Benign disease
The risk of recurrent disease after complete resection of benign pheochromocytoma may be as low as 5 events for 100 patients followed up for 5 years.[89]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.ncbi.nlm.nih.gov/pubmed/27080352?tool=bestpractice.com
Recurrences may be benign or malignant. Hereditary tumors are more likely to recur.
Up to 20% of patients who have surgical resection for benign disease retain some degree of hypertension, and follow-up for long-term blood pressure management is necessary.[90]Fung MM, Viveros OH, O'Connor DT. Diseases of the adrenal medulla. Acta Physiol (Oxf). 2008 Feb;192(2):325-35.
http://www.ncbi.nlm.nih.gov/pubmed/18021328?tool=bestpractice.com
Metastatic disease
There are no curative treatments for metastatic disease, which has a 5-year survival of 42%.[91]Hamidi O, Young WF Jr, Gruber L, et al. Outcomes of patients with metastatic phaeochromocytoma and paraganglioma: a systematic review and meta-analysis. Clin Endocrinol (Oxf). 2017 Nov;87(5):440-50.
http://www.ncbi.nlm.nih.gov/pubmed/28746746?tool=bestpractice.com
Metastatic disease is, however, unpredictable, with reports of patients surviving over 20 years after diagnosis.[92]Yoshida S, Hatori M, Noshiro T, et al. Twenty-six-years' survival with multiple bone metastasis of malignant pheochromocytoma. Arch Orthop Trauma Surg. 2001 Nov;121(10):598-600.
http://www.ncbi.nlm.nih.gov/pubmed/11768644?tool=bestpractice.com
Factors that are thought to prolong survival include younger age, female sex, early diagnosis, and complete excision of the primary tumor.[93]Mei L, Khurana A, Al-Juhaishi T, et al. Prognostic factors of malignant pheochromocytoma and paraganglioma: a combined SEER and TCGA databases review. Horm Metab Res. 2019 Jul;51(7):451-7.
http://www.ncbi.nlm.nih.gov/pubmed/30919391?tool=bestpractice.com
Male sex and synchronous metastases are associated with increased mortality risk.[91]Hamidi O, Young WF Jr, Gruber L, et al. Outcomes of patients with metastatic phaeochromocytoma and paraganglioma: a systematic review and meta-analysis. Clin Endocrinol (Oxf). 2017 Nov;87(5):440-50.
http://www.ncbi.nlm.nih.gov/pubmed/28746746?tool=bestpractice.com
Tumor mutational status can be used as prognostic biomarkers.[21]Jhawar S, Arakawa Y, Kumar S, et al. New insights on the genetics of pheochromocytoma and paraganglioma and its clinical implications. Cancers (Basel). 2022 Jan 25;14(3):594.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8833412
http://www.ncbi.nlm.nih.gov/pubmed/35158861?tool=bestpractice.com
Tumor mutations in succinate dehydrogenase (SDH) gene subunits are often multiple, aggressive, metastatic, and have a poorer prognosis.[94]Nölting S, Ullrich M, Pietzsch J, et al. Current management of pheochromocytoma/paraganglioma: a guide for the practicing clinician in the era of precision medicine. Cancers (Basel). 2019 Oct 8;11(10):1505.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6827093
http://www.ncbi.nlm.nih.gov/pubmed/31597347?tool=bestpractice.com