Prognosis

Benign disease

Surgery is a curative treatment in more than 85% of cases.[67] There is a 95% 5-year survival with benign disease with recurrence in <10%.[68] Recurrences may be benign or malignant. At least 10 years of follow-up are recommended after removal of a phaeochromocytoma.[93] Hereditary tumours are more likely to recur and, therefore, require long-term follow-up. In benign disease, however, there is still a considerable risk of morbidity and mortality secondary to complications associated with hypercatecholaminaemia. 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.[68]

Metastatic disease

There are no curative treatments for metastatic disease, which has a 5-year survival of 42%.[94] Metastatic disease is, however, unpredictable, with reports of patients with metastatic disease surviving over 20 years after diagnosis.[95] Factors that are thought to prolong survival include younger age, female sex, early diagnosis, and complete excision of the primary tumour.[96] Male sex and synchronous metastases were associated with increased mortality risk.[94] Tumour mutational status can be used as prognostic biomarkers.[20] Tumour mutations in succinate dehydrogenase (SDH) gene subunits are often multiple, aggressive, metastatic, and have a poorer prognosis.[97]

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