Definition
Acromegaly (enlargement (megaly) of the extremities (acral)) is a slowly progressive disease resulting from the increased release of growth hormone (GH) and, consequently, insulin-like growth factor I (IGF-I). Acromegaly occurs when GH excess is present in individuals after epiphyseal closure. Prolonged exposure to hormone excess leads to progressive somatic disfigurement (mainly involving the face and extremities) and a wide range of systemic manifestations that are associated with increased mortality (Colao et al. 2019).
Clinical Features
Incidence
The global annual incidence is up to 13 cases per 100,000 individuals (Colao et al. 2019).
Age
It is most often diagnosed in middle-aged adults. A median age at diagnosis is 40–50 years (Colao et al. 2019).
Sex
Men and women are equally affected (Katznelson et al. 2014).
Site
More than 95% of patients with acromegaly have a sporadic GH-secreting pituitary neuroendocrine tumor that arises from either a somatotroph cell (a cell in...
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References and Further Reading
Colao, A., et al. (2004). Systemic complications of acromegaly: Epidemiology, pathogenesis, and management. Endocrine Reviews, 25, 102–152.
Colao, A., et al. (2019). Acromegaly. Nature Reviews Disease Primers, 5(1), 20.
Gadelha, M. R., et al. (2017). The genetic background of acromegaly. Pituitary, 20, 10–21.
Katznelson, L., et al. (2014). Acromegaly: An endocrine society clinical practice guideline. The Journal of Clinical Endocrinology and Metabolism, 99, 3933–3951.
Lopes, M. B. S. (2010). Growth hormone-secreting adenomas: Pathology and cell biology. Neurosurgical Focus, 29(4), E2.
Marques, P., & Korbonits, M. (2019). Pseudoacromegaly. Frontiers in Neuroendocrinology, 52, 113–143.
Melmed, S., et al. (2018). A consensus statement on acromegaly therapeutic outcomes. Nature Reviews Endocrinology, 14, 552–561.
Mete, O., & Lopes, M. B. (2017). Overview of the 2017 WHO classification of pituitary tumors. Endocrine Pathology, 28(3), 228–243.
Pivonello, R., et al. (2017). Complications of acromegaly: Cardiovascular, respiratory and metabolic comorbidities. Pituitary, 20(1), 46–62.
Rindi, G., et al. (2018). A common classification framework for neuroendocrine neoplasms: An International Agency for Research on Cancer (IARC) and World Health Organization (WHO) expert consensus proposal. Modern Pathology, 31, 1770–1786.
Saeger, W., et al. (2007). Pathohistological classification of pituitary tumors: 10 years of experience with the German pituitary tumor registry. European Journal of Endocrinology, 156, 203–216.
Tortosa, F., & Webb, S. (2017). Novel aspects in histopathology of the pituitary gland. Endocrinol, Diabetes and Nutrition, 64(3), 152–161.
Zahr, R., & Fleseriu, M. (2018). Updates in diagnosis and treatment of acromegaly. European Endocrinology, 14(2), 57–61.
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Colao, A., Pivonello, C., Grasso, L.F.S., Pirchio, R. (2020). Acromegaly. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_5098-1
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DOI: https://doi.org/10.1007/978-3-319-28845-1_5098-1
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