Fast Five Quiz: Adrenal Gland Disorders

Romesh Khardori, MD, PhD

Disclosures

December 11, 2019

Patients with Cushing syndrome may have weight gain. They also frequently notice changes in their skin, including purple stretch marks, easy bruising, and other signs of skin thinning. They may have difficulty climbing stairs, getting out of a low chair, and raising their arms owing to proximal muscle weakness. Menstrual irregularities, amenorrhea, infertility, and decreased libido may occur in women with Cushing syndrome. Men may have decreased libido and impotence. Psychological problems, such as depression, cognitive dysfunction, and emotional lability, may develop. New-onset or worsening of hypertension and diabetes mellitus, difficulty with wound healing, increased infections, osteopenia, and osteoporotic fractures may all occur.

Signs and symptoms specifically associated with endogenous Cushing syndrome include the following:

  • Patients with an adrenocorticotropic hormone (ACTH)-producing pituitary tumor: headaches, polyuria, nocturia, visual problems, or galactorrhea

  • Patients with tumor mass effect on the anterior pituitary: hyposomatotropism, hypothyroidism, hyperprolactinemia or hypoprolactinemia, hypogonadism

  • Patients with an adrenal carcinoma as underlying cause of Cushing syndrome: rapid onset of symptoms of glucocorticoid excess in conjunction with hyperandrogenism presenting as virilization in women or feminization in men

The clinical manifestations of endogenous Cushing syndrome typically occur more gradually than those of exogenous Cushing syndrome. Patients with drug-induced Cushing syndrome may have fewer issues related to low potassium syndrome and relatively less hypertension than patients with endogenous Cushing syndrome, although the incidence of high blood pressure associated with chronic steroid treatment is significant.

Imaging studies for Cushing syndrome should be performed after the biochemical evaluation has been performed. The rationale for this is that random imaging of the pituitary or adrenal glands may yield incidental nonfunctioning pituitary or adrenal adenomas, which may mislead clinicians away from proper therapy and surgery. Ideally, the biochemical abnormalities should reconcile with the anatomical abnormalities before definitive therapy is offered.

The diagnosis of Cushing syndrome requires demonstration of inappropriately high level of cortisol in the serum or urine. The levels should be measured when cortisol, according to its physiologic circadian rhythm, is supposed to be suppressed: that is, during the late evening or when a patient is given exogenous glucocorticoids. This concept gives rise to the following tests, which have been recommended as screening tests for Cushing syndrome:

  • Midnight serum or salivary cortisol

  • 24-hour urine free cortisol

  • Low-dose dexamethasone suppression test

The treatment of choice for endogenous Cushing syndrome is surgical resection of the causative tumor. The primary therapy for Cushing disease is transsphenoidal surgery, and the primary therapy for adrenal tumors is adrenalectomy. Other first-line treatments include surgical resection of ectopic ACTH-secreting tumors; transsphenoidal selective adenomectomy; blocking hormone receptors in bilateral micronodular adrenal hyperplasia; and surgical removal in cases of bilateral adrenal disorders. The choice of second-line treatments include medication, bilateral adrenalectomy, and radiation therapy (for corticotrope tumors).

Read more about Cushing syndrome.

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