Pheochromocytoma, diagnosis and treatment: Review of the literature

Abstract

Objective. We conducted an extensive review of the literature and tried to cite the most recent recommendations concerning the pheochromocytoma (PHEO).Methods. Pub Med and Google Scholar databases were searched systematically for studies concerning pheochromocytomas (intra-adrenal paragangliomas) from 1980 until 2016. Bibliographies were searched to fi nd additional articles.Results. More than four times elevation of plasma fractionated metanephrines or elevated 24-h urinary fractionated metanephrines are keys to diagnosing pheochromocytoma. If the results are equivocal then we perform the clonidine test. If we have not done it already, we preferably do a CT scan and/or an MRI scan. Th e patient needs pre-treatment with α1-blockers at least 10-14 days before operation. Alternatives or sometimes adjuncts are Calcium Channels Blockers and/ or β-Blockers. Several familial syndromes are associated with PHEO and genetic testing should be considered.Conclusions. Th e biggest problem for pheochromocytoma is to suspect it in the fi rst place. Elevated metanephrines establish the diagnosis. With the proper preoperative preparation the risks during operation and the postoperative period are minimal. If there is a risk of the hereditable mutation, it is strongly suggested that all the patients with pheochromocytoma need clinical genetic testing.

Do we need an individual approach to atrial fibrillation and adrenergic overload in the critically ill?

“…(21) A hyperadrenergic state is responsible for the reversible myocardial depression seen in both phaeochromocytoma crisis (22) and the stress-related ("broken heart", Takotsubo) cardiomyopathy. (23) Adrenergic overstimulation is associated with a poor prognosis in acute coronary syndromes, heart failure, liver cirrhosis and acute cerebrovascular disease.…”

Pheochromocytoma: A Clinicopathologic Case Series

“…Pheochromocytoma contributes to less than 7% of tumor which originates from the sympathetic nervous system. The incidence ranges from 0.005% to 0.1% in the general population [3]. Other literature states the estimated number of pheochromocytoma incidence is 0.3 cases per million per year or even less [4].…”

“…This number may be underestimated, where 50% of cases are diagnosed at autopsy [2]. This condition is more often found in the age group of 40-50 years [2,3]. Pheochromocytoma is known to affect both sexes equally [2].…”

“…The main signs and symptoms of excess catecholamines include hypertension, palpitations, headaches, sweating, and pallor. Less frequent signs and symptoms include fatigue, nausea, weight loss, constipation, and fever [3].…”

Ghrelin as a potential molecular marker of adrenal carcinogenesis: In vivo and in vitro evidence

“…Aldosterone‐producing tumours (APT), and equally frequent cortisol‐producing tumours (CPT) are found in 2%‐9% of all adrenal tumours, unlike androgen‐producing tumours, which are relatively rare . Tumours arising from the adrenal medulla are termed pheochromocytomas, and approximately 10%‐15% of these tumours are of hereditary origin . Other changes observed in the adrenals are myelolipomas that comprise about 8% of all adrenal tumours and are characterised by the predominance of mature adipocytes.…”