Prognosis and risk factors for malignant peripheral nerve sheath tumor: a systematic review and meta-analysis
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(2020) 18:257
RESEARCH
Open Access
Prognosis and risk factors for malignant peripheral nerve sheath tumor: a systematic review and meta-analysis Zhenyu Cai, Xiaodong Tang, Haijie Liang, Rongli Yang, Taiqiang Yan and Wei Guo*
Abstract Background: No available meta-analysis was printed to systematically introduce the MPNST clinic outcome and risk factors based on largely pooled data. This systematic review and meta-analysis aimed to investigate 5-year OS rate, 5-year EFS rate, and LR rate for MPNST, and to assess potential risk factors for prognosis. Methods: Electronic articles published between January 1, 1966 and February 29, 2020 were searched and critically evaluated. The authors independently reviewed the abstracts and extracted data for 5-year OS rate, 5-year EFS rate, LR rate, and potential risk factors for prognosis. Results: Twenty-eight literatures were finally included for meta-analysis. The pooled 5-year OS rate, 5-year EFS rate, and LR rate were 49%, 37%, and 38%, respectively. The significant prognostic factors for survival were NF1 status, tumor size, depth, location, malignant grade, margin status, chemotherapy, and radiotherapy. Age and sex were not associated with survival. Conclusion: Survival and local recurrence of MPNST are poor. Worse prognosis is mainly associated with NF 1, large size, deep to fascia, high grade, metastases, and location (trunk and head and neck). Complete resection with adequate surgical margins is the mainstay protective factor of MPNST patients, following necessary adjuvant therapies. Keywords: Malignant peripheral nerve sheath tumor, Prognosis, Local recurrence, Risk factors, Meta-analysis
Introduction Malignant peripheral nerve sheath tumor (MPNST) is a rare malignant mesenchymal lesion that accounts for 5% to 10% of all soft tissue sarcoma [1, 2]. Further, 50–60% of patients with MPNST are associated to neurofibromatosis type 1 (NF1); others are radiation-induced or sporadic [3]. The behavior of MPNST is badly aggressive with high local recurrence rate and poor survival. Resection surgery is the main therapy for MPNST, while radiation and systemic chemotherapy was also widely used despite their uncertain effect. Radical therapy surgery combined with adjuvant therapy has been applied past decades. * Correspondence: [email protected] Musculoskeletal Tumor Center, Peking University People’s Hospital, No. 11 Xizhimen South Street, Xicheng District, Beijing 100044, China
However, the prognosis for MPNST patients remains truly dissatisfactory, with 5-year overall survival (OS) rate of 15–66% [1, 4–10], 5-year event-free survival (EFS) of 24– 53% [6, 11–17], and local recurrence (LR) rate of 20% to 85.7% [2, 4, 18–20]. It was obvious that the reported rates varied widely in different literatures. As for genetic and pathology of MPNST, recurrent genetic mutations have been identified in recent studies, such as loss-of function in NF1, PRC2, TP53, CDKN2A, which may provide new opportunities for therapeutic intervention [21]. De Raedt et al. [22] revealed the loss of SUZ12
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