Phaeochromocytoma during pregnancy is extremely rare with a frequency of 0.002% pregnancies. However, the risks for the pregnant patient with this tumour are extremely high: with maternal and foetal mortality up to 50% if undiagnosed. In contrast, early diagnosis and treatment during pregnancy decrease the maternal and foetal mortality to <5 and 15% respectively.
A 37-year-old female underwent abdominal imaging to investigate iron deficiency anaemia. The anaemia settled with supplements, but a 19 mm, 40 Hounsfield Units incidental adrenal mass was found on non-contrast CT abdomen. She was routinely referred for endocrine evaluation at which new symptoms of breathlessness at rest, palpitations, panic attacks, and anxiety were elicited. This highly suggestive history, despite a very small and atypical lesion, prompted her to be started on α-blockade that day, pending confirmatory investigations. Subsequently, 24 h urinary metanephrines were confirmed as 2× ULN, with serum metanephrines 4× ULN, and with increased focal uptake of the right adrenal on meta-iodobenzylguianidine (MIBG) scan. Standard medical blockade was therefore continued and uptitrated with a view to early surgery.
The patient then reported that she was unexpectedly pregnant with her LMP 2 days prior to her MIBG scan. She was therefore counselled carefully, continued on alpha blockade in close collaboration with the medical obstetric, anaesthetic and surgical teams, and surgery deferred. She then underwent an elective uneventful laproscopic right adrenalectomy at 13 weeks gestation. Both mother and foetus remained well and her baby girl was delivered successfully via elective LSCS 6 weeks ago.
This case illustrates that, where the diagnosis has already been made, multidisciplinary management of the pregnant patient with a phaeochromocytoma is relatively straightforward. However, this patient was extremely lucky that a chance scan led her to be referred and diagnosed much earlier than is typical, that her symptoms were appreciated and taken seriously despite rather unimpressive imaging, and that her unplanned pregnancy arose just after rather than just before the MIBG scan.