From the Guidelines
Cushing syndrome should be treated with surgery as the first-line treatment, specifically transsphenoidal surgery for pituitary tumors and adrenalectomy for adrenal tumors, as it offers the best chance of cure and improvement in quality of life. The treatment of Cushing syndrome depends on the underlying cause, which can be a tumor in the pituitary gland, adrenal glands, or elsewhere in the body. Symptoms of Cushing syndrome include weight gain, thin skin, fatigue, muscle weakness, high blood pressure, and mood changes.
Diagnosis and Treatment
According to the most recent study 1, selective adenomectomy is recommended as the first-line treatment for Cushing disease. This approach maximizes the potential for normal pituitary tissue to remain in situ, reducing the risk of post-operative hypopituitarism. For adrenal tumors, adrenalectomy is usually required, and postoperative corticosteroid supplementation is necessary until the hypothalamus-pituitary-adrenal axis recovers 1.
Medical Management
Medical management of hypercortisolism can be achieved with adrenostatic agents, such as ketoconazole and mitotane, as stated in the study 1. However, the study 1 suggests that these medications have a limited role in long-term treatment due to their adverse effects and should be used to reduce cortisol burden while awaiting definitive surgery or the effect of pituitary radiotherapy.
Radiotherapy
Radiotherapy can be considered for recurrent Cushing disease not amenable to curative surgery, as recommended in the study 1. Focal external beam radiotherapy is more rapidly effective in children with Cushing disease than in adults and is often initiated 2-4 weeks after unsuccessful transsphenoidal surgery.
Quality of Life and Morbidity
Early diagnosis and treatment of Cushing syndrome are crucial to prevent serious complications like diabetes, bone loss, and cardiovascular problems, which can significantly impact the patient's quality of life and morbidity. Regular monitoring of cortisol levels and symptoms is essential during and after treatment to ensure the best possible outcome.
From the Research
Definition and Incidence of Cushing Syndrome
- Cushing syndrome is defined as a prolonged increase in plasma cortisol levels that is not due to a physiological etiology 2
- The estimated incidence of Cushing syndrome due to endogenous overproduction of cortisol ranges from 2 to 8 per million people annually 2
Clinical Presentation of Cushing Syndrome
- Cushing syndrome is associated with hyperglycemia, protein catabolism, immunosuppression, hypertension, weight gain, neurocognitive changes, and mood disorders 2
- Characteristic skin changes include facial plethora, easy bruising, and purple striae, while metabolic manifestations include hyperglycemia, hypertension, and excess fat deposition in the face, back of the neck, and visceral organs 2
Diagnosis of Cushing Syndrome
- Evaluation of patients with possible Cushing syndrome begins with ruling out exogenous steroid use 2
- Screening for elevated cortisol is performed with a 24-hour urinary free cortisol test or late-night salivary cortisol test or by evaluating whether cortisol is suppressed the morning after an evening dexamethasone dose 2
- Plasma corticotropin levels can help distinguish between adrenal causes of hypercortisolism and corticotropin-dependent forms of hypercortisolism 2
Treatment of Cushing Syndrome
- First-line therapy for Cushing syndrome due to endogenous overproduction of cortisol is surgery to remove the causative tumor 2, 3
- Many patients will require additional treatment with medications, radiation, or bilateral adrenalectomy 2
- Pituitary-directed agents, such as cabergoline and pasireotide, may be used in cases of persistent or recurrent disease after surgery 4
- Combination therapy with cabergoline and ketoconazole may be an effective second-line treatment for Cushing's disease 5