Acromegaly secondary to growth hormone releasing hormone secretion

Agha, Amar; Farrell, Liam; Downey, Paul; Keeling, P. W. N.; Leen, Eamonn; Sreenan, Séamus

doi:10.1007/bf02914554

Cited by 11 publications

(18 citation statements)

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“…5 Similarly, regardless of the cause, serum GH and IGF-1 levels are invariably elevated and GH levels fail to suppress (<1 ng/mL) during OGTT in all forms of acromegaly. 6 No dynamic tests are helpful in differentiating the causes.…”

Section: Discussionmentioning

confidence: 99%

An unusual cause of acromegaly

et al. 2014

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Section: Discussionmentioning

confidence: 99%

An unusual cause of acromegaly

et al. 2014

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Section: Ectopic Ghrh Productionmentioning

confidence: 99%

“…Prevalence, presentation and diagnosis: Ectopic growth hormone releasing hormone producing tumor account for 0.5-1% of all acromegaly patients [109][110]. Many cases of ectopic GHRH were reported over the past 25 years.…”

Section: Ectopic Ghrh Productionmentioning

confidence: 99%

“…The decrease in GHRH level upon tumor resection would further prove ectopic GHRH secretion however in many cases metastasis is present at the time of the diagnosis and surgery would not be a valid option [108][109]. From the registry of the sole laboratory in France which measures plasma GHRH assays, 21 patients with ectopic GHRH secretion presenting with acromegaly were identified between 1983 and 2008.…”

Section: /12mentioning

confidence: 99%

“…GHRH acts through binding to its receptor on somatotroph cells, hence activating adenylate cyclase resulting in an increase in intracellular cyclic AMP. Acromegaly results from an increase in IGF 1 secretion in peripheral tissues resulting from excessive secretion of GHRH [109][110].Prevalence, presentation and diagnosis: Ectopic growth hormone releasing hormone producing tumor account for 0.5-1% of all acromegaly patients [109][110]. Many cases of ectopic GHRH were reported over the past 25 years.…”

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Endocrine Paraneoplastic Syndromes: A Review

Arabi¹

2015

EMIJ

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It is important for the endocrinologist and oncologist to recognize these paraneoplastic endocrine syndromes since this may help in defining the cancer type and hence lead to the appropriate management. In addition, these syndromes are a major cause of morbidity and mortality. Moreover, AbstractParaneoplastic endocrine syndromes result from ectopic production of hormones by different tumors. Hypercalcemia of malignancy is the most common, mostly caused by ectopic parathyroid hormone related peptide (PTHrP) production which increases bone resorption. Other causes include the rare ectopic parathyroid hormone (PTH) production, ectopic production of 1, 25-(OH)2 vitamin D by the tumor and its adjacent macrophages and bone metastasis which by itself in addition to the local production of PTHrP at the level of the bone lead to bone resorption and thus hypercalcemia. Treatment includes extracellular fluid volume repletion, bisphosphonates or denosumab and calcitonin. Ectopic Cushing's syndrome caused by ectopic ACTH production results in hypokalemia, proximal muscle weakness, easy bruisability, hypertension, diabetes and psychiatric abnormalities including depression and mood disorders. Different diagnostic measures help to differentiate Cushing's disease from ectopic Cushing's syndrome. Treatment includes surgical resection of tumor and medical therapy to suppress excess cortisol production. Ectopic secretion of ADH has been associated with different tumor types. The best treatment options include removal of the underlying tumor, chemotherapy, or radiotherapy in addition to free water restriction, demeclocycline and vaptans. Ectopic growth hormone releasing hormone secretion has been identified with carcinoids, phechromocytoma and other tumors. Surgery is the mainstay of therapy with systemic therapy used in metastatic disease. Other rare ectopic hormone syndromes have also been identified including ectopic prolactin, ectopic erythropoietin, and thrombopoetin in addition to others. Keywords 2/12paraneoplastic endocrine syndromes may be the first presenting feature of malignancy [2,3]. This paper provides an overview on the common PNES, their prevalence, diagnosis, prognosis and response to treatment (Table 1). Methodology of Literature SearchEligible studies were identified by a search, from year 1920 up till February 2014, in Medline, PUBMED and EMBASE using a refined search strategy with several terms used including "endocrine paraneoplastic syndromes", "ectopic hormones", "humoral hypercalcemia of malignancy", "ectopic parathyroid hormone related peptide", "ectopic ACTH", "ectopic Cushing syndrome", "ectopic antidiuretic hormone", "syndrome of inappropriate ADH", "ectopic growth hormone releasing hormone", "ectopic growth hormone", "ectopic prolactin", "ectopic thyroid stimulating hormone" in addition to several others. In addition, searches of personal collections and the reference lists of eligible studies were identified with their full text papers being retrieved. A-Hypercalcemia of MalignancyHistorical Perspec...

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Neuroendocrine tumors secreting growth hormone-releasing hormone: Pathophysiological and clinical aspects

et al. 2006

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Hypothalamic GHRH is secreted into the portal system, binds to specific surface receptors of the somatotroph cell and elicits intracellular signals that modulate pituitary GH synthesis and/or secretion. Moreover, GHRH is synthesized and expressed in multiple extrapituitary tissues. Excessive peripheral production of GHRH by a tumor source would therefore be expected to cause somatotroph cell hyperstimulation, increased GH secretion and eventually pituitary acromegaly. Immunoreactive GHRH is present in several tumors, including carcinoid tumors, pancreatic cell tumors, small cell lung cancers, endometrial tumors, adrenal adenomas, and pheochromocytomas which have been reported to secrete GHRH. Acromegaly in these patients, however, is uncommon. The distinction of pituitary vs. extrapituitary acromegaly is extremely important in planning effective management. Regardless of the cause, GH and IGF-1 are invariably elevated and GH levels fail to suppress (<1 microg/l) after an oral glucose load in all forms of acromegaly. Dynamic pituitary tests are not helpful in distinguishing acromegalic patients with pituitary tumors from those harbouring extrapituitary tumors. Plasma GHRH levels are usually elevated in patients with peripheral GHRH-secreting tumors, and are normal or low in patients with pituitary acromegaly. Unique and unexpected clinical features in an acromegalic patient, including respiratory wheezing or dyspnea, facial flushing, peptic ulcers, or renal stones sometimes are helpful in alerting the physician to diagnosing non pituitary endocrine tumors. If no facility to measure plasma GHRH is available, and in the absence of MRI evidence of pituitary adenoma, a CT scan of the thorax and abdominal ultrasound could be performed to exclude with good approximation the possibility of an ectopic GHRH syndrome. Surgical resection of the tumor secreting ectopic GHRH should be the logical approach to a patient with ectopic GHRH syndrome. Standard chemotherapy directed at GHRH-producing carcinoid tumors is generally unsuccessful in controlling the activated GH axis. Somatostatin analogs provide an effective option for medical management of carcinoid patients, especially those with recurrent disease. In fact, long-acting somatostatin analogs may be able to control not only the ectopic hormonal secretion syndrome, but also, in some instances, tumor growth. Therefore, although cytotoxic chemotherapy, pituitary surgery, or irradiation still remain available therapeutic options, long-acting somatostatin analogs are now preferred as a second-line therapy in patients with carcinoid tumors and ectopic GHRH-syndrome.

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Acromegaly secondary to growth hormone releasing hormone secretion

Abstract: Although ectopic GHRH production is very rare, endocrinologists should be aware of this possibility in acromegaly patients if a pituitary tumour was not detected using pituitary imaging.

Cited by 11 publications

References 4 publications

An unusual cause of acromegaly

An unusual cause of acromegaly

Endocrine Paraneoplastic Syndromes: A Review

Neuroendocrine tumors secreting growth hormone-releasing hormone: Pathophysiological and clinical aspects

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