Individuals with an inherited pathogenic variant in the SDHD gene are at risk of developing rare tumours known as PPGLs. Our study synthesized global research to clarify how this cancer risk evolves across a patient’s lifetime. We found that the likelihood of developing a tumour rises significantly with age, reaching 82% by the age of 60. While 75% of affected patients develop multiple tumours, these growths are fortunately rarely malignant. Only 3% of such tumours metastasize, and the associated mortality rate is just 1%. This underscores that although lifelong medical surveillance is essential, patients with SDHD pathogenic variants have a generally favorable prognosis. (https://jmg.bmj.com/content/early/2026/04/15/jmg-2025-111235)