Clinical predictors of survival in malignant peripheral nerve sheath tumors of the head and neck: A cox regression and nomogram study.
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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.