Imura Yoshinori, Outani Hidetatsu, Takenaka Satoshi, Yasuda Naohiro, Nakai Sho, Nakai Takaaki, Wakamatsu Toru, Tamiya Hironari, Hamada Kenichiro, Kakunaga Shigeki
Department of Orthopedic Surgery, Osaka University Graduate School of Medicine, 2-2 Yamadaoka, Suita 565-0871, Osaka, Japan.
Musculoskeletal Oncology Service, Osaka International Cancer Institute, 3-1-69 Otemae, Chuo-ku, Osaka 541-8567, Japan.
Sarcoma. 2021 Nov 24;2021:8335290. doi: 10.1155/2021/8335290. eCollection 2021.
Few studies have described the characteristics and prognostic factors of patients with malignant peripheral nerve sheath tumour (MPNST). In this study, we retrospectively investigated the clinicopathological features, clinical outcomes, and prognostic factors of these patients. . We recruited patients with MPNST who were treated at our institutions from 1991 to 2020. We collected and statistically analysed information on patient-, tumour-, and treatment-related factors. The median follow-up period was 61 months (range, 1-335.8 months).
A total of 60 patients (31 males, 29 females) with a median age of 55 years (range, 8-84 years) at initial diagnosis were included. The median tumour size was 7 cm (range, 1.6-30 cm) in the greatest dimension. The 5-year overall survival (OS) rate of all patients was 69.5%. Univariate analysis revealed that large-sized tumour, metastasis at diagnosis, and no surgery of the primary tumour were significantly associated with patients with worse OS. Multivariate analysis identified surgery of the primary tumour as an independent prognostic factor for improved OS. Among patients with localised disease at diagnosis who underwent surgery of the primary tumour at our institutions, the 5-year OS, local recurrence-free survival (LRFS), and metastasis-free survival (MFS) rates were 81.1%, 78.2%, and 70.3%, respectively. Univariate analysis revealed that positive surgical margin was significantly correlated with unfavourable OS and LRFS, and high grade was a poor prognostic indicator for MFS.
Complete surgical resection with negative surgical margins is necessary for a successful MPNST treatment. Multidisciplinary management of MPNST with aggressive features is important for optimising patient outcomes.
很少有研究描述恶性外周神经鞘瘤（MPNST）患者的特征和预后因素。在本研究中，我们回顾性调查了这些患者的临床病理特征、临床结局和预后因素。我们招募了1991年至2020年在我们机构接受治疗的MPNST患者。我们收集并统计分析了与患者、肿瘤和治疗相关因素的信息。中位随访期为61个月（范围1 - 335.8个月）。
共纳入60例患者（男性31例，女性29例），初诊时中位年龄为55岁（范围8 - 84岁）。肿瘤最大径的中位大小为7 cm（范围1.6 - 30 cm）。所有患者的5年总生存率（OS）为69.5%。单因素分析显示，肿瘤体积大、诊断时转移以及原发肿瘤未手术与OS较差的患者显著相关。多因素分析确定原发肿瘤手术是改善OS的独立预后因素。在我们机构诊断为局限性疾病且接受原发肿瘤手术的患者中，5年OS、无局部复发生存率（LRFS）和无转移生存率（MFS）分别为81.1%、78.2%和70.3%。单因素分析显示，手术切缘阳性与不良的OS和LRFS显著相关，高分级是MFS的不良预后指标。
MPNST治疗成功需要进行手术切缘阴性的完整手术切除。对具有侵袭性特征的MPNST进行多学科管理对于优化患者结局很重要。
