Tumor-node-metastasis (TNM) Staging and Overall Survival in Patients With Pheochromocytoma and Sympathetic Paraganglioma
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
| Outcome | HR (95% CI) | |
|---|---|---|
| OS by germline mutationRET 634/918vs No germline mutationsBetter OS with RET 634/918 | 0.28 (0.12–0.69) | |
| 0.10.31310Hazard ratio (log scale) |
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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