Oncologic outcomes of sporadic, neurofibromatosis-associated, and radiation-induced malignant peripheral nerve sheath tumors Journal Article
| Authors: | LaFemina, J.; Qin, L. X.; Moraco, N. H.; Antonescu, C. R.; Fields, R. C.; Crago, A. M.; Brennan, M. F.; Singer, S. |
| Article Title: | Oncologic outcomes of sporadic, neurofibromatosis-associated, and radiation-induced malignant peripheral nerve sheath tumors |
| 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. © 2012 Society of Surgical Oncology. |
| Keywords: | immunohistochemistry; adolescent; adult; cancer survival; aged; cancer surgery; major clinical study; intensity modulated radiation therapy; doxorubicin; gemcitabine; cancer adjuvant therapy; follow up; cancer grading; neurofibromatosis; dacarbazine; radiation; etoposide; cyclophosphamide; vincristine; ifosfamide; cancer size; dactinomycin; brachytherapy; protein s 100; external beam radiotherapy; mesna; malignant peripheral nerve sheath tumor; disease specific survival; neurofibroma; cancer prognosis |
| Journal Title: | Annals of Surgical Oncology |
| Volume: | 20 |
| Issue: | 1 |
| ISSN: | 1068-9265 |
| Publisher: | Springer |
| Date Published: | 2013-01-01 |
| Start Page: | 66 |
| End Page: | 72 |
| Language: | English |
| DOI: | 10.1245/s10434-012-2573-2 |
| PROVIDER: | scopus |
| PUBMED: | 22878618 |
| PMCID: | PMC3567771 |
| DOI/URL: | |
| Notes: | --- - "Export Date: 1 February 2013" - "CODEN: ASONF" - "Source: Scopus" |
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