Abstract
Pheochromocytoma in pregnancy is a life-threatening condition. Although it is rare, if the diagnosis is missed or mismanaged, it has detrimental effect on both mother and fetus, with mortality up to 58%. Diagnosis of Pheochromocytoma in pregnancy remains a huge challenge faced by clinicians due to its non-specific signs and symptoms and the fact that it mimics other conditions which occur much more commonly in pregnancy. On the other hand, early recognition, prompt diagnosis and appropriate treatment during pregnancy are associated with a significant reduction in maternal and fetal mortality. In this review, we provide a brief overview regarding the clinical features, diagnostic tests, and management of Pheochromocytoma in pregnancy. We highlight that a delay or missed management is associated with adverse fetal and maternal outcome and that a multidisciplinary team approach is essential and each case considered individually to achieving the best outcome in both mother and fetus. This review should provide a guide for clinicians who come across the condition.
Highlights
Pheochromocytoma is a neuroendocrine tumour of the chromaffin cells of the adrenal medulla that secrete catecholamines
The occurrence is sporadic in the majority of cases, up to 25% of cases may be syndrome-associated-von Hippel-Lindau (VHL) syndrome, multiple endocrine neoplasia type 2 (MEN 2), and neurofibromatosis type 1 (NF1) [3]
After adequate α-receptor blockade is obtained, β antagonists may be considered. This is because β blockade on its own is associated with dramatic blood pressure elevations attributed to unopposed α-adrenergic effects by catecholamines, especially in patients with Pheochromocytoma [34]
Summary
Pheochromocytoma is a neuroendocrine tumour of the chromaffin cells of the adrenal medulla that secrete catecholamines. Methyldopa is an indirect-acting α-adrenergic antagonist that is widely used to treat hypertension during pregnancy It is not recommended for the treatment of patients with pheochromocytoma, as it may worsen the symptoms of pheochromocytoma and blood pressure control [32]. In order to manage acute catecholamine excess in emergencies during pregnancy, phentolamine a competitive α1- and α2-antagonist or phenoxybenzamine, a non-selective, irreversible α1- and α2-blocker, may be considered for the treatment of catecholamine-induced severe hypertension because of the quick onset of action [33,34]. After adequate α-receptor blockade is obtained, β antagonists may be considered This is because β blockade on its own is associated with dramatic blood pressure elevations attributed to unopposed α-adrenergic effects by catecholamines, especially in patients with Pheochromocytoma [34].
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