Cancer of the adrenal cortex

Cancer of the Adrenal Cortex

Cancer of the adrenal cortex, also known as adrenocortical carcinoma (ACC), is a rare and aggressive malignancy originating from the cortex of the adrenal gland. The adrenal glands are small, triangular-shaped glands located on top of each kidney. They are responsible for producing a variety of hormones, including cortisol, aldosterone, and androgens.

Epidemiology

Adrenocortical carcinoma is an uncommon cancer, with an estimated incidence of 1 to 2 cases per million people per year. It can occur at any age but has a bimodal distribution, with peaks in childhood and in the fourth to fifth decades of life. There is a slight female predominance.

Pathophysiology

The adrenal cortex is divided into three zones: the zona glomerulosa, zona fasciculata, and zona reticularis, each responsible for producing different hormones. ACC can arise from any of these zones, leading to overproduction of hormones such as cortisol, aldosterone, or androgens, depending on the zone of origin.

Clinical Presentation

Patients with adrenocortical carcinoma may present with symptoms related to hormone excess or due to the mass effect of the tumor. Common symptoms include:

• Cushing's syndrome: Due to excess cortisol production, leading to weight gain, hypertension, diabetes, and skin changes.
• Virilization or feminization: Due to excess androgen or estrogen production, leading to changes in secondary sexual characteristics.
• Abdominal pain or fullness: Due to the tumor mass.

Diagnosis

The diagnosis of adrenocortical carcinoma involves a combination of clinical, biochemical, and imaging studies:

• Biochemical tests: To assess hormone levels and confirm hormone excess.
• Imaging studies: CT or MRI scans to evaluate the size and extent of the tumor.
• Histopathological examination: A biopsy may be performed to confirm the diagnosis.

Treatment

The primary treatment for adrenocortical carcinoma is surgical resection. Complete surgical removal of the tumor offers the best chance for cure. Additional treatments may include:

• Mitotane: An adrenolytic medication used to treat ACC, especially in cases where surgery is not possible or in metastatic disease.
• Radiation therapy: May be used in certain cases to control local disease.
• Chemotherapy: Used in advanced cases, although ACC is often resistant to conventional chemotherapy.

Prognosis

The prognosis for adrenocortical carcinoma is generally poor, with a 5-year survival rate of approximately 20-35%. Prognosis depends on factors such as the stage of the disease at diagnosis, the completeness of surgical resection, and the presence of metastases.

Also see

• Adrenal gland
• Cushing's syndrome
• Endocrine system
• Hormone

Classification
External resources