ACTH-producing pituitary gland carcinoma

From WikiMD's medical encyclopedia

It is a rare, hormonally functioning or non-functioning pituitary gland adenocarcinoma that produces corticotropin.

Associated diseases

It may be associated with Cushing disease.

CushingsFace.jpg

ACTH-secreting pituitary adenoma or adenocarcinoma is a condition characterized by elevated levels of a hormone called cortisol secreted by a tumor in the pituitary gland.

Classification

It is part of a group of diseases that cause Cushing’s syndrome, characterized by signs and symptoms that may include weight gain around the trunk and in the face, stretch marks, easy bruising, a hump on the upper back, muscle weakness, tiredness, thin bones that are prone to fracture (osteoporosis), mood disorders and memory problems, as well as an increased risk of infections, high blood pressure and diabetes.

Symptoms

Women may have irregular menses and a lot of hair in the body (hirsutism). It occurs when a benign pituitary tumor (adenoma) or pituitary hyperplasia causes the adrenal glands to produce large amounts of cortisol.

Causes

Some cases are caused by somatic mutations in the AIP and the GNAS genes.

Inheritance

Rarely, an ACTH-secreting pituitary adenoma can be inherited, either as an isolated condition or as part of a genetic syndrome (such as multiple endocrine neoplasia type 1 (MEN1) and familial isolated pituitary adenoma), but most cases are sporadic.

Treatment

Treatment generally involves surgery to remove the tumor and medications to decrease cortisol levels

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Contributors: Prab R. Tumpati, MD