para>Test visiual fields regularly.
Consider MRI without contrast if new symptoms develop.
If planned pregnancy, consider discontinuing long-acting somatostatin (11)[C].
á
ADDITIONAL THERAPIES
Radiotherapy á
- Used if acromegaly is uncontrolled by surgery or medical therapy
- Conventional, proton beam, and stereotactic radiotherapy have been used.
- Complication: hypopituitarism (50%)
SURGERY/OTHER PROCEDURES
- Transsphenoidal surgical resection by an experienced neurosurgeon is the treatment of choice for patients with pituitary tumors that are resectable.
- If serum IGF-1 is normalized after surgery (within 7 to 10 days after resection), no further therapy is recommended.
ONGOING CARE
FOLLOW-UP RECOMMENDATIONS
Patient Monitoring
- At baseline
- Echocardiogram
- Colonoscopy
- Every 3 to 4 months
- Cardiovascular exam and BP measurement
- Measurement of serum IGF-1
- Annually
- Patients with complications (cardiomyopathy, sleep apnea, colon polyps) should be followed appropriately according to guidelines for these conditions.
PATIENT EDUCATION
Pituitary Patient Resource Guide, published by the Pituitary Network Association (www.acromegaly.org) á
PROGNOSIS
- Patients with GH levels <2.5 after treatment have a mortality similar to the general population (9).
- Death is primarily caused by cancer (15%), respiratory diseases (25%), and cardiovascular diseases (60%).
- If untreated, there is an associated 30% decrease in lifespan related to the above complications.
COMPLICATIONS
See section on "Commonly Associated Conditions"Ł á
REFERENCES
11 Holdaway áIM, Rajasoorya áC. Epidemiology of acromegaly. Pituitary. 1999;2(1):29-41.22 Borson-Chazot áF, Garby áL, Raverot áG, et al. Acromegaly induced by ectopic secretion of GHRH: a review 30 years after GHRH discovery. Ann Endocrinol (Paris). 2012;73(6):497-502.33 Trivellin áG, Daly áAF, Faucz áFR, et al. Gigantism and acromegaly due to Xq26 microduplications and GPR101 mutation. N Engl J Med. 2014;371(25):2363-2374.44 Colao áA, Ferone áD, Marzullo áP, et al. Systemic complications of acromegaly: epidemiology, pathogenesis, and management. Endocr Rev. 2004;25(1):102-152.55 Sanno áN, Teramoto áA, Osamura áRY, et al. Pathology of pituitary tumors. Neurosurg Clin N Am. 2003;14(1):25-39, vi.66 Subbarayan áSK, Fleseriu áM, Gordon áMB, et al. Serum IGF-1 in the diagnosis of acromegaly and the profile of patients with elevated IGF-1 but normal glucose-suppressed growth hormone. Endocr Pract. 2012;18(6):817-825.77 Abu Dabrh áAM, Mohammed áK, Asi áN, et al. Surgical interventions and medical treatments in treatment-na »ve patients with acromegaly: systematic review and meta-analysis. J Clin Endocrinol Metab. 2014;99(11):4003-4014.88 Murray áRD, Melmed áS. A critical analysis of clinically available somatostatin analog formulations for therapy of acromegaly. J Clin Endocrinol Metab. 2008;93(8):2957-2968.99 Sandret áL, Maison áP, Chanson áP. Place of cabergoline in acromegaly: a meta-analysis. J Clin Endocrinol Metab. 2011;96(5):1327-1335.1010 Stone áJC, Clark áJ, Cuneo áR, et al. Estrogen and selective estrogen receptor modulators (SERMs) for the treatment of acromegaly: a meta-analysis of published observational studies. Pituitary. 2014;17(3):284-295.1111 Katznelson áL, Atkinson áJL, Cook áDM, et al. American Association of Clinical Endocrinologists medical guidelines for clinical practice for the diagnosis and treatment of acromegaly-2011 update. Endocr Pract. 2011;17(Suppl 4):1-44.
ADDITIONAL READING
- Dekkers áOM, Biermasz áNR, Pereira áAM, et al. Mortality in acromegaly: a metaanalysis. J Clin Endocrinol Metab. 2008;93(1):61-67.
- Melmed áS. Medical progress: acromegaly. N Engl J Med. 2006;355(24):2558-2573.
- Pita-Gutierrez áF, Pertega-Diaz áS, Pita-Fernandez áS, et al. Place of preoperative treatment of acromegaly with somatostatin analog on surgical outcome: a systematic review and meta-analysis. PLoS One. 2013;8(4):e61523.
- Wolinski áK, Czarnywojtek áA, Ruchala áM. Risk of thyroid nodular disease and thyroid cancer in patients with acromegaly-meta-analysis and systematic review. PLoS One. 2014;9(2):e88787.
SEE ALSO
Algorithm: Tall Stature á
CODES
ICD10
E22.0 Acromegaly and pituitary gigantism á
ICD9
253.0 Acromegaly and gigantism á
SNOMED
- Acromegaly (disorder)
- Myopathy in acromegaly (disorder)
- Neuropathy in acromegaly (disorder)
- Gigantism (disorder)
- Gigantism and acromegaly
CLINICAL PEARLS
- The onset of acromegaly is insidious and may be initially subtle.
- The serum IGF-1 level is the preferred screening test and is elevated in acromegaly.
- An elevated IGF-1 should prompt an MRI of the pituitary.
- First-line treatment of acromegaly is generally transsphenoidal surgery for resectable tumors, followed by long-acting somatostatin analogues, GH receptor antagonists, and/or radiotherapy if surgery alone is not effective at reducing IGF-1 levels to normal.