Table 1: Summary of characteristic presentations, treatments, and outcomes of pregnant women with phaeochromocytoma from the 10 most recent case reports.

 

Age (years)

Gestation at diagnosis (weeks)

Presentation

Diagnosis

Tumour location

Management

Outcome

Reference

27

2 days postnatal after Caesarean section at term.

Headache, confusion, nausea and vomiting, shortness of breath, tachypnoea.

Severe hypertension unresponsive to medical treatments.

Urine epinephrine.

Urine norepinephrine. Urine metanephrine.

Urine normetanephrine.

Urine VMA.

Abdominal CT.

Left adrenal mass measuring 58 mm × 50 mm × 30 mm.

α-adrenergic antagonist.

β-antagonist. Surgical adrenalectomy.

Both mother and baby are well.

Naghshineh E, et al. [52]

24

37

Severe hypertension.

Urine metadrenaline. MIBG Iodine scan (postnatally).

 

Bilateral phaechromocytoma on the adrenal glands.

α-adrenergic antagonist.

β-antagonist.

Laparoscopic excision of tumours at 5 months postnatal.

Patient delivered by Caesarean section.

Both mother and baby are well.

Weingarten M, et al. [53]

32

12

Right sided abdominal discomfort.

Urine metanephrine.

Urine normetanephrine.

Abdominal ultrasound. MRI.

Bilateral phaechromocytoma on the adrenal glands. (right side, 49 × 44 × 42 mm; left side, 73 × 61 × 75 mm).

α-adrenergic antagonist. Hydration.

Exploratory laparotomy and bilateral adrenalectomy at 15 weeks gestation.

Pregnancy continued and uneventfully. Patient had normal vaginal delivery at 39 weeks gestation.

Both mother and baby are well.

Kitayama K, et al. [54]

34

9

Palpitation, headache, sweating, nonspecific gastrointestinal disorders.

Pressure on right renal area.

Paroxysmal hypertension.

Urine catecholamine

Urine VMA.

Abdominal ultrasound. MRI.

Right adrenal tumour 10 cm in diameter.

α-adrenergic antagonist.

β-antagonist.

Surgical resection of tumour at 14 weeks gestation.

Baby was delivered uneventfully at the 36 weeks gestation. Both mother and baby are well.

Kiroplastis K, et al. [55]

24

33

Acute diffuse abdominal pain.

Severe hypertensive crisis.

Acute pulmonary oedema.

Urine noradrenaline.

Plasma renin

Serum noradrenaline Abdominal CT.

MRI

Left adrenal mass measuring 56 x 49 mm.

α-adrenergic antagonist.

β-antagonist.

Calcium channel blockers.

ACE1 inhibitor. Open left adrenalectomy 2 weeks later.

Had Caesarean section for fetal distress, baby died 2 weeks later. Mother is well.

Santos, et al. [1]

34

13

Severe hypertension unresponsive to medical treatments.

Urine VMA.

MRI.

Right adrenal mass measuring 30 x 25 mm

α-adrenergic antagonist.

β-antagonist. Open right adrenalectomy. Blood pressure was controlled with glyceryl-trinitrate and volatile agents intraoperatively.

She had uneventful pregnancy and delivered vaginally at term. Both mother and baby are well.

Memon, et al. [56]

23

 

26

Sweating, abdominal pain, palpitations.

Severe hypertension unresponsive to medical treatments.

Abdominal CT

Right adrenal mass

β-antagonist. Hydralazine. Open adrenalectomy.

The pregnancy was terminated, baby died 2 days postnatal. The mother had good post-operative outcome.

Lalitha R, et al. [57]

27

22

Intermittent headache and epigastric pain. Severe hypertension unresponsive to medical treatments.

Urine norepinephrine.

Plasma norepinephrine. Urine metanephrine.

MRI.

Right adrenal mass measuring 40 x 40 mm.

α-adrenergic antagonist. Laparoscopic adrenalectomy at 25 weeks gestation.

Both mother and baby are well.

Doo AR, et al. [58]

43

 

Term

Patient had a background medical history of gestational diabetes mellitus.

Sudden malignant hypertension with haemoptysis, sweating, and tachycardia during a planned Caesarean section.

Baby was successfully resuscitated. The mother died after resistant cardiac arrest.

Autopsy and pathological analyses showed an acute pulmonary oedema and a necrotic left adrenal gland tumour, which was a pheochromocytoma

Left adrenal tumour

-

Mother died

Baby survived.

Plu I, et al. [59]

34

Term

Hypertensive crises and a grand-mal seizure following elective caesarean section.

Treatment for presumed eclamptic seizure was initiated followed by profound hypotensive episodes accompanied by severe biventricular failure and fluctuating systemic vascular resistance.

Abdominal ultrasound. Abdominal CT.

Urinary assays.

Left suprarenal mass measuring 53mm.

α-adrenergic antagonist.

β-antagonist. Surgical excision 6 weeks postnatal.

Both mother and baby are well. The cardiac function returned to normal and she has made a complete recovery.

Petrie J, et al. [60]

 

Abbreviations

VMA: Vanillylmandelic Acid; CT: Computed Tomography; MIBG: Metaiodobenzylguanidine; MRI: Magnetic Resonance Imaging