Endocrine Abstracts

A 22 year old lady, in her third pregnancy, presented at 35 weeks gestation with modest hypertension (BP 130/90). She was known to carry a high risk mutation for Von Hippel Lindau syndrome with no prior disease manifestations. Urinary catecholamine levels were normal at 18 weeks gestation and she remained asymptomatic throughout her pregnancy. Repeat measurement at 35 weeks demonstrated elevated noradrenaline and normetadrenaline values (1761 nmol/24h and 9.69 micromol/24h respectively) and she was commenced on phenoxybenzamine. MRI demonstrated the presence of a 2.5 cm right-sided adrenal mass. In view of her multiparous state and previous uncomplicated labours, we proceeded to non-operative delivery, ensuring optimal analgesic control via early epidural anaesthesia, invasive blood pressure monitoring, and a passive second stage of labour using syntocinon and suction delivery. Labour passed without incident but was followed by a transient rise in arterial pressure which settled without intervention. A laparoscopic right-sided adrenalectomy was performed at 6 weeks post-delivery.

A second lady, aged 32 and in her first pregnancy, presented at 11 weeks gestation with significant hypertension (BP 170/120). Urinary catecholamines demonstrated elevated noradrenaline and normetadrenaline levels (5696 nmol/24h and 5.51 micromol/24h respectively). MRI confirmed the presence of a large paraganglioma in the organ of Zuckerkandl, immediately posterior to the gravid uterus. Samples were sent for analysis of germline mutations in RET, VHL and SDHB/D and she was commenced on phenoxybenzamine and atenolol. In view of the relatively early gestation, the increasing risk of tumour compression with time and a higher potential risk of malignancy in extra-adrenal disease, we proceeded to laparotomy at 22 weeks gestation.

Phaeochromocytoma presenting in pregnancy is rare and these cases illustrate the heterogeneous modes of presentation and complexity of treatment. Optimal delivery and operative strategy requires a multidisciplinary approach involving an endocrinologist, anaesthetist, obstetrician and endocrine surgeon.