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. 2017 Jul;49(3):717-726.
doi: 10.4143/crt.2016.271. Epub 2016 Dec 1.

Outcomes of Treatment for Malignant Peripheral Nerve Sheath Tumors: Different Clinical Features Associated with Neurofibromatosis Type 1

In Kyung Hwang  1   2 , Seung Min Hahn  1   2 , Hyo Sun Kim  1   2 , Sang Kyum Kim  3 , Hyo Song Kim  4 , Kyoo-Ho Shin  5 , Chang Ok Suh  6 , Chuhl Joo Lyu  1   2 , Jung Woo Han  1   2
Affiliations

Outcomes of Treatment for Malignant Peripheral Nerve Sheath Tumors: Different Clinical Features Associated with Neurofibromatosis Type 1

In Kyung Hwang et al. Cancer Res Treat. 2017 Jul.

Abstract

Purpose: Malignant peripheral nerve sheath tumors (MPNSTs) are a rare subtype of sarcoma that occur spontaneously or in association with neurofibromatosis type 1 (NF-1). This study aimed to clinically differentiate these types of MPNSTs.

Materials and methods: The study reviewed 95 patients diagnosed with and treated for MPNST at Yonsei University Health System, Seoul, Korea over a 27-year period. The clinical characteristics, prognostic factors, and treatment outcomes of sporadic MPNST (sMPNST) and NF-1 associated MPNST (NF-MPNST) cases were compared.

Results: Patients with NF-MPNST had a significantly lower median age (32 years vs. 45 years for sMPNST, p=0.012), significantly larger median tumor size (8.2 cm vs. 5.0 cm for sMPNST, p < 0.001), and significantly larger numbers of imaging studies and surgeries (p=0.004 and p < 0.001, respectively). The 10-year overall survival (OS) rate of the patients with MPNST was 52±6%. Among the patients with localized MPNST, patients with NF-MPNST had a significantly lower 10-year OS rate (45±11% vs. 60±8% for sMPNST, p=0.046). Univariate analysis revealed the resection margin, pathology grade, and metastasis to be significant factors affecting the OS (p=0.001, p=0.020, and p < 0.001, respectively). Multivariate analysis of the patients with localized MPNST identified R2 resection and G1 as significant prognostic factors for OS.

Conclusion: NF-MPNST has different clinical features from sMPNST and requires more careful management. Further study will be needed to develop specific management plans for NF-MPNST.

Keywords: Neoplasms; Neurilemmoma; Neurofibromatoses; Sarcoma.

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Conflict of interest statement

Conflict of interest relevant to this article was not reported.

Figures

Fig. 1.
Fig. 1.
Scheme of first-line treatment modalities. sMPNST, sporadic malignant peripheral nerve sheath tumors; NF-MPNST, malignant peripheral nerve sheath tumors associated with neurofibromatosis type 1; Chemo, chemotherapy; RT, radiotherapy; Both, chemotherapy and radiotherapy; NED, no evidence of disease; R0/R1/2, resection margin, see details in Materials and Methods section; Tx, treatment; F/U, follow-up.
Fig. 2.
Fig. 2.
Overall survival of patients with malignant peripheral nerve sheath tumors.
Fig. 3.
Fig. 3.
Overall survival according to neurofibromatosis type 1 (NF-1) status. (A) Overall survival associated with nonmetastatic malignant peripheral nerve sheath tumors. (B) Overall survival associated with metastatic malignant peripheral nerve sheath tumors.
Fig. 4.
Fig. 4.
Overall survival according to the risk factors. (A) Overall survival associated with the margin status (positive vs. negative). (B) Overall survival associated with the pathologic grade (not acquired [N/A] vs. G1 vs. G2 vs. G3). (C) Overall survival associated with the metastatic status (localized vs. metastasis).
See this image and copyright information in PMC

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