Adrenal disease

Tumor-node-metastasis (TNM) Staging and Overall Survival in Patients With Pheochromocytoma and Sympathetic Paraganglioma

J Clin Endocrinol MetabPublished Retrospective cohort study

Authors' conclusion

OS rates correlated with the recently developed AJCC TNM staging and were not worse in hereditary disease. Stage IV disease exhibited a significantly shorter OS compared with stages I-III. Future staging systems could be adjusted to better separate stages I and II.

Study context

Population
Patients with pheochromocytoma and sympathetic paraganglioma, TNM stage applied at diagnosis of primary tumor
Participants
458 patients
Exposure
American Joint Committee on Cancer (AJCC) TNM stages I–IV
  • Stage In = 126reference
  • Stage IIn = 213
  • Stage IIIn = 47
  • Stage IVn = 72
Outcome
OS

Main finding

As reported in the abstract

10 years

HR for death

1.50

CI 0.87–2.57

Stage II

HR for death

2.85

CI 1.45–5.63

Stage III

HR for death

8.88

CI 5.16–15.29P < 0.001

Stage IV

Stage I

0.844

95% CI 0.768–0.928

Stage II

0.792

95% CI 0.726–0.865

Stage III

0.595

95% CI 0.435–0.813

Stage IV

0.221

95% CI 0.127–0.384

Median overall survival (OS), whole cohort
18.0 (95% CI, 15.6-not reached) years

Observational data: associations, not effects.

Further findings

OutcomeHR (95% CI)
OS by germline mutationRET 634/918vs No germline mutationsBetter OS with RET 634/9180.28 (0.12–0.69)

OS by germline mutation

Other germline mutations, incl. SDHB, did not show worse OS than patients with metastasis and sporadic disease

Original publication

TNM Staging and Overall Survival in Patients With Pheochromocytoma and Sympathetic Paraganglioma. J Clin Endocrinol Metab. Published 26 November 2022. doi:10.1210/clinem/dgac677

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