European journal of cancer (Oxford, England : 1990) · 2026
Clinical determinants, diagnostic complexity, and molecular heterogeneity in NF1-associated and sporadic malignant peripheral nerve sheath tumors: A 25-year referral-center cohort.
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Coste Clio, Brahmi Mehdi, Tirode Franck, Dufresne Armelle, Ray-Coquard Isabelle, Karanian Marie, Corradini Nadège, Vaz Gualter, Sunyach Marie-Pierre, Combemale Patrick, Blay Jean-Yves, Amini-Adle Mona
Аннотация
UNLABELLED: Malignant peripheral nerve sheath tumors (MPNST) are rare, aggressive soft-tissue sarcomas frequently associated with neurofibromatosis type 1 (NF1). Prognostic factors and optimal treatment strategies remain poorly defined.
METHODS: Retrospective cohort study including all MPNST patients managed at Centre Léon Bérard (2000-2025). Overall survival (OS) was estimated using Kaplan-Meier and multivariable Cox models with multiple imputation. Transcriptional heterogeneity was explored via bulk RNA sequencing in a tumor subset.
RESULTS: The cohort comprised 206 patients and 219 tumor episodes. Median OS was 53.6 months. NF1-associated tumors had inferior OS compared with sporadic tumors (median OS 26.6 vs 141.0 months, p = 0.012), with a 5-year restricted mean survival time (RMST) reduction of 7.6 months (95% CI -13.8 to -1.2; p = 0.025). Independent predictors of worse OS were higher stage (HR 2.37, p < 0.001), FNCLCC grade (HR 1.79, p < 0.001), NF1 status (HR 1.74, p = 0.006), central location (HR 1.75, p = 0.013) and older age (HR 1.41 per 10 years, p < 0.001); female sex was protective (HR 0.46, p < 0.001). Major biopsy-surgical histologic discordance occurred in 21.3% of cases. Radiotherapy showed context-dependent survival associations, with benefit in grade 3 tumors (HR 0.39, p < 0.001), locally advanced disease (HR 0.32, p = 0.003) and after macroscopically incomplete resections (HR 0.34, p = 0.027). Exploratory transcriptomic analysis identified two NF1-independent expression states defined by Schwann-lineage/immune versus proliferative programs.
CONCLUSIONS: Outcomes remain poor, particularly in NF1. Discordance supports centralized expert review. Disease extent and anatomical location are major prognostic drivers. Radiotherapy may be beneficial in selected high-risk contexts. The transcriptomic landscape supports hypothesis-generating biomarker development based on tumor-state biology beyond NF1 status alone.
METHODS: Retrospective cohort study including all MPNST patients managed at Centre Léon Bérard (2000-2025). Overall survival (OS) was estimated using Kaplan-Meier and multivariable Cox models with multiple imputation. Transcriptional heterogeneity was explored via bulk RNA sequencing in a tumor subset.
RESULTS: The cohort comprised 206 patients and 219 tumor episodes. Median OS was 53.6 months. NF1-associated tumors had inferior OS compared with sporadic tumors (median OS 26.6 vs 141.0 months, p = 0.012), with a 5-year restricted mean survival time (RMST) reduction of 7.6 months (95% CI -13.8 to -1.2; p = 0.025). Independent predictors of worse OS were higher stage (HR 2.37, p < 0.001), FNCLCC grade (HR 1.79, p < 0.001), NF1 status (HR 1.74, p = 0.006), central location (HR 1.75, p = 0.013) and older age (HR 1.41 per 10 years, p < 0.001); female sex was protective (HR 0.46, p < 0.001). Major biopsy-surgical histologic discordance occurred in 21.3% of cases. Radiotherapy showed context-dependent survival associations, with benefit in grade 3 tumors (HR 0.39, p < 0.001), locally advanced disease (HR 0.32, p = 0.003) and after macroscopically incomplete resections (HR 0.34, p = 0.027). Exploratory transcriptomic analysis identified two NF1-independent expression states defined by Schwann-lineage/immune versus proliferative programs.
CONCLUSIONS: Outcomes remain poor, particularly in NF1. Discordance supports centralized expert review. Disease extent and anatomical location are major prognostic drivers. Radiotherapy may be beneficial in selected high-risk contexts. The transcriptomic landscape supports hypothesis-generating biomarker development based on tumor-state biology beyond NF1 status alone.