Outcomes of Treatment for Malignant Peripheral Nerve Sheath Tumors: Different Clinical Features Associated with Neurofibromatosis Type 1 / Journal of the Korean Cancer Association, 대한암학회지
Cancer Research and Treatment
;
: 717-726, 2017.
Article
in English
| WPRIM
| ID: wpr-167296
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 ANDMETHODS:
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.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Pathology
/
Sarcoma
/
Multivariate Analysis
/
Neurofibromatosis 1
/
Neurofibromatoses
/
Seoul
/
Korea
/
Neoplasm Metastasis
/
Neurilemmoma
Type of study:
Prognostic study
Limits:
Humans
Country/Region as subject:
Asia
Language:
English
Journal:
Cancer Research and Treatment
Year:
2017
Type:
Article
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