocrine neoplasia, type 1 (MEN 1)

Children under age 5 are also at higher risk for this condition. Keep in mind, ACC is a rare cancer. Just because you have one or more risk factors does not mean that you will get ACC.

Symptoms of Adrenocortical carcinoma

• Weight gain
• Muscle weakness
• Pink or purple stretch marks on the skin
• Hormone changes in women that might cause excess facial hair, hair loss on the head and irregular periods
• Hormone changes in men that might cause enlarged breast tissue and shrinking testicles
• Nausea
• Vomiting
• Abdominal bloating
• Back pain
• Fever
• Loss of appetite
• Loss of weight without trying

What Are the Symptoms of a Functioning Adrenal Cortical Carcinoma?

The symptoms of a functioning tumor depend on which hormones it is producing.

Testosterone and other androgens:

• increased facial and body hair, particularly in females
• deepened voice in females

Estrogen:

• early signs of puberty in children
• enlarged breast tissue in males

Aldosterone:

• weight gain
• high blood pressure

Cortisol:

• high blood sugar and pressure
• muscle weakness in the legs
• bruising in the body
• excessive weight gain in the chest and abdomen

If they become swollen, both functional and nonfunctioning tumors may cause abdominal pain. Tumors that are not working may not produce hormonal changes or cause specific symptoms.

Cushing’s syndrome is a disease caused by adrenal tumors releasing cortisol. While Cushing’s can be caused by ACC, most of the condition-related tumors are benign. It doesn’t mean you have cancer if you have Cushing’s.

Early-stage cancer is highly curable by specially qualified surgical oncologists, such as endocrine surgeons, who specialize in the treatment of the disease, by surgical resection of the tumor. The problem is that at advanced stages, many, if not most, ACCs are detected.

Although metastatic and persistent ACC are not available for curative care, patients can still benefit from different therapy modalities.

Surgical resection is the main recommended route if the tumor can be completely removed. Full surgical resection over partial resection is preferred where possible (higher reported survival rates support this). Mitotane therapy (a drug therapy) can be used after surgery to potentially improve cure rates and increase survival.

Although this is an attractive concept, it has been somewhat inconsistent with positive clinical literature. Some studies showed improved benefit with therapy, whereas other studies did not. Probably one of the best studies to date was published by Terzolo et al. (2007). In this study, patients who received postoperative mitotane therapy had higher rates of recurrence-free survival compared with those who did not receive mitotane.

In the absence of a resection of the tumor, the patient may develop distant metastases or advanced adrenocortical carcinoma. In patients with unresectable tumors of any stage, mitotane is also used. Vomiting, fatigue, and anorexia are possible gastrointestinal and neurological side effects, which can also be due to a lack of cortisol. Occasionally, the side effects of this medication can restrict its use.

There are several chemotherapies available for the treatment of ACC, such as cisplatin, etoposide, doxorubicin, streptozotocin, and vinca alkaloids. New treatments, such as gemcitabine, taxanes, capecitabine, and bevacizumab, may have been used, but there is no supporting evidence.

Supportive treatment may be necessary in the form of hormone-replacement therapy, particularly for those who have had complete surgical resection of their adrenal gland(s). Since a tumor is surgically extracted, if hormones are not administered to the body, hormone deficiency also occurs.