Abstract
Background
Malignant peripheral nerve sheath tumors (MPNSTs) occur sporadically, after prior radiation therapy (RT), or in association with neurofibromatosis type 1 (NF1). It is controversial whether patients with NF1-associated MPNST have worse outcomes. We investigated the prognostic significance of sporadic, NF1-associated, and RT-induced MPNST.
Methods
Patients with primary high-grade MPNST from 1982 to 2011 were identified from a prospectively maintained database. Patients with sporadic MPNST were included only if the MPNST was not associated with NF1 or a neurofibroma or if it was immunohistochemically S100-positive.
Results
We studied 105 patients; 42 had NF1-associated tumors, 49 sporadic, and 14 RT-induced. Median age at diagnosis was 38 years. Median follow-up for surviving patients was 4 years. Mean tumor diameter was 5.5 cm for RT-induced tumors and 9.7 cm for NF1-associated and sporadic tumors (P = 0.004). In multivariate analysis, factors associated with worse disease-specific survival (DSS) were larger size (HR 1.08; 95 % CI 1.04–1.13; P < 0.001) and positive margin (HR 3.30; 95 % CI 1.74–6.28; P < 0.001). Age, gender, site of disease, and S100 staining were not associated with DSS. The 3-year and median DSS were similar for NF1 and sporadic cases; combined 3-year DSS was 64 % and median DSS was 8.0 years. For RT-induced tumors, 3-year DSS was 49 % and median DSS was 2.4 years. The relationship between RT association and DSS approached statistical significance (HR 2.29; 95 % CI 0.93–5.67; P = 0.072).
Conclusions
Margin status and size remain the most important predictors of DSS in patients with MPNST. NF1-associated and sporadic MPNSTs may be associated with improved DSS compared with RT-induced tumors.
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Acknowledgment
This work was supported by Soft Tissue Sarcoma Program Project grant P01 CA 047179 (SS), SPORE in Soft Tissue Sarcoma P50 CA 14014 (SS), and the Dr. Murray F. Brennan/Gorin Fellowship Endowment Fund (JL).
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The authors have no conflicts of interest to report.
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LaFemina, J., Qin, LX., Moraco, N.H. et al. Oncologic Outcomes of Sporadic, Neurofibromatosis-Associated, and Radiation-Induced Malignant Peripheral Nerve Sheath Tumors. Ann Surg Oncol 20, 66–72 (2013). https://doi.org/10.1245/s10434-012-2573-2
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DOI: https://doi.org/10.1245/s10434-012-2573-2