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Malignant Peripheral Nerve Sheath Tumors: A Single Institution’s Experience Using Combined Surgery and Radiation Therapy

Bishop, Andrew, J., MD*; Zagars, Gunar, K., MD*; Torres, Keila, E., MD, PhD; Bird, Justin, E., MD; Feig, Barry, W., MD; Guadagnolo, B., Ashleigh, MD, MPH*,§

American Journal of Clinical Oncology: May 2018 - Volume 41 - Issue 5 - p 465–470
doi: 10.1097/COC.0000000000000303
Original Articles: Soft Tissue

Purpose: The purpose of this study is to investigate local control (LC), survival outcomes, and associated prognostic factors for patients with malignant peripheral nerve sheath tumors (MPNSTs) treated with combined surgery and radiation therapy (RT).

Methods: We reviewed the medical records of 71 consecutive patients treated with surgery and RT for localized MPNST between 1965 and 2012. Preoperative RT was used to treat 23 patients (32%) to a median dose of 50 Gy (range, 50 to 60 Gy), whereas 48 (68%) received postoperative RT to a median dose of 64 Gy (range, 45 to 70 Gy).

Results: Median follow-up for living patients was 118 months (range, 21 to 512 mo). The 5-year LC, distant metastatic free survival, and disease-specific survival rates were 84%, 62%, and 66%, respectively. To identify predictors of outcome, several multivariate models were constructed: (1) positive/uncertain surgical margin status was the only factor adversely associated local relapse at 5 years (28% vs. 5% for negative margins; P=0.02; hazard ratios 5.92; 95% confidence interval, 1.3-27.4). (2) No factors were significantly associated with distant metastatic free survival. Of the 35 patients (49%) who sustained disease relapse, only 3 were ultimately salvaged. Only 2 patients had grade 2 late toxicities (necrosis, fibrosis) based on Common Terminology Criteria for Adverse Events version 4.03 criteria, and 1 patient had grade 1 edema.

Conclusions: Combination therapy with surgery and RT provides favorable LC. Distant recurrences, however, continue to be challenging with limited salvage success at the time of relapse.

Departments of *Radiation Oncology

Surgical Oncology

Orthopedic Oncology

§Health Services Research, The University of Texas MD Anderson Cancer Center, Houston, TX

Supported in part by Cancer Center Support (Core) Grant CA016672 to The University of Texas MD Anderson Cancer Center.

The authors declare no conflicts of interest.

Reprints: B. Ashleigh Guadagnolo, MD, MPH, Department of Radiation Oncology, Unit 97, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd., Houston, TX 77030. E-mail: aguadagn@mdanderson.org.

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