Prognosis

Acromegaly is associated with serious complications and premature death. Prior to the routine implementation of more effective treatment modalities, the mean mortality estimate for acromegaly was 2- to 3-fold the expected level in the general population.[24] Prognosis has improved due to modern surgical and pharmacological treatment strategies; life expectancy is now close to that of the general population.[24]​ Patients' survival, as well as their quality of life, has been improved by the definition of strict criteria of biochemical control and more aggressive treatment of acromegaly comorbidities such as hypertension, diabetes mellitus, and sleep apnoea.[26] There have been several attempts to develop and validate a tool to measure disease activity in acromegaly patients to support decision-making in clinical practice.[59][60]

Quality of life

Quality of life (QoL) in patients with acromegaly is reduced irrespective of disease state. Strong predictors of poor QoL include diagnostic factors, including diagnostic delay and lack of diagnostic acumen in medical care provision.[61] Biochemical control, or treatment of acromegaly overall, might not be followed by improved QoL in some patients.[62][63]​​​​ A meta-analysis using the goal of improvement in symptom control and health-related QOL (measured by the acromegaly QoL questionnaire) as the primary outcome, determined that acromegaly treatments improve symptoms and health-related QOL.[64]​ It concluded that as symptoms may be only partly caused by growth hormone (GH) overproduction or may be only partly reversible, ongoing attention to symptom improvement is required even after biochemical disease control is established.[64]​ It is important that treatment algorithms not only focus on normalising biochemical markers, but also attempt to improve QoL.

Surgical remission

Surgery is effective in the biochemical control of 80% to 90% of enclosed microadenomas (<10 mm diameter) and 50% to 75% of macroadenomas.[13]​ It is potentially a one-time treatment that can result in GH normalisation in the immediate post-surgical period. Insulin-like growth factor 1 (IGF-1) levels decline more slowly with normalisation in successful cases, taking 2 to 3 months.

Somatostatin analogue (SSA) responders

In cases of tumour persistence or recurrence after surgery or as first-line treatment, first-generation SSAs are effective in disease control in about 40% of patients, with a GH/IGF-1 decrease in 3 to 6 months.​​[31]​​ Tachyphylaxis has not been seen with long-term SSA treatment. Discontinuation of first-generation SSA treatment leads to loss of biochemical control of the disease and sometimes tumour growth rebound. Cost for this lifelong treatment is significant.[31]​​[65]

SSA resistance or intolerance

Patients are considered resistant to first-generation SSA when acromegaly is not biochemically controlled after 6 months of treatment despite maximum recommended doses.

There are now several factors described to predict resistance to SSA, mainly sparsely granulated tumours and T2 characteristics on MRI.[66]

In cases of SSA resistance or intolerance, pegvisomant treatment should be considered, if available. While this treatment is effective in normalising IGF-1 levels in over 70%, it has no effect on GH secretion and does not arrest tumour growth.​​[24]​​

Pasireotide has been studied in patients previously controlled with pegvisomant and first-generation SSA, and induced a pegvisomant sparing effect in 66% of patients; however, hyperglycaemia and diabetes mellitus increased from 32.8% at baseline to 68.9% at 24 weeks.[67]

Aggressive tumours

Though rare, aggressive GH-secreting tumours represent a clinical conundrum and usually require multimodal therapy (surgery, medication, and radiation).[68]

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