The diagnosis and management of pheochromocytoma and paraganglioma during pregnancy

Abstract

Diagnosis of pheochromocytoma or paraganglioma (PPGL) in pregnancy has been associated historically with high rates of materno-fetal morbidity and mortality. Recent evidence suggests outcomes are improved by recognition of PPGL before or during pregnancy and appropriate medical management with alpha-blockade. Whether antepartum surgery (before the third trimester) is required remains controversial and open to case-based merits. Women with PPGL in pregnancy are more commonly delivered by Caesarean section, although vaginal delivery appears to be safe in selected cases. At least some PPGLs express the luteinizing hormone/chorionic gonadotropin receptor (LHCGR) which may explain their dramatic manifestation in pregnancy. PPGLs in pregnancy are often associated with heritable syndromes, and genetic counselling and testing should be offered routinely in this setting. Since optimal outcomes are only achieved by early recognition of PPGL in (or ideally before) pregnancy, it is incumbent for clinicians to be aware of this diagnosis in a pregnant woman with hypertension occurring before 20 weeks’ gestation, and acute and/or refractory hypertension particularly if paroxysmal and accompanied by sweating, palpitations and/or headaches. All women with a past history of PPGL and/or heritable PPGL syndrome should be carefully assessed for the presence of residual or recurrent disease before considering pregnancy.

A rare case of pheochromocytoma in a pregnant woman presenting with chest pain: extraordinary management

“…The diagnosis of pheochromocytoma is challenging due to its varied clinical manifestations. In addition to various paroxysmal symptoms, such as hypertension, palpitations, sweating, and headache, excessive catecholamines can trigger cardiac arrhythmias, coronary spasms, myocardial infarction [ 1 , 10 ], hemodynamic collapse, heart failure, pulmonary edema, hypotension, and cardiac arrest [ 4 , 11 – 14 ]. Hypertension is often paroxysmal, as in our reported case; additionally, blood pressure may be unstable [ 2 ].…”

“…The ambiguous presentation of pheochromocytomas is frequently misinterpreted as preeclampsia. Although rare in pregnant women [ 2 – 4 ], pheochromocytomas pose a high risk of maternal and fetal death if not appropriately managed [ 5 ]. This case report describes a pregnant woman with a pheochromocytoma who underwent a cesarean section.…”

Maternal pheochromocytoma and childbirth in Sweden 1973–2015: a population-based study on short and long-term outcome

“…Pc is, in fact, a rare cause of hypertension but should always be taken into consideration as a differential diagnosis in hypertensive pregnant women, especially in absence of proteinuria and/or onset before the 20th gestational week [ 22 , 23 ]. A number of described cases suggest that it is safe to resect Pcs either during late first or early second trimester [ 24 ], or at full-term pregnancy, either with concurrent tumor removal and caesarean section or postponed surgery after delivery [ 12 , 25 ]. The rarity of this disease makes a prospective, randomized trial impossible to conduct but repeated reviews of large cohorts have come to similar conclusions without finding any significant difference in mortality when comparing early and late tumor resection [ 6 , 26 ], in line with the findings of the current study.…”

Management of pheochromocytoma during pregnancy. A multidisciplinary challenge: A case reports

“…The diagnosis should also be suspected in the context of therapy-resistant hypertension, although there are no vasomotor disorders. Other symptoms are not so obvious: ascending constrictive abdomino-thoracic pain, anxiety, tremors, pallor and digestive disorders [ 9 ]. Biology allows the diagnosis to be established by measuring catecholamines (adrenaline, noradrenaline, dopamine) in the blood or urine, and their urinary metabolites (metanephrine, normetanephrine and vanylmandelic acid (VMA), which remain unchanged by pregnancy [ 10 , 11 ].…”

“…Ultrasound, which has a sensitivity of 89–97 % for adrenal locations outside of pregnancy, does not allow exploration of the mediastinum and is technically difficult in late pregnancy [ [7] , [8] , [9] , [10] ]. Computed-tomography is feasible, but magnetic resonance imaging is the reference examination during pregnancy, as it is non-irradiating and very sensitive.…”