‘Open-and-close’ pituitary surgery in an acromegalic man presenting with excessive sweatiness

Cheung, Karen; Chow, Francis Chun Chung; Lo, Anthony W.I.

doi:10.1136/bcr-2016-215183

Cited by 2 publications

(1 citation statement)

References 5 publications

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“…The most described symptom in cases at preliminary diagnosis was acromegaly, recognized by clinical signs and confirmed with GH level not suppressed after oral glucose tolerance test (OGTT) and/or elevated insulin-like growth factor 1 (IGF-1) concentration. Ectopic source should be suspected when no improvement is observed after pituitary surgery, which was the case in 21 patients ( 17 , 19 , 35 , 40 , 42 , 56 , 57 , 61 , 66 , 73 , 76 – 82 ). Other findings leading to ectopic GHRH secretion diagnosis were acromegalic features without any pituitary lesion in magnetic resonance imaging or conversely, acromegaly in the setting of a previously known non-pituitary tumor, sometimes accompanied by other manifestations mentioned above (20.5% and 39.3% of the patients, respectively).…”

Section: Methodsmentioning

confidence: 99%

Acromegaly Caused by Ectopic Growth Hormone Releasing Hormone Secretion: A Review

et al. 2022

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IntroductionEctopic acromegaly is a rare condition caused most frequently by growth hormone releasing hormone (GHRH) secretion from neuroendocrine tumors. The diagnosis is often difficult to establish as its main symptoms do not differ from those of acromegaly of pituitary origin.ObjectivesTo determine most common clinical features and diagnostic challenges in ectopic acromegaly.Patients and MethodsA search for ectopic acromegaly cases available in literature was performed using PubMed, Cochrane, and MEDline database. In this article, 127 cases of ectopic acromegaly described after GHRH isolation in 1982 are comprehensively reviewed, along with a summary of current state of knowledge on its clinical features, diagnostic methods, and treatment modalities. The most important data were compiled and compared in the tables.ResultsNeuroendocrine tumors were confirmed in 119 out of 121 patients with histopathological evaluation, mostly of lung and pancreatic origin. Clinical manifestation comprise symptoms associated with pituitary hyperplasia, such as headache or visual field disturbances, as well as typical signs of acromegaly. Other endocrinopathies may also be present depending on the tumor type. Definitive diagnosis of ectopic acromegaly requires confirmation of GHRH secretion from a tumor using either histopathological methods or GHRH plasma concentration assessment. Hormonal evaluation was available for 84 patients (66%) and histopathological confirmation for 99 cases (78%). Complete tumor resection was the main treatment method for most patients as it is a treatment of choice due to its highest effectiveness. When not feasible, somatostatin receptor ligands (SRL) therapy is the preferred treatment option. Prognosis is relatively favorable for neuroendocrine GHRH-secreting tumors with high survival rate.ConclusionAlthough ectopic acromegaly remains a rare disease, one should be aware of it as a possible differential diagnosis in patients presenting with additional symptoms or those not responding to classic treatment of acromegaly.

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Section: Methodsmentioning

confidence: 99%

Acromegaly Caused by Ectopic Growth Hormone Releasing Hormone Secretion: A Review

et al. 2022

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Ockham’s Razor for a Retinal Lesion and Acromegaly and Breaking the Vicious Circle

Rak-Makowska

Khoo

Gupta

et al. 2022

Journal of the Endocrine Society

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Acromegaly due to ectopic secretion of growth hormone-releasing hormone (GHRH) is rare. Treatment consist of surgical removal of the primary tumour, cytostatic therapy, ‘cold’ or radioactive somatostatin analogue treatment, and medical therapy for acromegaly, if needed. A 53 year-old female had an ocular lesion noted on a routine optician visit, originally considered to be an ocular melanoma. She had a bronchial carcinoid successfully removed 22 years previously. She had acromegalic features with an enlarged pituitary gland on MRI, but additionally metastatic lesions in her bones, liver and thyroid gland. Elevated GHRH levels (>250x upper-limit-of-normal) suggested a metastatic lung neuroendocrine tumour secreting GHRH. ‘Cold’ and radioactive somatostatin analogue therapy reduced both GHRH and IGF-1 levels, but normalisation of the biochemical markers of acromegaly was only achieved after pegvisomant was introduced. Complete control of IGF-1 was achieved, and this may have hindered the growth of the metastatic lesions as well, as the patient remains well 13 years after the diagnosis of metastatic disease, and 35 years after the original lung operation. A gradual rise in prolactin levels over last 4 years was noted, which is likely due to the prolonged effect of GHRH on prolactin-secreting cells. The diagnosis of this case applied the law of parsimony from the ‘Ockham’s razor’ principle. We consider that breaking the vicious circle of IGF-1 feeding the metastatic tumour was key for the long-term outcome of this case.

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‘Open-and-close’ pituitary surgery in an acromegalic man presenting with excessive sweatiness

Cited by 2 publications

References 5 publications

Acromegaly Caused by Ectopic Growth Hormone Releasing Hormone Secretion: A Review

Acromegaly Caused by Ectopic Growth Hormone Releasing Hormone Secretion: A Review

Ockham’s Razor for a Retinal Lesion and Acromegaly and Breaking the Vicious Circle

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