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. |
|
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