In the immediate postoperative period catecholamine levels can still remain high. The patient requires assessment of plasma metanephrines and normetanephrines to check for normalization about 2-6 weeks postoperatively.[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.
http://www.eje-online.org/content/174/5/G1.long
http://www.ncbi.nlm.nih.gov/pubmed/27048283?tool=bestpractice.com
If these plasma studies are positive, this necessitates 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.
http://www.eje-online.org/content/174/5/G1.long
http://www.ncbi.nlm.nih.gov/pubmed/27048283?tool=bestpractice.com
At present it is recommended that patients should have yearly follow-ups for at least 10 years to check for local or metastatic recurrences or new tumors.[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.
http://www.eje-online.org/content/174/5/G1.long
http://www.ncbi.nlm.nih.gov/pubmed/27048283?tool=bestpractice.com
As patients with a history of pheochromocytoma have an overall increased risk of death, some experts advocate lifelong follow-up and screening for recurrent tumors in sporadic cases and for associated tumors in hereditary cases.[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.
http://www.eje-online.org/content/174/5/G1.long
http://www.ncbi.nlm.nih.gov/pubmed/27048283?tool=bestpractice.com
[100]Khorram-Manesh A, Ahlman H, Nilsson O, et al. Long-term outcome of a large series of patients surgically treated for pheochromocytoma. J Intern Med. 2005 Jul;258(1):55-66.
http://www.ncbi.nlm.nih.gov/pubmed/15953133?tool=bestpractice.com
Based on more recent data, the risk of recurrent disease after complete resection of a pheochromocytoma may be as low as 5 events for 100 patients followed up for 5 years, and, therefore, lifelong follow-up may not be necessary for some sporadic cases. Yearly monitoring should be tailored to the patient's risk for recurrence.[101]Amar L, Lussey-Lepoutre C, Lenders JW, et al. Management of endocrine disease: Recurrence or new tumors after complete resection of pheochromocytomas and paragangliomas: a systematic review and meta-analysis. Eur J Endocrinol. 2016 Oct;175(4):R135-45.
http://www.eje-online.org/content/175/4/R135.long
http://www.ncbi.nlm.nih.gov/pubmed/27080352?tool=bestpractice.com
Follow-up consists of a minimum of once yearly BP measurement, as well as measurement of plasma metanephrine and normetanephrine. Chromogranin A is a nonspecific marker of the presence of a neuroendocrine tumor and can be used as a screening tool to detect recurrences.[56]Grossrubatscher E, Dalino P, Vignati F, et al. The role of chromogranin A in the management of patients with pheochromocytoma. Clin Endocrinol. 2006 Sep;65(3):287-93.
http://www.ncbi.nlm.nih.gov/pubmed/16918946?tool=bestpractice.com
Imaging tests should be performed every 1 to 2 years in patients with biochemically inactive pheochromocytoma to screen for recurrence or new tumors.[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.
http://www.eje-online.org/content/174/5/G1.long
http://www.ncbi.nlm.nih.gov/pubmed/27048283?tool=bestpractice.com
In the future, follow-up may be tailored to the specific molecular characteristics of a tumor that may act as markers for recurrent disease. There are, however, currently known characteristics that may predict recurrence: namely, bilateral disease, disease occurring at a young age, larger tumors, and hereditary pheochromocytomas.
Up to 40% of pheochromocytomas are hereditary in adults; therefore, genetic testing is 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
[102]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
[103]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 particular tests indicated can be guided by the features of the tumor, the hormones produced, as well as comorbidities and family history.