90例1型神经纤维瘤病患者臀部、腿部和足部周围神经鞘瘤的手术治疗
Surgery for Peripheral Nerve Sheath Tumours of the Buttocks, Legs and Feet in 90 Patients With Neurofibromatosis Type 1.
作者信息
Friedrich Reinhard E, Tuzcu Caglayan T
机构信息
Department of Oral and Craniomaxillofacial Surgery, Eppendorf University Hospital, University of Hamburg, Hamburg, Germany
Department of Oral and Craniomaxillofacial Surgery, Eppendorf University Hospital, University of Hamburg, Hamburg, Germany.
出版信息
In Vivo. 2021 Mar-Apr;35(2):889-905. doi: 10.21873/invivo.12329.
BACKGROUND/AIM: Neurofibromatosis type 1 (NF1) is an autosomal dominant tumour predisposition syndrome that can cause plexiform neurofibromas (PNFs). This study examines the surgical procedures that have been performed on large PNFs of the lower extremities.
PATIENTS AND METHODS
Surgical procedures on the lower extremity performed on 90 patients with NF1 with PNFs were evaluated. The topography of the tumours was classified according to dermatomes and functional units.
RESULTS
A total of 243 surgical interventions on the regions of interest were performed. Neurological complications were rarely noted and usually occurred temporarily. There was no preference for dermatomes affected by PNF. The proportion of patients with malignant peripheral nerve sheath tumours (MPNSTs) in this group was 4/90 (4.4%).
CONCLUSION
PNFs often require repeated local interventions to achieve the treatment goal. Local tumour recurrences are to be expected even after extensive tumour reduction. Rapid tumour growth combined with new pain sensations can be signs of a MPNST.
背景/目的：1型神经纤维瘤病（NF1）是一种常染色体显性肿瘤易感综合征，可导致丛状神经纤维瘤（PNF）。本研究探讨了对下肢大型PNF所施行的外科手术。
患者与方法
对90例患有PNF的NF1患者下肢进行的外科手术进行评估。根据皮节和功能单位对肿瘤的部位进行分类。
结果
对感兴趣区域共进行了243次外科手术干预。很少注意到神经并发症，且通常为暂时性的。PNF对皮节无偏好。该组中恶性外周神经鞘瘤（MPNST）患者的比例为4/90（4.4%）。
结论
PNF通常需要反复进行局部干预以实现治疗目标。即使在肿瘤大量切除后，局部肿瘤复发仍可预期。肿瘤快速生长并伴有新的疼痛感可能是MPNST的迹象。
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