Acromegaly in a patient with a pulmonary neuroendocrine tumor: case report and review of current literature

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Abstract

Background

Pulmonary neuroendocrine tumors (NET) form a heterogeneous group of rare diseases. In these tumors, paraneoplastic syndromes have been described to drive the course of the disease, among them acromegaly induced by paraneoplastic secretion of growth hormone-releasing hormone (GHRH).

Case presentation

We report the case of a 43 years old patient initially diagnosed with acromegaly accompanied by weight gain and acral enlargement. Subsequently, further diagnostic work-up identified a solitary pulmonary neuroendocrine tumor (NET). Laboratory tests revealed markedly increased growth hormone (GH) and insulin-like growth factor 1 (IGF-1) without GHRH elevation in the absence of pituitary pathologies confirming the paraneoplastic origin of clinical presentation with acromegaly. Curative surgery was performed leading to normalization of the elevated hormone levels and improvement of the clinical symptoms. Immunohistochemically, a typical carcinoid (TC) was seen with low proliferation index and abundant IGF-1 expression.

Conclusions

The association of acromegaly and pulmonary NET has only rarely been reported. We present an individual case of paraneoplastic GH- and IGF-1 secretion in a patient with pulmonary NET. Based on their rarity, the knowledge of paraneoplastic syndromes occurring in patients with pulmonary NET such as acromegaly due to paraneoplastic GH- and IGF-1 secretion is mandatory to adequately diagnose and treat these patients.

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Most cited references 34

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Pulmonary neuroendocrine (carcinoid) tumors: European Neuroendocrine Tumor Society expert consensus and recommendations for best practice for typical and atypical pulmonary carcinoids.

M.E. Caplin, E Baudin, P Ferolla … (2015)

Pulmonary carcinoids (PCs) are rare tumors. As there is a paucity of randomized studies, this expert consensus document represents an initiative by the European Neuroendocrine Tumor Society to provide guidance on their management.

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Epidemiology of acromegaly.

I Holdaway, C. Rajasoorya (1999)

Acromegaly is a consequence of chronic growth hormone (GH) excess, due in the majority of cases to a GH-secreting pituitary adenoma, and occurring with a population prevalence of 60 per million and an incidence of 3-4 per million per year. Males and females appear to be equally affected with an average age of presentation of 44 years. Younger patients may have more aggressive tumours and higher GH concentrations. There is co-existent hyperprolactinaemia in about one third of cases, and a variable proportion of [figure: see text] tumours appear to have activating mutations of the gsp gene or other genetic abnormalities. Acute complications such as carpal tunnel syndrome, sweating and obstructive sleep apnoea are usually readily reversible with treatment of the condition, but chronic complications such as hypertension, diabetes and heart disease are less readily corrected and post-treatment GH levels of < 2.5 ug/L (5 mU/L) are needed to achieve the prevalence found in the general community. Such 'curative' levels of GH are achieved in only about 50% of patients with current therapies, and as a result there is an ongoing excess of patients with chronic complications of acromegaly leading to increased morbidity and mortality from the disorder, with observed-to-expected mortality ratios ranging from 1.6-3.3 and only approaching unity in those with growth hormone levels < 2.5 ug/L following treatment. Prognostic factors include in some studies the presence of diabetes and [table: see text] hypertension prior to diagnosis as well as measures of exposure to excessive growth hormone derived from the product of preoperative serum GH and the time from first symptoms to treatment. Overall, however, the most important prognostic variable appears to be the serum GH concentration achieved by treatment, with an increasing consensus that this needs to be < 2.5 ug/L (5 mU/L) to achieve cure of the condition.

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Predictors of morbidity and mortality in acromegaly: an Italian survey.

M. Arosio, G Reimondo, E Malchiodi … (2012)

To describe demographic and hormonal characteristics, comorbidities (diabetes mellitus and hypertension), therapeutic procedures and their effectiveness, as well as predictors of morbidity and mortality in a nationwide survey of Italian acromegalic patients. Retrospective multicenter epidemiological study endorsed by the Italian Society of Endocrinology and performed in 24 tertiary referral Italian centers. The mean follow-up time was 120 months. A total of 1512 patients, 41% male, mean age: 45±13 years, mean GH: 31±37 μg/l, IGF1: 744±318 ng/ml, were included. Diabetes mellitus was reported in 16% of cases and hypertension in 33%. Older age and higher IGF1 levels at diagnosis were significant predictors of diabetes and hypertension. At the last follow-up, 65% of patients had a controlled disease, of whom 55% were off medical therapy. Observed deaths were 61, with a standardized mortality ratio of 1.13 95% (confidence interval (CI): 0.87-1.46). Mortality was significantly higher in the patients with persistently active disease (1.93; 95% CI: 1.34-2.70). Main causes of death were vascular diseases and malignancies with similar prevalence. A multivariate analysis showed that older age, higher GH at the last follow-up, higher IGF1 levels at diagnosis, malignancy, and radiotherapy were independent predictors of mortality. Pretreatment IGF1 levels are important predictors of morbidity and mortality in acromegaly. The full hormonal control of the disease, nowadays reached in the majority of patients with modern management, reduces greatly the disease-related mortality.

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Author and article information

Contributors

Sebastian Krug: sebastian.krug@uk-halle.de

Michael Boch: Bochm@students.uni-marburg.de

Peter Rexin: rexin@med.uni-marburg.de

Andreas Pfestroff: pfestrof@staff.uni-marburg.de

Thomas Gress: gress@med.uni-marburg.de

Patrick Michl: patrick.michl@uk-halle.de

Anja Rinke: sprengea@uni-marburg.de

Journal

Journal ID (nlm-ta): BMC Res Notes

Journal ID (iso-abbrev): BMC Res Notes

Title: BMC Research Notes

Publisher: BioMed Central (London )

ISSN (Electronic): 1756-0500

Publication date (Electronic): 27 June 2016

Publication date PMC-release: 27 June 2016

Publication date Collection: 2016

Volume: 9

Electronic Location Identifier: 326

Affiliations

[ ]Department of Gastroenterology, Endocrinology and Metabolism, Philipps-University Marburg, Marburg, Germany

[ ]Institute of Pathology, Philipps-University Marburg, Marburg, Germany

[ ]Department of Gastroenterology and Hepatology, Martin-Luther University Halle/Wittenberg, Ernst-Grube Straße 40, 06120 Halle (Saale), Germany

[ ]Department of Nuclear Medicine, University Hospital Marburg, Marburg, Germany

Article

Publisher ID: 2132

DOI: 10.1186/s13104-016-2132-1

PMC ID: 4924317

PubMed ID: 27349224

SO-VID: b0baf635-190e-4948-888a-ce1059677739

License:

Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License ( http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

History

Date received : 20 November 2015

Date accepted : 18 June 2016

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ScienceOpen disciplines: Medicine

Keywords: paraneoplastic syndromes,acromegaly,pulmonary neuroendocrine tumor,gh,igf-1

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ScienceOpen disciplines: Medicine

Keywords: paraneoplastic syndromes, acromegaly, pulmonary neuroendocrine tumor, gh, igf-1

Acromegaly in a patient with a pulmonary neuroendocrine tumor: case report and review of current literature

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Abstract

Background

Case presentation

Conclusions

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ScienceOpen Clinical Case Reports Collection

Most cited references 34

Pulmonary neuroendocrine (carcinoid) tumors: European Neuroendocrine Tumor Society expert consensus and recommendations for best practice for typical and atypical pulmonary carcinoids.

Epidemiology of acromegaly.

Predictors of morbidity and mortality in acromegaly: an Italian survey.

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