Characteristics that may predict recurrence include bilateral disease, disease occurring at a young age, larger tumors, and hereditary pheochromocytomas.
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 3-12 months after resection, depending on symptoms.[48]National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: neuroendocrine and adrenal tumors [internet publication].
https://www.nccn.org/guidelines/category_1
Surveillance including physical exam and blood pressure is recommended every 6 to 12 months through years 1 to 3 postoperatively. Annual surveillance is recommended for patients with resected pheochromocytoma from years 4 to 10 postoperatively.[48]National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: neuroendocrine and adrenal tumors [internet publication].
https://www.nccn.org/guidelines/category_1
The European Society of Endocrinology recommends assessment of plasma or urinary metanephrines and normetanephrines 2-6 weeks after recovery from surgery.[57]Plouin PF, Amar L, Dekkers OM, et al; Guideline Working Group. European Society of Endocrinology clinical practice guideline for long-term follow-up of patients operated on for a phaeochromocytoma or a paraganglioma. Eur J Endocrinol. 2016 May;174(5):G1-10.
https://academic.oup.com/ejendo/article/174/5/G1/6655102
http://www.ncbi.nlm.nih.gov/pubmed/27048283?tool=bestpractice.com
Patients with elevated metanephrines or normetanephrines postoperatively should undergo an imaging test at 3 months to evaluate for persistent tumor.[57]Plouin PF, Amar L, Dekkers OM, et al; Guideline Working Group. European Society of Endocrinology clinical practice guideline for long-term follow-up of patients operated on for a phaeochromocytoma or a paraganglioma. Eur J Endocrinol. 2016 May;174(5):G1-10.
https://academic.oup.com/ejendo/article/174/5/G1/6655102
http://www.ncbi.nlm.nih.gov/pubmed/27048283?tool=bestpractice.com
Unresectable disease
Patients with unresectable pheochromocytoma or distant metastases should have follow-ups every 3-12 months, depending on symptoms.[48]National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: neuroendocrine and adrenal tumors [internet publication].
https://www.nccn.org/guidelines/category_1
Genetic testing
Indicated for all patients with pheochromocytomas to identify potential hereditary tumor disorders that would necessitate more detailed evaluation and follow-up.[1]Neumann HPH, Young WF Jr, Eng C. Pheochromocytoma and paraganglioma. N Engl J Med. 2019 Aug 8;381(6):552-65.
http://www.ncbi.nlm.nih.gov/pubmed/31390501?tool=bestpractice.com
[3]Martucci VL, Pacak K. Pheochromocytoma and paraganglioma: diagnosis, genetics, management, and treatment. Curr Probl Cancer. 2014 Jan-Feb;38(1):7-41.
https://www.cpcancer.com/article/S0147-0272(14)00002-6/fulltext
http://www.ncbi.nlm.nih.gov/pubmed/24636754?tool=bestpractice.com
[96]Rana HQ, Rainville IR, Vaidya A. Genetic testing in the clinical care of patients with pheochromocytoma and paraganglioma. Curr Opin Endocrinol Diabetes Obes. 2014 Jun;21(3):166-76.
http://www.ncbi.nlm.nih.gov/pubmed/24739310?tool=bestpractice.com
[97]Hampel H, Bennett RL, Buchanan A, et al. A practice guideline from the American College of Medical Genetics and Genomics and the National Society of Genetic Counselors: referral indications for cancer predisposition assessment. Genet Med. 2015 Jan;17(1):70-87.
https://www.nature.com/articles/gim2014147
http://www.ncbi.nlm.nih.gov/pubmed/25394175?tool=bestpractice.com
The tests indicated can be guided by the features of the tumor, the hormones produced, as well as comorbidities and family history.