Nelson's syndrome

Nelson's syndrome is a rare disorder that occurs in some patients who have undergone surgical removal of both adrenal glands (bilateral adrenalectomy) as a treatment for Cushing's disease. This condition is characterized by the rapid growth of an existing pituitary adenoma and an increase in the production of adrenocorticotropic hormone (ACTH).

Pathophysiology

Nelson's syndrome develops due to the loss of negative feedback from the adrenal glands on the pituitary gland after bilateral adrenalectomy. The absence of adrenal hormones leads to an increase in the secretion of ACTH by the pituitary gland. This can cause the pituitary adenoma to grow and produce excessive amounts of ACTH, which can lead to hyperpigmentation of the skin and other symptoms.

Symptoms

The symptoms of Nelson's syndrome can vary but often include:

• Hyperpigmentation of the skin
• Visual disturbances due to the growth of the pituitary tumor
• Headaches
• Symptoms of pituitary insufficiency due to the mass effect of the tumor

Diagnosis

Diagnosis of Nelson's syndrome typically involves:

• Clinical evaluation of symptoms
• Measurement of plasma ACTH levels
• Imaging studies such as magnetic resonance imaging (MRI) to assess the size and growth of the pituitary tumor

Treatment

Treatment options for Nelson's syndrome may include:

• Radiation therapy to control the growth of the pituitary tumor
• Surgical removal of the pituitary tumor (transsphenoidal surgery)
• Medical therapy to manage hormone levels and symptoms

Prognosis

The prognosis for patients with Nelson's syndrome varies depending on the size and growth rate of the pituitary tumor and the effectiveness of treatment. Regular monitoring and follow-up are essential to manage the condition effectively.

Related Pages

• Cushing's disease
• Pituitary adenoma
• Adrenocorticotropic hormone
• Hyperpigmentation
• Pituitary gland
• Transsphenoidal surgery
• Radiation therapy