TY - JOUR
T1 - TNM Staging and Overall Survival in Patients with Pheochromocytoma and Sympathetic Paraganglioma
AU - Jimenez, Camilo
AU - Ma, Junsheng
AU - Gonzalez, Alejandro Roman
AU - Varghese, Jeena
AU - Zhang, Miao
AU - Perrier, Nancy
AU - Habra, Mouhammed Amir
AU - Graham, Paul
AU - Waguespack, Steven G.
N1 - Publisher Copyright:
© The Author(s) 2022. Published by Oxford University Press on behalf of the Endocrine Society. All rights reserved.
PY - 2023/5/1
Y1 - 2023/5/1
N2 - Context: Pheochromocytomas and paragangliomas (PPGL) are rare neuroendocrine tumors. Metastases develop in 15% to 20%. The American Joint Committee on Cancer (AJCC) established inaugural guidelines for PPGL tumor-node-metastasis (TNM) staging. Objective: The objective of this analysis is to investigate the associations between TNM staging and overall survival (OS). Methods: We retrospectively applied the TNM staging at the time of diagnosis of the primary tumor. The primary outcome was OS. Unadjusted survival rates were estimated by the Kaplan–Meier method. Cox proportional hazards regression models were used to evaluate the associations between OS and covariates of interest. Results: The study included 458 patients. Median OS was 18.0 (95% CI, 15.6-not reached) years. At diagnosis, 126 (27.5%) tumors were stage I, 213 (46.5%) were stage II, 47 (10.3%) were stage III, and 72 (15.7%) were stage IV. The 10-year OS probabilities were 0.844 (95% CI, 0.768-0.928) for patients with stage I tumors, 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 with 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). Compared with patients with no germline mutations, those with RET 634/918 had better OS (HR: 0.28; 95% CI, 0.12-0.69). Other germline mutations, including SDHB, did not exhibit worse OS than patients with metastasis and sporadic disease. 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.
AB - Context: Pheochromocytomas and paragangliomas (PPGL) are rare neuroendocrine tumors. Metastases develop in 15% to 20%. The American Joint Committee on Cancer (AJCC) established inaugural guidelines for PPGL tumor-node-metastasis (TNM) staging. Objective: The objective of this analysis is to investigate the associations between TNM staging and overall survival (OS). Methods: We retrospectively applied the TNM staging at the time of diagnosis of the primary tumor. The primary outcome was OS. Unadjusted survival rates were estimated by the Kaplan–Meier method. Cox proportional hazards regression models were used to evaluate the associations between OS and covariates of interest. Results: The study included 458 patients. Median OS was 18.0 (95% CI, 15.6-not reached) years. At diagnosis, 126 (27.5%) tumors were stage I, 213 (46.5%) were stage II, 47 (10.3%) were stage III, and 72 (15.7%) were stage IV. The 10-year OS probabilities were 0.844 (95% CI, 0.768-0.928) for patients with stage I tumors, 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 with 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). Compared with patients with no germline mutations, those with RET 634/918 had better OS (HR: 0.28; 95% CI, 0.12-0.69). Other germline mutations, including SDHB, did not exhibit worse OS than patients with metastasis and sporadic disease. 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.
KW - RET
KW - SDHB
KW - AJCC 8th edition cancer staging manual
KW - paraganglioma
KW - pheochromocytoma
KW - prognosis
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U2 - 10.1210/clinem/dgac677
DO - 10.1210/clinem/dgac677
M3 - Article
C2 - 36433823
AN - SCOPUS:85152488156
SN - 0021-972X
VL - 108
SP - 1132
EP - 1142
JO - Journal of Clinical Endocrinology and Metabolism
JF - Journal of Clinical Endocrinology and Metabolism
IS - 5
ER -