Hirsutismus und Struma multinodosa bei einer 40-jährigen Uhrmacherin

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.

show abstract

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

Zendran

Gut

Kałużny

et al. 2022

Front. Endocrinol.

View full text Add to dashboard Cite

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.

show abstract

scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.

Contact Info

hi@scite.ai

10624 S. Eastern Ave., Ste. A-614

Henderson, NV 89052, USA

Blog Terms and Conditions API Terms Privacy Policy Contact Cookie Preferences Do Not Sell or Share My Personal Information

Made with 💙 for researchers

Part of the Research Solutions Family.

Hirsutismus und Struma multinodosa bei einer 40-jährigen Uhrmacherin

Cited by 3 publications

References 7 publications

The clinical and biochemical spectrum of ectopic acromegaly

The clinical and biochemical spectrum of ectopic acromegaly

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

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

Contact Info

Product

Resources

About