Wong W W, Hirose T, Scheithauer B W, Schild S E, Gunderson L L
Department of Radiation Oncology, Mayo Clinic, Scottsdale, AZ 85259, USA. wong.williammayo.edu.
Int J Radiat Oncol Biol Phys. 1998 Sep 1;42(2):351-60. doi: 10.1016/s0360-3016(98)00223-5.
To analyze the results of therapy for malignant peripheral nerve sheath tumor (MPNST) and to identify prognostic factors of survival, and of local and distant control of disease.
From 1975 through 1993, 134 MPNSTs were diagnosed and treated at our institution. Tumor sites included extremities in 36 (27%) cases and non-extremities in 98 (73%). Median follow-up for survivors was 53 months (range: 7-280). There were 14 tumors of histologic grade I disease (10%), 43 of grade II disease (32%), 43 of grade III disease (32%), and 32 of grade IV (24%). Seventy-three patients (54%) underwent radiation therapy (RT) as part of their initial treatment of the primary tumor, including 14 (10%) who had brachytherapy and 16 (12%) who had intraoperative electron irradiation (IOERT) as part of their radiation course.
The 5- and 10-year survival rates were 52% and 34%, respectively. Local and distant failure rates at 5 years were both 49%. On univariate analysis, prognostic factors significantly related to survival (log-rank: p < 0.05) included tumor size, location of disease, history of neurofibromatosis type 1 (NF-1), history of prior irradiation, surgical margin status, use of IOERT or brachytherapy, disease stage, histologic grade and tumor subtype, as well as mitotic rate and the presence or absence of necrosis. On multivariate analysis, only history of prior irradiation (p = 0.023), and surgical margin status (p = 0.0044) remained significant. For local control of disease, univariate analysis showed location of disease, surgical margin status, history of NF-1, history of prior irradiation, mitotic rate, radiation dose > or = 60 Gy, and use of IOERT or brachytherapy to be significant prognostic factors. On multivariate analysis, only surgical margin status (p = 0.0024), RT dose (p = 0.021), and use of IOERT or brachytherapy (p = 0.016) remained significant. For distant control of disease, significant prognostic factors on univariate analysis included tumor size, stage, tumor grade, mitotic rate, presence or absence of necrosis, and histologic subtype. On multivariate analysis, tumor size (p = 0.0065), grade (p = 0.036), and histologic subtype (p = 0.001) remained significant. Patients with perineurial MPNSTs had a much lower rate of distant metastasis and a better overall survival as compared with other histologic subtypes.
Management of patients with MPNST involves a multi-modality approach. The goal of surgery is complete resection with negative margins. Adjuvant irradiation to doses > or = 60 Gy and the inclusion of IOERT or brachytherapy are associated with improved local control of disease.
分析恶性外周神经鞘瘤（MPNST）的治疗结果，确定生存以及疾病局部和远处控制的预后因素。
1975年至1993年期间，我院诊断并治疗了134例MPNST。肿瘤部位包括36例（27%）位于四肢，98例（73%）位于非四肢部位。幸存者的中位随访时间为53个月（范围：7 - 280个月）。组织学I级疾病的肿瘤有14例（10%），II级疾病的有43例（32%），III级疾病的有43例（32%），IV级疾病的有32例（24%）。73例患者（54%）在初始治疗原发性肿瘤时接受了放射治疗（RT），其中14例（10%）接受了近距离放射治疗，16例（12%）在放射治疗过程中接受了术中电子照射（IOERT）。
5年和10年生存率分别为52%和34%。5年时局部和远处失败率均为49%。单因素分析显示，与生存显著相关的预后因素（对数秩检验：p < 0.05）包括肿瘤大小、疾病部位、1型神经纤维瘤病（NF - 1）病史、既往放疗史、手术切缘状态、IOERT或近距离放射治疗的使用、疾病分期、组织学分级和肿瘤亚型，以及有丝分裂率和坏死的有无。多因素分析显示，只有既往放疗史（p = 0.023）和手术切缘状态（p = 0.0044）仍然显著。对于疾病的局部控制，单因素分析显示疾病部位、手术切缘状态、NF - 1病史、既往放疗史、有丝分裂率、放射剂量≥60 Gy以及IOERT或近距离放射治疗的使用是显著的预后因素。多因素分析显示，只有手术切缘状态（p = 0.0024）、RT剂量（p = 0.021）以及IOERT或近距离放射治疗的使用（p = 0.016）仍然显著。对于疾病的远处控制，单因素分析的显著预后因素包括肿瘤大小、分期、肿瘤分级、有丝分裂率、坏死的有无以及组织学亚型。多因素分析显示，肿瘤大小（p = 0.0065）、分级（p = 0.036）和组织学亚型（p = 0.001）仍然显著。与其他组织学亚型相比，束膜型MPNST患者的远处转移率低得多，总体生存率更好。
MPNST患者的治疗采用多模式方法。手术的目标是切缘阴性的完整切除。辅助放疗至剂量≥60 Gy以及采用IOERT或近距离放射治疗与改善疾病的局部控制相关。
