The following is a summary of “TNM Staging and Overall Survival in Patients With Pheochromocytoma and Sympathetic Paraganglioma,” published in the May 2023 issue of Endocrinology & Metabolism by Jimenez, et al.
Pheochromocytomas and paragangliomas (PPGL) are uncommon neuroendocrine tumors, with metastasis occurring in about 15% to 20% of cases. The American Joint Committee on Cancer (AJCC) recently introduced TNM staging guidelines for PPGL. For a study, researchers sought to investigate the relationship between TNM staging and overall survival (OS) in patients with PPGL.
A retrospective analysis was conducted, applying the TNM staging system at the time of primary tumor diagnosis. The primary outcome of interest was overall survival. Unadjusted survival rates were estimated using the Kaplan-Meier method. Cox proportional hazards regression models assessed the associations between OS and relevant covariates.
The study included 458 patients with PPGL. The median overall survival was 18.0 years (95% CI, 15.6 – not reached). At diagnosis, 126 (27.5%) tumors were classified as stage I, 213 (46.5%) as stage II, 47 (10.3%) as stage III, and 72 (15.7%) as stage IV. The 10-year OS probabilities were 0.844 (95% CI, 0.768-0.928) for stage I, 0.792 (95% CI, 0.726-0.865) for stage II, 0.595 (95% CI, 0.435-0.813) for stage III, and 0.221 (95% CI, 0.127-0.384) for stage IV. Compared to stage I, the hazard ratios (HR) for death were 1.50 (0.87-2.57) for stage II, 2.85 (1.45-5.63) for stage III, and 8.88 (5.16-15.29) for stage IV (P < 0.001). Patients with RET 634/918 germline mutations had better overall survival than those without germline mutations (HR: 0.28; 95% CI, 0.12-0.69). Other germline mutations, including SDHB, did not have worse overall survival than patients with metastasis and sporadic disease.
The study findings showed that overall survival rates were correlated with the newly developed AJCC TNM staging for PPGL. In hereditary cases, overall survival was not worse. Stage IV disease was associated with significantly shorter overall survival than stages I-III. Future staging systems could be adjusted to better distinguish between stages I and II.
Source: academic.oup.com/jcem/article-abstract/108/5/1132/6847640?redirectedFrom=fulltext