{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,17]],"date-time":"2026-10-17T16:05:09Z","timestamp":1792253109738,"version":"4.4.0"},"reference-count":29,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"1","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JNS"],"published-print":{"date-parts":[[2013,1]]},"abstract":"<jats:sec>\n<jats:title>Object<\/jats:title>\n<jats:p>Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive sarcomas that often arise from major peripheral nerves. Approximately half of MPNSTs arise in patients with neurofibromatosis Type 1 (NF1) who, in comparison with patients without NF1, present at younger ages and with larger tumors that are commonly associated with extensive plexiform neurofibromas. These tumors therefore pose a particularly difficult treatment challenge because of the morbidity often associated with attempted gross-total resection (GTR). Here, the authors aim to examine what role the extent of resection and other covariates play in the long-term survival of patients with NF1 in the setting of MPNST.<\/jats:p><\/jats:sec>\n<jats:sec>\n<jats:title>Methods<\/jats:title>\n<jats:p>The authors retrospectively reviewed the records of 23 adult patients with NF1 who underwent surgery for MPNSTs at their institution between 1991 and 2008. The primary end points of the study were mortality, local recurrence, and metastasis. Kaplan-Meier survival curves were evaluated for all patients. Differences for each of the primary end points were evaluated based on cause-specific covariates, which included tiered tumor size, tumor location, grade, resection margin status, postoperative weakness, and use of chemotherapy and radiation therapy. Multivariate analysis was performed using Cox proportional hazards models.<\/jats:p><\/jats:sec>\n<jats:sec>\n<jats:title>Results<\/jats:title>\n<jats:p>Gross-total resection (p = 0.01) and surgical margin status (p = 0.034) had a statistically important role in prolonging overall survival in patients with NF1 by univariate analysis. When tumor size, location, grade, postoperative weakness, and radiation therapy were also taken into account using multivariate analysis, GTR continued to be a significant prognostic factor (p = 0.035).<\/jats:p><\/jats:sec>\n<jats:sec>\n<jats:title>Conclusions<\/jats:title>\n<jats:p>These findings suggest that GTR offers significant long-term benefit on survival in patients with NF1. Benefit on survival occurred independently of all other covariates, suggesting that complete resection should be the principal goal of treatment in this patient population.<\/jats:p><\/jats:sec>","DOI":"10.3171\/2012.9.jns101610","type":"journal-article","created":{"date-parts":[[2012,10,26]],"date-time":"2012-10-26T14:09:22Z","timestamp":1351260562000},"page":"142-148","source":"Crossref","is-referenced-by-count":96,"title":["Role of resection of malignant peripheral nerve sheath tumors in patients with neurofibromatosis Type 1"],"prefix":"10.3171","volume":"118","author":[{"given":"Gavin P.","family":"Dunn","sequence":"first","affiliation":[{"name":"Departments of Neurosurgery,"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Konstantinos","family":"Spiliopoulos","sequence":"additional","affiliation":[{"name":"Departments of Neurosurgery,"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Scott R.","family":"Plotkin","sequence":"additional","affiliation":[{"name":"Neurology,"},{"name":"Cancer Center, and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francis J.","family":"Hornicek","sequence":"additional","affiliation":[{"name":"Orthopedic Oncology,"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David C.","family":"Harmon","sequence":"additional","affiliation":[{"name":"Cancer Center, and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas F.","family":"Delaney","sequence":"additional","affiliation":[{"name":"Cancer Center, and"},{"name":"Radiation Oncology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ziv","family":"Williams","sequence":"additional","affiliation":[{"name":"Departments of Neurosurgery,"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1754","reference":[{"key":"b1-jns101610","series-title":"Cancer","doi-asserted-by":"publisher","first-page":"1065","DOI":"10.1002\/cncr.22098","article-title":"Malignant peripheral nerve sheath tumors: prognostic factors and survival in a series of patients treated at a single institution","volume":"107","author":"Anghileri","year":"2006"},{"key":"b2-jns101610","series-title":"Neurology","doi-asserted-by":"publisher","first-page":"696","DOI":"10.1212\/01.WNL.0000078813.05925.2C","article-title":"Malignant peripheral nerve sheath tumor: the clinical spectrum and outcome of treatment","volume":"61","author":"Baehring","year":"2003"},{"key":"b3-jns101610","series-title":"J Neurooncol","doi-asserted-by":"publisher","first-page":"335","DOI":"10.1023\/B:NEON.0000041891.39474.cb","article-title":"Peripheral nerve tumors: management strategies and molecular insights","volume":"69","author":"Bhattacharyya","year":"2004"},{"key":"b4-jns101610","series-title":"Int J Cancer","doi-asserted-by":"publisher","first-page":"563","DOI":"10.1002\/ijc.24783","article-title":"Preclinical in vivo evaluation of rapamycin in human malignant peripheral nerve sheath explant xenograft","volume":"126","author":"Bhola","year":"2010"},{"key":"b5-jns101610","series-title":"Clin Orthop Relat Res","doi-asserted-by":"publisher","first-page":"69","DOI":"10.1097\/01.blo.0000131256.82455.c5","article-title":"Survival data for patients with malignant schwannoma","volume":"426","author":"Cashen","year":"2004"},{"key":"b6-jns101610","series-title":"Am J Hum Genet","doi-asserted-by":"publisher","first-page":"1288","DOI":"10.1086\/374821","article-title":"Elevated risk for MPNST in NF1 microdeletion patients","volume":"72","author":"De Raedt","year":"2003"},{"key":"b7-jns101610","series-title":"Int J Radiat Oncol Biol Phys","doi-asserted-by":"publisher","first-page":"1117","DOI":"10.1016\/S0360-3016(03)00186-X","article-title":"Neoadjuvant chemotherapy and radiotherapy for large extremity soft-tissue sarcomas","volume":"56","author":"DeLaney","year":"2003"},{"key":"b8-jns101610","series-title":"Eur J Surg Oncol","doi-asserted-by":"publisher","first-page":"78","DOI":"10.1016\/S0748-7983(05)80073-3","article-title":"Malignant peripheral nerve sheath tumors in patients with and without neurofibromatosis","volume":"21","author":"Doorn","year":"1995"},{"key":"b9-jns101610","series-title":"Cancer","doi-asserted-by":"publisher","first-page":"2006","DOI":"10.1002\/1097-0142(19860515)57:10<2006::AID-CNCR2820571022>3.0.CO;2-6","article-title":"Malignant peripheral nerve sheath tumors. 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