Kim Daniel H, Murovic Judith A, Tiel Robert L, Moes Gregory, Kline David G
Department of Neurosurgery, Stanford University Medical Center, Stanford, California 94305-5327, USA.
J Neurosurg. 2005 Feb;102(2):246-55. doi: 10.3171/jns.2005.102.2.0246.
This is a retrospective review of 397 benign and malignant peripheral neural sheath tumors (PNSTs) that were surgically treated between 1969 and 1999 at the Louisiana State University Health Sciences Center (LSUHSC). The surgical techniques and adjunctive treatments are presented, the tumors are classified with respect to type and prevalence at each neuroanatomical location, and the management of malignant PNSTs is reviewed.
There were 361 benign PNSTs (91%). One hundred forty-one benign lesions were brachial plexus tumors: 54 schwannomas (38%) and 87 neurofibromas (62%), of which 55 (63%) were solitary neurofibromas and 32 (37%) were neurofibromatosis Type 1 (NF1)-associated neurofibromas. Among the brachial plexus lesions supraclavicular tumors predominated with 37 (69%) of 54 schwannomas; 34 (62%) of 55 solitary neurofibromas; and 19 (59%) of 32 NF1-associated neurofibromas. One hundred ten upper-extremity benign PNSTs consisted of 32 schwannomas (29%) and 78 neurofibromas (71%), of which 45 (58%) were sporadic neurofibromas and 33 (42%) were NF1-associated neurofibromas. Twenty-five benign PNSTs were removed from the pelvic plexus. Lower-extremity PNSTs included 32 schwannomas (38%) and 53 neurofibromas (62%), of which 31 were solitary neurofibromas and 22 were NF1-associated neurofibromas. There were 36 malignant PNSTs: 28 neurogenic sarcomas and eight other sarcomas (fibro-, spindle cell, synovial, and perineurial sarcomas).
The majority of tumors were benign PNSTs from the brachial plexus region. Most of the benign PNSTs in all locations were neurofibromas, with sporadic neurofibromas predominating. Similar numbers of schwannomas were found in the upper and lower extremities, whereas neurofibromas were more prevalent in the upper extremities. Despite aggressive limb-ablation or limb-sparing surgery plus adjunctive therapy, malignant PNSTs continue to be associated with high morbidity and mortality rates.
这是一项对1969年至1999年在路易斯安那州立大学健康科学中心（LSUHSC）接受手术治疗的397例良性和恶性周围神经鞘瘤（PNSTs）的回顾性研究。介绍了手术技术和辅助治疗方法，根据类型和在每个神经解剖位置的患病率对肿瘤进行分类，并对恶性PNSTs的治疗进行了综述。
有361例良性PNSTs（91%）。141例良性病变为臂丛神经肿瘤：54例神经鞘瘤（38%）和87例神经纤维瘤（62%），其中55例（63%）为孤立性神经纤维瘤，32例（37%）为1型神经纤维瘤病（NF1）相关神经纤维瘤。在臂丛神经病变中，锁骨上肿瘤占主导，54例神经鞘瘤中有37例（69%）；55例孤立性神经纤维瘤中有34例（62%）；32例NF1相关神经纤维瘤中有19例（59%）。110例上肢良性PNSTs包括32例神经鞘瘤（29%）和78例神经纤维瘤（71%），其中45例（58%）为散发性神经纤维瘤，33例（42%）为NF1相关神经纤维瘤。25例良性PNSTs从盆腔神经丛切除。下肢PNSTs包括32例神经鞘瘤（38%）和53例神经纤维瘤（62%），其中31例为孤立性神经纤维瘤，22例为NF1相关神经纤维瘤。有36例恶性PNSTs：28例神经源性肉瘤和8例其他肉瘤（纤维肉瘤、梭形细胞肉瘤、滑膜肉瘤和神经束膜肉瘤）。
大多数肿瘤是来自臂丛神经区域的良性PNSTs。所有部位的大多数良性PNSTs是神经纤维瘤，以散发性神经纤维瘤为主。上肢和下肢发现的神经鞘瘤数量相似，而神经纤维瘤在上肢更为常见。尽管进行了积极的肢体切除或保肢手术加辅助治疗，但恶性PNSTs仍然与高发病率和死亡率相关。
