Prognosis

Without treatment, hypercortisolism persists and in many patients worsens. Untreated disease carries a dismal survival rate of 50% at 5 years.[113] This leads to worsening of the Cushing phenotype and increased mortality, mainly from cardiovascular disease. With therapy to normalise cortisol levels, patients have a mortality rate similar to the general population.[66][67] Within the first year of effective therapy, many of the characteristic features such as facial plethora, striae, and supraclavicular fat pads will resolve or show marked improvement. Patients lose a significant amount of weight with improved control or resolution of diabetes and hypertension. Bone density steadily improves after hypercortisolism resolves. Despite improvement of complications in most patients, cardiovascular risk, hypertension, obesity, and decreased quality of life may persist in some patients even after biochemical cure.[11][114][115]

Patient-reported outcomes are scarce, but they show that decreased quality of life persists even in patients with biochemical normalisation and they are worse in Cushing's disease compared with Cushing syndrome.[116]

Pituitary adenomectomy

Outcomes are largely based on the size of the initial tumour in patients with Cushing's disease. Micro-adenomas (size <1 cm) have superior outcomes with remission rates quoted as 48% to 100% of patients. An average of approximately 32% of Cushing's disease patients, and up to 75% of Cushing's disease patients initially submitted to pituitary surgery, will require a second-line treatment during the disease course. The long-term failure of pituitary surgery is evidently higher in patients with macro-adenomas (range, 0 to 71.4; mean, 48.8; median, 52.2) than in patients with micro-adenomas (range, 0 to 55.5; mean, 25; median, 21.2).[69][73][117] Patients with macro-adenomas (size >1 cm) have remission rates <65% after surgery and show recurrence rates of 12% to 45% at about 16 months.[67][113][118] Patients may require temporary or prolonged treatment for deficiencies of thyrotropin, gonadotrophin, growth hormone, or antidiuretic hormone and corticosteroids.

A 2018 study did not find significant differences between endoscopic and microscopic pituitary surgery in remission percentage. Although the short term recurrence was lower for endoscopic surgery, both groups had a recurrence rate of approximately 4% per person/year.[119] A 2021 study in young people with pituitary tumours found favourable outcomes and lower re-treatment rates with endonasal endoscopic surgery compared with microscopic transsphenoidal surgery.[120]

Adrenocorticotrophic hormone (ACTH)-independent Cushing syndrome

Patients who undergo unilateral adrenalectomy for a causative adrenal adenoma are uniformly cured of their disease. Recurrence in the contralateral adrenal gland is exceedingly rare. Patients with bilateral adrenalectomy will need monitoring and corticosteroid and mineralocorticoid replacement. Outlook for patients with adrenal carcinoma depends upon the stage at which it is diagnosed. Beyond surgical resection, benefit of additional treatments is debated and should be patient-specific.

Ectopic ACTH production

Encompasses a diverse group. The nature of the tumour secreting ACTH largely determines the outcomes. Many ACTH-secreting tumours are aggressive and grow rapidly. In these cases, complete resection of the tumour is difficult, and patients generally succumb to tumour-related complications. Individuals with indolent ACTH-secreting tumours often have complete tumour resection with resolution of hypercortisolism as well as symptoms.

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