Prognosis

Benign disease

The risk of recurrent disease after complete resection of benign phaeochromocytoma may be as low as 5 events for 100 patients followed up for 5 years.[87]​ Recurrences may be benign or malignant. Hereditary tumours 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.[88]

Metastatic disease

There are no curative treatments for metastatic disease, which has a 5-year survival of 42%.[89] Metastatic disease is, however, unpredictable, with reports of patients surviving over 20 years after diagnosis.[90] Factors that are thought to prolong survival include younger age, female sex, early diagnosis, and complete excision of the primary tumour.[91]

Male sex and synchronous metastases are associated with increased mortality risk.[89] Tumour mutational status can be used as prognostic biomarkers.[21] Tumour mutations in succinate dehydrogenase (SDH) gene subunits are often multiple, aggressive, metastatic, and have a poorer prognosis.[92]

Use of this content is subject to our disclaimer