Endocrine Abstracts

Introduction

Paragangliomas (PGL) of the urinary bladder are an extremely rare entity. During pregnancy, PGL can carry higher risk of foetal and maternal mortality, which can be significantly reduced when the diagnosis is made antepartum and adequate multidisciplinary management and surveillance is started. However, despite clinical stability, delivery complications rates are still higher than in the general obstetric population.

Case description

We present a 29-year-old SDHB mutation carrier with a functional urinary bladder PGL diagnosed at the age of ten. She had a past history of hypertension, cardiomyopathy and retinopathy and experienced several recurrences of the disease, requiring two surgical interventions. In 2008, she presented uncontrolled high blood pressure and began treatment with an α-adrenergic receptor antagonist (αAA). Concurrently, vertebral bone lesions were identified and she was submitted to radiotherapy. A year later αAA was withdrawn as she was asymptomatic, with persistently normal metanephrines and stable vertebral bone lesions. Considering the patient’s desire for pregnancy and after explaining the risks inherent to her clinical condition, she was referred to maternity unit for close monitoring. She was followed regularly throughout pregnancy and ambulatory blood pressure (BP) monitoring at 15 weeks revealed slightly high diastolic BP but no medical therapy was required. Repetitively normal levels of free plasma metanephrines, 3-methoxytyramine and chromogranin A were observed. She delivered vaginally at 36 weeks and during labour presented with significantly high BP requiring labetalol bolus. Aminotransaminases were elevated (10 times above reference value) and thrombocytopenia (< 20 000/ul) was detected. She was admitted to the intensive care unit with the diagnosis of HELLP syndrome and progressed satisfactorily afterwards. Her postpartum recovery was uneventful.

Discussion

We present a challenging case of a pregnant woman with known metastatic PGL. Even though she presented a HELLP syndrome, there is no data regarding the relationship between these two entities. PGL is not an absolute contraindication, but it does represent a risk factor for potential complications during pregnancy. It is important for the clinician to respect the maternal wiling, after adequate information of the risks involved. The decision to treat with an αAA requires a fine balance between reducing the risk of hypertensive peaks and the risk of uteroplacental insufficiency.