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Clinical features and prognostic factors of patients with primitive neuroectodermal tumors
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DOI：10.1007/s12672-026-05752-0.png)
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Abstract
En 中文
Primitive neuroectodermal tumors (PNET) are characterized by a poor prognosis, and their management continues to present significant clinical challenges. Despite this, research focusing on patients with PNET remains relatively scarce. This study aims to evaluate the prognosis of this patient population and to identify independent prognostic factors influencing outcomes. Patients diagnosed with PNET, not otherwise specified (NOS), were identified from the Surveillance, Epidemiology, and End Results (SEER) database of the National Cancer Institute, covering the period from 2000 to 2023. Prognostic assessments were conducted utilizing the Kaplan-Meier survival analysis and Cox proportional hazards regression models. The cohort comprised 1001 eligible PNET patients, with the brain representing the most frequent primary tumor site (56.44%). The 5-year overall survival (OS) and cancer-specific survival (CSS) rates were 46.5% and 49.0%, respectively, while the 10-year OS and CSS rates were 41.3% and 44.2%, respectively. Univariate analyses identified age, tumor size, marital status, tumor stage, surgical intervention, and chemotherapy as significant factors associated with OS and CSS. Multivariable analyses revealed that tumor size exceeding 45 mm and distant-stage disease were independently correlated with poorer OS and CSS. Chemotherapy was associated with reduced hazards of both all-cause and cancer-specific mortality. Conversely, radiotherapy did not demonstrate an independent association with OS or CSS after adjustment for covariates. Furthermore, age, marital status, and surgical status were not statistically significant predictors in fully adjusted models. A sensitivity analysis restricted to patients with brain PNET indicated that gross total resection (GTR) was linked to decreased hazards of all-cause and cancer-specific mortality, whereas radiotherapy remained non-significant with respect to OS and CSS after controlling for extent of resection. Larger tumor size and distant-stage disease independently predict poorer overall and CSS in patients with PNET. Chemotherapy is associated with improved survival outcomes, whereas radiotherapy does not exhibit an independent survival benefit after covariate adjustment; however, potential residual confounding by indication cannot be excluded.
Keywords:
Primitive neuroectodermal tumors
Clinical characteristics
Prognostic factors
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