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2.1
Ectopic Acromegaly

Presentation

The present review summarizes the current knowledge on clinical presentation, diagnosis and prognosis. Tumors secreting GHRH are neuroendocrine tumors, usually well differentiated and mainly from pancreatic or bronchial origin. [semanticscholar.org]

Ectopic pituitary adenoma presenting as midline nasopharyngeal mass. Ir J Med Sci 2010;179:593-5 - Warczy,ska A, Zieli,ski G, Koziarski A, Podgórski JK. Ectopic pituitary adenoma. Case report and review of literature. [cuerpomedico.hdosdemayo.gob.pe]

(See Presentation .) Growth hormone and insulinlike growth factor GH is necessary for normal linear growth. [emedicine.medscape.com]

Raynaud's disease is present in one-third of cases. [ncbi.nlm.nih.gov]

Entire Body System

  • Hodgkin Lymphoma

    Acromegaly caused by secretion of growth hormone by a non-Hodgkin's lymphoma. N Engl J Med. 2000; 342 :1871–6. doi: 10.1056/NEJM200006223422504. [ PubMed ] [ CrossRef ] [ Google Scholar ] Seth J, Ellis A, Al-Sadie R. [ncbi.nlm.nih.gov]

Musculoskeletal

  • Osteophyte

    Vertebral enlargement, widened intervertebral spaces and osteophyte formation also being observed. [ncbi.nlm.nih.gov]

Workup

(See Presentation and Workup .) Robert Wadlow, 19 years of age, with his father (postcard photo prior to 1937). Courtesy of Wikimedia Commons (https://commons.wikimedia.org/wiki/File:Robert_Wadlow_postcard.jpg). [emedicine.medscape.com]

Colonoscopy

  • Polyps

    Most studies suggest that as many as 30% of patients may have a premalignant colon polyp at diagnosis and that as many as 5% may have a colonic malignancy. [21] In studies, polyps were generally multiple and proximal to the splenic flexure, making them [emedicine.medscape.com]

    As colon cancer may be the consequence of colon polyps degeneration, many studies have been done to assess the prevalence of colon polyps in patients with acromegaly. [ncbi.nlm.nih.gov]

Treatment

Effect of different dopaminergic agents in the treatment of acromegaly. J Clin Endocrinol Metab 1997;82:518-23. Abs R, Verhelst J, Maiter D, et al. Cabergoline in the treatment of acromegaly: a study in 64 patients. [medigraphic.com]

Currently, if surgical treatment fails to cure acromegaly, medical treatment with somatostatin analogs is recommended rather than radiotherapy. [ncbi.nlm.nih.gov]

(See Prognosis, Treatment, and Medication .) [emedicine.medscape.com]

It contains information on symptoms, proper testing, how to get a diagnosis, and the treatment options that are available. It also includes Pituitary Network Association's patient resource listings for expert medical care. [pituitary.org]

American Association of clinical Endocrinologists Medical Guidelines for clinical Practice for the Diagnosis and Treatment of Acromegaly Endocrine Practice Vol 10 N° 3 May /june 2004 (214-225). - Etxabe J, Gaztambide S. La Torre P et al. [cuerpomedico.hdosdemayo.gob.pe]

Prognosis

The present review summarizes the current knowledge on clinical presentation, diagnosis and prognosis. Tumors secreting GHRH are neuroendocrine tumors, usually well differentiated and mainly from pancreatic or bronchial origin. [semanticscholar.org]

(See Prognosis, Treatment, and Medication .) [emedicine.medscape.com]

High GH/IGF-I concentrations, arterial hypertension, and cardiomyopathy are factors in a poor prognosis, while the duration of symptoms and other factors (diabetes, lipid disorders and cancer) are less important [ 30 ]. [ncbi.nlm.nih.gov]

Etiology

(See Pathophysiology and Etiology .) GH acts indirectly, by stimulating the formation of IGF hormones (also called somatomedins). [emedicine.medscape.com]

Etiology [ 34, 35 ] Acromegaly of pituitary origin More than 95% of patients with acromegaly have a benign monoclonal pituitary adenoma which develops from the somatotrope cells that normally produce GH in the pituitary. [ncbi.nlm.nih.gov]

Epidemiology

Acromegaly: an epidemiological study. J. Endocrinol Invest 1993. 16: 181 – 189. - Guerra García R. Vásquez C (1979) Mayor prevalencia de acromegalia en los sisbib.unmsm.edu.pe/Bv Revista/Acta Andina/V09. - Krepostman Unger, Abraham. [cuerpomedico.hdosdemayo.gob.pe]

Epidemiology, clinical characteristics, outcome, morbidity and mortality in acromegaly based on the Spanish Acromegaly Registry. European Journal of Endocrinology 2004;151:439-46. Philippe C and Sylvie S. Acromegaly Orphanet. [medigraphic.com]

Systemic complications of acromegaly: epidemiology, pathogenesis, and management. [ncbi.nlm.nih.gov]

Epidemiology Gigantism is extremely rare, with approximately 100 reported cases to date. [emedicine.medscape.com]

Pathophysiology

(See Pathophysiology and Etiology .) GH acts indirectly, by stimulating the formation of IGF hormones (also called somatomedins). [emedicine.medscape.com]

Prevention

Author Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public [emedicine.medscape.com]

Treatment is aimed at correcting (or preventing) tumor compression by excising the disease-causing lesion, and at reducing GH and IGF-I levels to normal values. Transsphenoidal surgery is often the first-line treatment. [ncbi.nlm.nih.gov]

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