PL was first diagnosed with phaeochromocytoma at age 23. He sustained a football injury, resulting in a right adrenal hemorrhage leading to a right adrenalectomy. Histopathology revealed the incidental finding of a phaeochromocytoma.
Over the next twenty years, PL experienced multiple recurrences of phaeochromocytomas. These were discovered on surveillance 24-hour urinary catecholamines and imaging studies. In 2000, he had elevated 24-hour urine adrenaline level of 174 nmol (0-80) associated with three Iodine-123 MIBG avid foci around the right kidney and base of the liver. He underwent a right nephrectomy and partial hepatectomy, followed by external beam radiotherapy to the right adrenal bed. Histopathology confirmed multiple foci of phaeochromocytoma.
In 2004 and 2008, he underwent two further surgical resections of the liver and on Morrison’s pouch respectively. These were detected based on recurrent palpitations and follow-up surveillance imaging. 24 urinary catecholamines were negative on both occasions and plasma metanephrines were not performed. PL remained well in between events and was never hypertensive.
In 2017, he represented with palpitations and diarrhoea. 24-hour urinary catecholamines were normal, however a Gallium 68 DOTATATE PET scan identified two focal areas of intense tracer uptake in the right bowel wall. Repeat 24-hour urinary catecholamines became positive five months later with normetanephrine 2.5umol (0.3-2.0), noradrenaline 1.07 umol (0.00-0.70), and dopamine 4.3 umol (0.0-3.5). Three sets of plasma metanephrines were normal.
PL was referred to the oncologists for consideration of peptide receptor radionuclide therapy (PRRT) however it was deemed unsuitable given the small volume disease. He underwent a right hemicolectomy, which confirmed two foci of phaeochromocytoma. Interestingly, PL did not receive alpha blockade pre or intra-operatively. In spite of this, there was no hypertensive crisis. Genetic assessment found no pathological mutation on the phaeochromocytoma and paraganglioma panel. PL recovered well post operatively and his symptoms have completely resolved.