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Updated: May 30, 2026
Genetic Profiling and Genome-Scale Dropout Screening to Identify Therapeutic Targets in Mouse Models of Malignant Peripheral Nerve Sheath Tumor
Published on: August 25, 2023
Malignant peripheral nerve sheath tumors (MPNST): the Mayo Clinic experience
Chee-Chee H Stucky1, Kevin N Johnson, Richard J Gray
1Department of General Surgery, Mayo Clinic Arizona, Phoenix, AZ, USA.
Background:
Malignant peripheral nerve sheath tumors (MPNST) are a rare form of soft tissue sarcoma with few studies reporting on patient outcomes and prognostic variables.
Methods:
A retrospective review of 175 patients diagnosed with MPNST from 1985 to 2010 was performed. Patient, tumor, and treatment characteristics were evaluated to identify prognostic variables.
Results:
The median age of our study population was 44 years, and 51% were female. Median tumor size was 6 cm, and 61% of patients had high-grade tumors. Tumors were most commonly located on the extremities (45%), then trunk (34%) and head/neck (19%). The majority of patients underwent surgical resection (95%) and adjuvant treatment with chemotherapy (6%), radiation (42%) or both (22%). Margin status was R0 in 69%, R1 in 2%, R2 in 9%, and unknown in 20%. The local recurrence rate was 22%, and 5- and 10-year disease-specific survival (DSS) were 60% and 45%, respectively. On univariate analysis, no predictors for local recurrence were identified. Tumor size ≥ 5 cm, high tumor grade, tumor location, presence of neurofibromatosis type 1, local recurrence, and adjuvant chemotherapy were all associated with DSS. On multivariate analysis, size ≥ 5 cm [hazard ratio (HR)= 6.1, 95% confidence interval (CI) 1.5-25.0], local recurrence (HR = 4.4, 95% CI 1.7-11.4), high tumor grade (HR = 3.8, 95% CI 1.1-13.2), and truncal location (HR = 3.7, 95% CI 1.1-12.7) were poor prognostic indicators for DSS.
Conclusions:
High tumor grade and tumor size ≥ 5 cm predict adverse DSS for MPNST. In the context of a multidisciplinary treatment regimen, local recurrence and survival outcomes at 5 and 10 years were better than previously reported for MPNST.
Insights
Malignant peripheral nerve sheath tumors (MPNST) larger than 5 cm and high tumor grade predict poor survival. Multidisciplinary care improved local recurrence and survival outcomes for MPNST patients.
Area of Science:
- Oncology
- Surgical Pathology
- Sarcoma Research
Background:
- Malignant peripheral nerve sheath tumors (MPNST) are rare soft tissue sarcomas.
- Limited data exists on MPNST patient outcomes and prognostic factors.
Purpose of the Study:
- To identify prognostic variables for malignant peripheral nerve sheath tumors.
- To evaluate patient, tumor, and treatment characteristics influencing outcomes.
Main Methods:
- Retrospective review of 175 MPNST patients diagnosed between 1985 and 2010.
- Analysis of patient demographics, tumor characteristics, and treatment modalities.
- Statistical evaluation for local recurrence and disease-specific survival (DSS).
Main Results:
- Tumor size ≥ 5 cm, high tumor grade, truncal location, and local recurrence were associated with poorer DSS.
- 5-year DSS was 60%, and 10-year DSS was 45%.
- Multivariate analysis identified tumor size, local recurrence, high grade, and truncal location as poor prognostic indicators.
Conclusions:
- High tumor grade and tumor size ≥ 5 cm are significant predictors of adverse DSS in MPNST.
- Multidisciplinary treatment approaches may improve survival outcomes compared to historical data.
