Extra-adrenal functional paraganglioma (phaeochromocytoma) associated with renal-artery stenosis in a pregnant woman

Paraganglioma Presenting as Postpartum Fever of Unknown Origin

“…The occurrence of paragangliomas in pregnancy is extremely rare and has been reported to be about 5 per million pregnancies in some studies to 18.5 per million live births in others [ 6 , 9 , 10 ]. Paragangliomas have been reported to occur at various sites during pregnancy including bladder and heart [ 11 , 12 ].…”

“…Timely diagnosis and treatment are of paramount importance as the maternal and fetal mortality can be as high as 50% if this condition is undiagnosed and untreated [ 6 ]. A hypertensive crisis can be precipitated by uterine contractions during labor and vaginal delivery [ 10 ]. Data indicates that about 20% of patients pass through pregnancy undiagnosed due to the nonspecific nature of signs and symptoms and the much higher prevalence of gestational hypertension [ 14 ].…”

“…After biochemical confirmation, imaging is used to identify the location of the tumour. In pregnant females, the imaging modality of choice is Magnetic Resonance Imaging whose sensitivity can be as high as 90% in diagnosing paragangliomas and it minimizes fetal exposure to ionizing radiation [ 10 , 14 ]. Ultrasound can also be used for detection but it is not the preferred modality as it is not as sensitive as MRI in detecting adrenal tumors [ 13 ].…”

“…Surgical resection is the mainstay of treatment during pregnancy [ 10 ]. Presurgical preparation of patients is done by initiating alpha blocking agents like phenoxybenzamine or doxazosin followed by beta blocking drugs like propranolol or atenolol to avoid unopposed alpha stimulation which can lead to a hypertensive crisis.…”

Pregnancy and the Kidney

“…Fewer than 70 cases of Cushing's syndrome have been reported. It is occasionally unmasked during labor and delivery, when fatal hypertensive crisis is precipitated by vaginal delivery, uterine contractions, and anesthesia (Del Giudice et al, 1998). Low dose dexamethasone suppression tests can therefore give false negatives in pregnancy, but a high dose dexamethasone test that fails to suppress serum cortisol would be helpful in diagnosing an adrenal tumor causing Cushing's syndrome (Anjali et al, 2004).…”