Endocrine Tumors Causing Arterial Hypertension: Pathophysiological Mechanisms and Clinical Implications
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REVIEW ARTICLE
Endocrine Tumors Causing Arterial Hypertension: Pathophysiological Mechanisms and Clinical Implications Agata Buonacera1 • Benedetta Stancanelli1 • Lorenzo Malatino1
Received: 25 February 2017 / Accepted: 3 April 2017 Ó Springer International Publishing Switzerland 2017
Abstract Some tumors are a relatively rare and amendable cause of hypertension, often associated with a higher cardiovascular morbidity and mortality, as compared with that of both general population and patients with essential hypertension. This worse prognosis is not entirely related to blood pressure increase, because the release of substances from the tumor can directly influence blood pressure behavior. Diagnostic approach is challenging and needs a deep knowledge of the different neuro-hormonal and genetic mechanisms determining blood pressure increase. Surgical tumor removal can, but not always, cause blood pressure normalization, depending on how early was tumor detection, since a long-standing history of hypertension is often associated with a much weaker effect on blood pressure. Moreover, target organ damage can be affected by the substances themselves released by the tumors as well as by tumor removal. In this review we consider the phenotype and genetic features of patients with tumor-induced hypertension and focus on their diagnostic work-up. Keywords Secondary hypertension Tumor-induced hypertension Endocrine hypertension Neuroendocrine tumors
& Lorenzo Malatino [email protected] 1
From the Academic Unit of Internal Medicine and Hypertension Centre, Department of Clinical and Experimental Medicine, University of Catania, c/o Cannizzaro Hospital, Via Messina 829, 95126 Catania, Italy
1 Introduction In the work-up of patients with established arterial hypertension (AH), a secondary form should be always ruled out. The removal of causes of secondary hypertension allows in fact a better control or normalization of blood pressure (BP) values and a significant reduction in the cardiovascular risk [1, 2]. Secondary hypertension should be suspected when patients show: (a) grade 3 hypertension (systolic BP C180 mmHg and/or diastolic BP C110 mmHg) or resistant hypertension [1]; (b) sudden new onset hypertension or unexpected worsening; (c) proven prepuberal onset of hypertension; (d) beginning of hypertension within the 3rd decade without either familial history or associated cardiovascular risk factor; (e) malignant or accelerated hypertension; (f) organ damage disproportionate to the standing of AH. Medical history and physical examination are essential to address the adequate diagnostic work-up [1]. Prevalence of secondary hypertension is around 10% [3]. However, although the prevalence of neoplastic causes of AH is not yet well established, in the PAPY study aldosterone-producing adenoma (APA) were found in 4.8% of newly diagnosed hypertensive patients [4]. Diagnostic approach to neoplastic causes of AH should always be guided by several symptoms and signs: orthostatic hypotension associated with severe hypertensi
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