Clinical predictors of survival in malignant peripheral nerve sheath tumors of the head and neck: A cox regression and nomogram study
Objectives: Malignant Peripheral Nerve Sheath Tumors (MPNST) are rapidly progressing Schwann cell neoplasms. This study aimed to develop a practical clinical nomogram that predicts prognosis in patients with Head and Neck MPNST (HN-MPNST) using the Surveillance, Epidemiology, and End Results (SEER)...
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Elsevier
2025-11-01
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| Series: | Brazilian Journal of Otorhinolaryngology |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S1808869425001466 |
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| author | Sun LiNa Tu Jianfei Tian Zhifeng Wang Xinbin Wu Hui |
| author_facet | Sun LiNa Tu Jianfei Tian Zhifeng Wang Xinbin Wu Hui |
| author_sort | Sun LiNa |
| collection | DOAJ |
| description | Objectives: Malignant Peripheral Nerve Sheath Tumors (MPNST) are rapidly progressing Schwann cell neoplasms. This study aimed to develop a practical clinical nomogram that predicts prognosis in patients with Head and Neck MPNST (HN-MPNST) using the Surveillance, Epidemiology, and End Results (SEER) database. Methods: We extracted clinical data from patients with HN MPNST between 2000 and 2020 from the SEER database. Patients were randomly divided into training cohort and validation cohorts. To estimate the chance of survival in patients with HN MPNST, we developed a nomogram. The nomogram performance was evaluated by discrimination and calibration. In addition, a decision curve analysis was conducted to evaluate the clinical usefulness of this newly developed model. Molecular data highlighting the most frequent mutations in MPNST were obtained from the Catalogue Of Somatic Mutations In Cancer (COSMIC) database. Results: In the primary cohort, 431 patients met the inclusion criteria to be entered into this study. The median Overall Survival (OS) was 3.9-years (95% CI 2.8–6.7), and the 1-, 3-, and 5-year OS rates were 75.2%, 52.6%, and 47.1%, respectively. We included 129 consecutive patients in the validation cohort. AJCC (American Joint Committee on Cancer) staging, Collaborative Stage (CS) extension, and CS tumor size were included in the nomogram. The calibration plots showed an agreement between the predictions and observations. Based on the clinical decision curve, the model had a net clinical benefit for patients with MPNST. Analysis of COSMIC data revealed frequent mutations of MPNST in NF1 (22%), SMARCB1 (21%), TP53 (12%), SUZ12 (19%), and less commonly in BRAF and CDKN2A (each 2%). Conclusion: Radiotherapy improves survival in patients with metastatic disease or tumors ≥ 6 cm. Using this nomogram can assist in clinical decision making, as it has satisfactory accuracy. However, an additional external validation is required. Level of evidence: 2. |
| format | Article |
| id | doaj-art-2bc770b7130843a384b45f3102987aa6 |
| institution | Kabale University |
| issn | 1808-8694 |
| language | English |
| publishDate | 2025-11-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Brazilian Journal of Otorhinolaryngology |
| spelling | doaj-art-2bc770b7130843a384b45f3102987aa62025-08-24T05:12:00ZengElsevierBrazilian Journal of Otorhinolaryngology1808-86942025-11-0191610170210.1016/j.bjorl.2025.101702Clinical predictors of survival in malignant peripheral nerve sheath tumors of the head and neck: A cox regression and nomogram studySun LiNa0Tu Jianfei1Tian Zhifeng2Wang Xinbin3Wu Hui4Department of Neurology, Lishui Central Hospital and Fifth Affiliated Hospital of Wenzhou Medical College, Zhejiang, ChinaDepartment of Oncology, Lishui Central Hospital and Fifth Affiliated Hospital of Wenzhou Medical College, Zhejiang, ChinaDepartment of Oncology, Lishui Central Hospital and Fifth Affiliated Hospital of Wenzhou Medical College, Zhejiang, ChinaDepartment of Oral and Maxillofacial Surgery, Lishui Central Hospital and Fifth Affiliated Hospital of Wenzhou Medical College, Zhejiang, ChinaDepartment of Oncology, Lishui Central Hospital and Fifth Affiliated Hospital of Wenzhou Medical College, Zhejiang, China; Corresponding author.Objectives: Malignant Peripheral Nerve Sheath Tumors (MPNST) are rapidly progressing Schwann cell neoplasms. This study aimed to develop a practical clinical nomogram that predicts prognosis in patients with Head and Neck MPNST (HN-MPNST) using the Surveillance, Epidemiology, and End Results (SEER) database. Methods: We extracted clinical data from patients with HN MPNST between 2000 and 2020 from the SEER database. Patients were randomly divided into training cohort and validation cohorts. To estimate the chance of survival in patients with HN MPNST, we developed a nomogram. The nomogram performance was evaluated by discrimination and calibration. In addition, a decision curve analysis was conducted to evaluate the clinical usefulness of this newly developed model. Molecular data highlighting the most frequent mutations in MPNST were obtained from the Catalogue Of Somatic Mutations In Cancer (COSMIC) database. Results: In the primary cohort, 431 patients met the inclusion criteria to be entered into this study. The median Overall Survival (OS) was 3.9-years (95% CI 2.8–6.7), and the 1-, 3-, and 5-year OS rates were 75.2%, 52.6%, and 47.1%, respectively. We included 129 consecutive patients in the validation cohort. AJCC (American Joint Committee on Cancer) staging, Collaborative Stage (CS) extension, and CS tumor size were included in the nomogram. The calibration plots showed an agreement between the predictions and observations. Based on the clinical decision curve, the model had a net clinical benefit for patients with MPNST. Analysis of COSMIC data revealed frequent mutations of MPNST in NF1 (22%), SMARCB1 (21%), TP53 (12%), SUZ12 (19%), and less commonly in BRAF and CDKN2A (each 2%). Conclusion: Radiotherapy improves survival in patients with metastatic disease or tumors ≥ 6 cm. Using this nomogram can assist in clinical decision making, as it has satisfactory accuracy. However, an additional external validation is required. Level of evidence: 2.http://www.sciencedirect.com/science/article/pii/S1808869425001466Oral cancerHead and neck cancerMalignant Peripheral Nerve Tumors (MPNST)SurvivalSEER databaseCOSMIC |
| spellingShingle | Sun LiNa Tu Jianfei Tian Zhifeng Wang Xinbin Wu Hui Clinical predictors of survival in malignant peripheral nerve sheath tumors of the head and neck: A cox regression and nomogram study Brazilian Journal of Otorhinolaryngology Oral cancer Head and neck cancer Malignant Peripheral Nerve Tumors (MPNST) Survival SEER database COSMIC |
| title | Clinical predictors of survival in malignant peripheral nerve sheath tumors of the head and neck: A cox regression and nomogram study |
| title_full | Clinical predictors of survival in malignant peripheral nerve sheath tumors of the head and neck: A cox regression and nomogram study |
| title_fullStr | Clinical predictors of survival in malignant peripheral nerve sheath tumors of the head and neck: A cox regression and nomogram study |
| title_full_unstemmed | Clinical predictors of survival in malignant peripheral nerve sheath tumors of the head and neck: A cox regression and nomogram study |
| title_short | Clinical predictors of survival in malignant peripheral nerve sheath tumors of the head and neck: A cox regression and nomogram study |
| title_sort | clinical predictors of survival in malignant peripheral nerve sheath tumors of the head and neck a cox regression and nomogram study |
| topic | Oral cancer Head and neck cancer Malignant Peripheral Nerve Tumors (MPNST) Survival SEER database COSMIC |
| url | http://www.sciencedirect.com/science/article/pii/S1808869425001466 |
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