Monitoring

Characteristics that may predict recurrence include bilateral disease, disease occurring at a young age, larger tumours, and hereditary phaeochromocytomas.

Assessment and monitoring differs for postoperative patients and those with unresectable disease.

Resectable disease

The National Comprehensive Cancer Network (NCCN) recommends measurement of plasma free or 24-hour urine fractionated metanephrines (also known as metadrenalines) 3-12 months after resection, depending on symptoms.[48] Surveillance including physical examination and blood pressure is recommended every 6 to 12 months through years 1 to 3 postoperatively. Annual surveillance is recommended for patients with resected phaeochromocytoma from years 4 to 10 postoperatively.[48]

The European Society of Endocrinology recommends assessment of plasma or urinary metanephrines and normetanephrines (also known as normetadrenaline) 2-6 weeks after recovery from surgery.[55] Patients with elevated metanephrines or normetanephrines postoperatively should undergo an imaging test at 3 months to evaluate for persistent tumour.[55]

Unresectable disease

Patients with unresectable phaeochromocytoma or distant metastases should have follow-ups every 3-12 months, depending on symptoms.[48]

Genetic testing

Indicated for all patients with phaeochromocytomas to identify potential hereditary tumour disorders that would necessitate more detailed evaluation and follow-up.[1][3][94][95]

The tests indicated can be guided by the features of the tumour, the hormones produced, as well as comorbidities and family history.

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