Catecholamine-secreting tumours arise from chromaffin cells of the adrenal medulla (pheochromocytoma) or the autonomic nervous system (extra-adrenal pheochromocytomas, also called extra-adrenal catecholamine-secreting paragangliomas) and are rare with an annual incidence of 2–8 cases per million.1
Of these, only 2% are mediastinal paragangliomas.2
Brown et al3
described a case series of 12 patients with mediastinal paragangliomas; in all patients the tumour was adjacent to the heart or great vessels. Medical pretreatment, often with α- and β-blockade, is recommended to prevent a hypertensive crisis. The majority of cardiac pheochromocytomas are successfully treated with surgical resection. However, in cases where these vascular tumours are significantly invading the myocardium, there can be significant mortality and inability to completely excise the tumour; therefore, it has been suggested that cardiac transplantation could be an alternative option.4
To our knowledge, cardiac pheochromocytoma presenting during pregnancy has not been previously described.
- In the non-pregnant patient, a wide range of imaging modalities is available for tumour localisation after a biochemical diagnosis of pheochromocytoma is made.
- During pregnancy, the imaging options are limited due to safety considerations, and, as in our case, the source of excess catecholamine production may not be found until after the child is born.
- The anatomic extent of the cardiac pheochromocytoma and the extent of involvement of the coronary arteries needs to be assessed, allowing for successful surgical planning and preparation.