Pinheiro Fernando Santos, Rothner A David, Moodley Manikum, Zahka Kenneth G
Department of Neurology, Cleveland Clinic, Cleveland, OH, USA
Pediatric Neurology, Cleveland Clinic Foundation, Cleveland, OH, USA.
J Child Neurol. 2015 Oct;30(11):1537-43. doi: 10.1177/0883073815571635. Epub 2015 Feb 18.
The authors review the literature on massive soft tissue neurofibroma. The methods included a review of 71 reports (PubMed search 1929-2012) with a total of 91 massive soft tissue neurofibroma patients and illustration of clinical and radiological progression of massive soft tissue neurofibroma on a patient with neurofibromatosis type 1. The mean age at initial examination was 21 years. Tumor onset was mostly in childhood years. The commonest affected body segment was the lower extremity (46%), followed by head/neck (30%). Surgical management was pursued in the majority of cases (79%). Bleeding was a common complication (25%). Recurrence was described in 12%; multiple resections cases were described. Malignant transformation occurred in 5%. Although massive soft tissue neurofibroma may be present early in life, massive tumor overgrowth may take years. Predicting disease progression and/or benefit of surgical intervention early in the disease course is challenging. Recurrence and malignant transformation are possible. Massive soft tissue neurofibroma does not respond to chemotherapy or radiotherapy and is associated with life-threatening surgical complications.
作者回顾了关于巨大软组织神经纤维瘤的文献。方法包括对71篇报告（通过1929年至2012年的PubMed检索）进行回顾，这些报告共涉及91例巨大软组织神经纤维瘤患者，并对1例1型神经纤维瘤病患者的巨大软组织神经纤维瘤的临床和影像学进展进行了说明。初次检查时的平均年龄为21岁。肿瘤大多在儿童期发病。最常受累的身体部位是下肢（46%），其次是头颈部（30%）。大多数病例（79%）采取了手术治疗。出血是常见并发症（25%）。12%的病例出现复发；有多次切除病例的描述。5%发生了恶性转化。虽然巨大软组织神经纤维瘤可能在生命早期就出现，但巨大肿瘤的过度生长可能需要数年时间。在疾病病程早期预测疾病进展和/或手术干预的益处具有挑战性。复发和恶性转化都有可能发生。巨大软组织神经纤维瘤对化疗或放疗无反应，且与危及生命的手术并发症相关。
