Incidence and prognosis of distant metastasis in malignant peripheral nerve sheath tumors
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ORIGINAL ARTICLE - TUMOR - OTHER
Incidence and prognosis of distant metastasis in malignant peripheral nerve sheath tumors Yao Xu 1 & Guijun Xu 1,2 & Zheng Liu 1,3 & Jincai Duan 1 & Yile Lin 1 & Jiajia Zhu 1 & Vladimir P. Baklaushev 4 & Vladimir P. Chekhonin 5 & Karl Peltzer 6 & Guowen Wang 1 & Xin Wang 7 & Chao Zhang 1 Received: 27 September 2020 / Accepted: 9 November 2020 # Springer-Verlag GmbH Austria, part of Springer Nature 2020
Abstract Background Malignant peripheral nerve sheath tumors (MPNSTs) are a type of soft tissue sarcomas (STS) with recurrence and metastatic potential. We aimed to investigate the risk factors for developing distant metastases (DM) and to identify the prognostic factors in patients with DM. Methods Based on the Surveillance, Epidemiology, and End Result (SEER) database, MPNST patients diagnosed between 2010 and 2016 were extracted in our study. The logistic regression model was performed for predicting DM development while the Cox proportional hazard regression model was conducted for revealing the prognostic factors. Results Eventually, 764 patients diagnosed with MPNSTs were included with 109 cases presenting with metastases at initial diagnosis. Larger tumor size and lymph node metastases were independent risk factors for developing DM. The median overall survival (OS) for patients with metastases was 8.0 (95% CI: 6.1–9.9) months. Multiple metastatic sites and no surgical treatment were prognostic factors for worse survival. Tumors located in non-head and neck region were related with better survival. Conclusions The incidence of DM was 14.3% with a dismal median OS of 8.0 months for metastatic MPNSTs. More evaluation should be applied for patients with large tumor size and lymph metastases. Tumors located in head and neck region and the presence of multiple metastases predicted worse survival outcome. Surgical treatment can significantly improve the survival of MPNST patients with distant metastasis. Keywords Malignant peripheral nerve sheath tumors . SEER program . Distant metastases . Prediction
Introduction Malignant peripheral nerve sheath tumors (MPNSTs), originating from the cells of peripheral nerve sheaths and showing malignant differentiation, are a rare entity in soft tissue
sarcomas (STS) [8]. It was reported that MPNSTs comprised 2–10% of STS and the incidence of MPNSTs was 0.1–0.5/ 100,000 per year [1, 6, 7]. Extremities and trunk are common sites while the tumors at the head and neck region account for only 8–16% of all MPNSTs [28]. Half of MPNSTs are
Yao Xu, Guijun Xu, and Zheng Liu contributed equally to the study This article is part of the Topical Collection on Tumor - Other * Chao Zhang [email protected] 1
Department of Bone and Soft Tissue Tumors, Key Laboratory of Cancer Prevention, Therapy, Tianjin’s Clinical Research Center for Cancer, Tianjin Medical University Cancer Institute and Hospital, National Clinical Research Center for Cancer, Huanhu Xi Road, Tiyuan Bei, Hexi District, Tianjin 300060, China
2
Department of Orthopedics, Tianjin Hospit
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