Background/Objectives: This retrospective observational study aimed to investigate the perioperative outcome in Malignant Peripheral Nerve Sheath Tumors (MPNSTs) with and without relation to Neurofibromatosis Type 1 (NF1) and to detect possible influencing factors. Methods: Clinical reports, histopathological evaluations, imaging, and treatment characteristics were reviewed in 35 operated MPNSTs in 33 patients. Possible predictive valuables included disease type, preoperative tumor volume, SUV and MIB-1 proliferation index, resection margins, the presence of metastasis, and whether radio-/chemotherapy was received. Results: Patients with NF1 were younger (mean age: 29 ± 13, 8–54 years) than sporadic cases (mean age: 45 ± 13, 24–67 years) and exhibited significantly larger preoperative tumor volumes (mean 299 vs. 18 cm3,p= 0.048). Most tumors were located in the facial/cervical/neck area (34%, n = 12), followed by the trunk (31%, n = 11), lower extremity (17%, n = 6), upper extremity (14%, n = 5), and intraspinal area (3%, n = 1). NF1-associated MPNSTs appeared predominantly on the trunk (39%) and sporadically in the facial/cervical/neck area (50%). Complete resection was possible in 66% and an improvement in or stability of function was achieved in most cases (motor 69%, sensory 74%), as well as a decrease in pain intensity (63%). NF1-associated MPNSTs exhibited more severe pain scores (median VRS scale 2,p= 0.002) compared to sporadic tumors (median VRS scale 0.5). Sporadic MPNSTs located at the head/facial/brachial plexus and upper extremities exhibited better preoperative functions compared to those on the lower extremities. In 12 cases with available [18F]FDG PET, the mean preoperative SUV (9.8 ± 7.2) positively correlated with the mean maximum MIB-1 index (34 ± 26%,p= 0.005) and the mean preoperative tumor volume (474.7 ± 68.6 cm3,p= 0.047). The overall survival (OS) was significantly longer in tumors with higher resection extents (R0,p= 0.01) and without accompanying metastasis (p= 0.046), and tended to be longer, but not significantly so, in sporadic MPNSTs. In six and seven tumors, with R1/R2 resection margins and present metastasis, respectively, solid or combined neo-/adjuvant radio-/chemotherapy led to a significantly shorter OS (p= 0.014). Conclusions: NF1-associated MPNSTs have larger tumor volumes, higher SUVs and MIB-1 proliferation indices, and a shorter overall survival period. Nevertheless, surgery can improve symptoms, particularly medication-resistant pain, and should also be considered in advanced disease for symptom control/improvement.
背景/目的：本研究为回顾性观察性研究，旨在探讨与1型神经纤维瘤病（NF1）相关及不相关的恶性外周神经鞘瘤（MPNSTs）的围手术期结局，并识别可能的影响因素。方法：回顾分析了33例患者共35例手术治疗的MPNSTs的临床报告、组织病理学评估、影像学资料及治疗特征。可能的预测变量包括疾病类型、术前肿瘤体积、SUV值及MIB-1增殖指数、切除边缘状态、是否存在转移以及是否接受放疗/化疗。结果：NF1相关MPNSTs患者（平均年龄：29±13岁，范围8-54岁）较散发病例（平均年龄：45±13岁，范围24-67岁）更为年轻，且术前肿瘤体积显著更大（平均299 vs. 18 cm³，p=0.048）。肿瘤最常见于头面/颈区（34%，n=12），其次为躯干（31%，n=11）、下肢（17%，n=6）、上肢（14%，n=5）和椎管内（3%，n=1）。NF1相关MPNSTs主要发生于躯干（39%），而散发性肿瘤则多见于头面/颈区（50%）。66%的病例实现了完全切除，大多数病例功能得到改善或保持稳定（运动功能69%，感觉功能74%），疼痛强度亦有所减轻（63%）。与散发性肿瘤（中位VRS评分0.5）相比，NF1相关MPNSTs表现出更严重的疼痛评分（中位VRS评分2，p=0.002）。位于头面部/臂丛及上肢的散发性MPNSTs较下肢肿瘤表现出更好的术前功能。在12例可行[¹⁸F]FDG PET检查的病例中，术前平均SUV值（9.8±7.2）与平均最大MIB-1指数（34±26%，p=0.005）及平均术前肿瘤体积（474.7±68.6 cm³，p=0.047）呈正相关。肿瘤切除范围更广（R0切除，p=0.01）且无伴随转移（p=0.046）的患者总生存期显著更长；散发性MPNSTs患者的总生存期有延长趋势，但未达统计学显著性。在分别存在R1/R2切除边缘（6例）和转移（7例）的肿瘤中，采用单纯或联合新辅助/辅助放疗/化疗导致总生存期显著缩短（p=0.014）。结论：NF1相关MPNSTs具有更大的肿瘤体积、更高的SUV值和MIB-1增殖指数，且总生存期更短。尽管如此，手术仍可改善症状，特别是药物难治性疼痛，对于晚期疾病也应考虑手术以控制/改善症状。
肿瘤(癌症)患者之家