Adrenal disease

Recurrent Disease in Patients With Sporadic Pheochromocytoma and Paraganglioma

J Clin Endocrinol MetabPublished Retrospective multicentre cohort study

Authors' conclusion

Patients with sporadic PPGL require long-term follow-up, as supported by the 14.7% prevalence of recurrent disease, including recurrences at more than 10 years after first diagnosis. The nature of follow-up could be individualized according to tumor size, location, and biochemical phenotype.

Study context

Population
Patients with PPGL with genetic test results, multicenter
Participants
1,127 patients
Exposure
Sporadic vs hereditary PPGL
Outcome
Prevalence of recurrence
Methodological detailsmulticenter
  • Multicenter

Main finding

As reported in the abstract

P < 0.001

Observational data: associations, not effects.

Further findings

Timing of recurrence in sporadic recurrent PPGL

Diagnosed ≥10 years after first diagnosis
29.1%
Diagnosed ≥15 years after first diagnosis
17.7%

Independent predictors of recurrence in sporadic PPGL (multivariable regression)

Noradrenergic/dopaminergic phenotype: HR
2.73 (95% CI 1.553-4.802; P < 0.001)
Larger primary tumor size: HR
1.82 (95% CI 1.113-2.962; P = 0.017)
Extra-adrenal location: HR
1.79 (95% CI 1.002-3.187; P = 0.049)

Original publication

Recurrent Disease in Patients With Sporadic Pheochromocytoma and Paraganglioma. J Clin Endocrinol Metab. Published 3 October 2022. doi:10.1210/clinem/dgac563

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