Adrenocortical Carcinoma

Adrenocortical carcinoma (ACC) is a rare and aggressive type of cancer that originates in the adrenal cortex, which is the outer layer of the adrenal gland. The adrenal glands are located on top of the kidneys and play a crucial role in producing hormones such as cortisol, aldosterone, and androgens.

Causes and Risk Factors

The exact cause of adrenocortical carcinoma is not well understood, but several genetic and environmental factors have been identified as potential risk factors. These include:

• Li-Fraumeni syndrome: a rare genetic disorder that increases the risk of developing multiple types of cancer, including ACC
• Beckwith-Wiedemann syndrome: a genetic disorder that affects fetal development and increases the risk of developing ACC
• Familial adenomatous polyposis: a genetic disorder that increases the risk of developing colon cancer and other types of cancer, including ACC
• Exposure to carcinogens: such as pesticides and heavy metals

Symptoms

The symptoms of adrenocortical carcinoma can vary depending on the location and size of the tumor, as well as the hormones it produces. Common symptoms include:

• Weight gain: particularly in the face, neck, and trunk
• Hirsutism: excessive hair growth on the face, chest, and back
• Amenorrhea: irregular or absent menstrual periods
• Acne: severe acne that does not respond to treatment
• Buffalo hump: a fatty deposit on the back of the neck
• Purple striae: purple stripes on the skin, particularly on the abdomen and thighs
• Hypertension: high blood pressure
• Hyperglycemia: high blood sugar levels

Diagnosis

The diagnosis of adrenocortical carcinoma typically involves a combination of imaging tests, laboratory tests, and surgical biopsy. Imaging tests may include:

• Computed tomography (CT) scan: to evaluate the size and location of the tumor
• Magnetic resonance imaging (MRI): to evaluate the tumor's relationship to surrounding tissues and organs
• Positron emission tomography (PET) scan: to evaluate the tumor's metabolic activity

Laboratory tests may include:

• Complete blood count (CBC): to evaluate for anemia, leukocytosis, or thrombocytopenia
• Electrolyte panel: to evaluate for electrolyte imbalances
• Hormone levels: to evaluate for hormonal imbalances, such as elevated cortisol or aldosterone levels

Treatment

The treatment of adrenocortical carcinoma typically involves a combination of surgery, chemotherapy, and radiation therapy. Surgical options may include:

• Adrenalectomy: removal of the affected adrenal gland
• Nephrectomy: removal of the affected kidney

Chemotherapy options may include:

• Mitotane: a medication that inhibits cortisol production and has anti-tumor effects
• Streptozocin: a medication that inhibits tumor growth and has anti-tumor effects

Radiation therapy may be used to treat tumors that are not resectable or to palliate symptoms such as pain or bleeding.

Prognosis

The prognosis for adrenocortical carcinoma is generally poor, with a 5-year survival rate of approximately 40%. However, the prognosis can vary depending on the stage and grade of the tumor, as well as the patient's overall health. Early detection and treatment are critical to improving outcomes.