Martin Enrico, Muskens Ivo S, Coert J H, Smith Timothy R, Broekman Marike L D
Computational Neuroscience Outcomes Center, Department of Neurosurgery, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
Department of Neurosurgery, Brain Center Rudolf Magnus, University Medical Center Utrecht, The Netherlands.
Neurooncol Pract. 2019 Mar;6(2):134-143. doi: 10.1093/nop/npy025. Epub 2018 Jul 19.
Currently, literature is scarce on differences across all possible tumor sites in malignant peripheral nerve sheath tumors (MPNSTs). To determine differences in treatment and survival across tumor sites and assess possible predictors for survival, we used the Surveillance, Epidemiology, and End Results (SEER) database.
MPNST cases were obtained from the SEER database. Tumor sites were recoded into: intracranial, spinal, head and neck (H&N), limbs, core (thorax/abdomen/pelvis), and unknown site of origin. Patient and tumor characteristics, treatment modalities, and survival were extracted. Overall survival (OS) was assessed using univariable and multivariable Cox regression hazard models. Kaplan-Meier survival curves were constructed per tumor site for OS and disease-specific survival (DSS).
A total of 3267 MPNST patients were registered from 1973 to 2013; 167 intracranial (5.1%), 119 spinal (3.6%), 449 H&N (13.7%), 1022 limb (31.3%), 1307 core (40.0%), and 203 unknown (6.2%). The largest tumors were found in core sites (80.0 mm, interquartile range [IQR]: 60.0-115.0 mm) and the smallest were intracranial (37.4 mm, IQR: 17.3-43.5 mm). Intracranial tumors were least frequently resected (58.1%), whereas spinal tumors were most often resected (83.0%). Radiation was administered in 35.5% to 41.8%. Independent factors associated with decreased survival were: older age, male sex, black race, no surgery, partial resection, large tumor size, high tumor grade, H&N site, and core site (all < .05). Intracranial and pediatric tumors show superior survival (both < .05). Intracranial tumors show superior OS and DSS curves, whereas core tumors have the worst ( < .001).
Superior survival is seen in intracranial and pediatric MPNSTs. Core and H&N tumors have a worse prognosis.
目前，关于恶性外周神经鞘瘤（MPNSTs）在所有可能肿瘤部位差异的文献较少。为了确定不同肿瘤部位在治疗和生存方面的差异，并评估可能的生存预测因素，我们使用了监测、流行病学和最终结果（SEER）数据库。
从SEER数据库中获取MPNST病例。肿瘤部位重新编码为：颅内、脊柱、头颈部（H&N）、四肢、躯干（胸/腹/盆腔）以及原发部位不明。提取患者和肿瘤特征、治疗方式及生存情况。使用单变量和多变量Cox回归风险模型评估总生存（OS）。针对每个肿瘤部位构建OS和疾病特异性生存（DSS）的Kaplan-Meier生存曲线。
1973年至2013年共登记了3267例MPNST患者；167例颅内（5.1%），119例脊柱（3.6%），449例头颈部（13.7%），1022例四肢（31.3%），1307例躯干（40.0%），203例不明（6.2%）。最大的肿瘤见于躯干部位（80.0 mm，四分位间距[IQR]：60.0 - 115.0 mm），最小的是颅内肿瘤（37.4 mm，IQR：17.3 - 43.5 mm）。颅内肿瘤切除率最低（58.1%），而脊柱肿瘤切除率最高（83.0%）。35.5%至41.8%的患者接受了放疗。与生存降低相关的独立因素包括：年龄较大、男性、黑人种族、未手术、部分切除、肿瘤体积大、肿瘤分级高、头颈部部位和躯干部位（均P <.05）。颅内和儿童肿瘤显示出较好的生存（均P <.05）。颅内肿瘤显示出较好的OS和DSS曲线，而躯干肿瘤最差（P <.001）。
颅内和儿童MPNSTs的生存情况较好。躯干和头颈部肿瘤预后较差。
