小儿上臂丛状神经纤维瘤的临床分析(附3例报告)
Plexiform Neurofibromas in the Upper Arm of Pediatric Patients: A Report on Three Cases
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摘要:目的 探讨小儿上臂丛状神经纤维的病理、临床表现及临床诊治。方法 结合相关文献对郑州大学第一附属医院小儿外科2008年4月到2012年3月收治的3例上臂丛状神经纤维瘤患儿的临床资料进行回顾性分析。结果 本病是神经干及其分支的弥漫性神经纤维瘤, 分为结节型、弥漫型, 是神经纤维瘤病Ⅰ型(NF1)的主要病变之一, 常伴有家族史, 有恶变倾向, 本组3例均为结节型, 无恶变, 术后3例患儿上肢均有不同程度功能障碍, 1例有家族史, 1例术后复发。结论 小儿上臂丛状神经纤维瘤临床上比较少见, 诊断困难, 易与丛状神经鞘瘤混淆, 彩超、CT、MRI等影像学检查有助于术前诊断, 病理结果为唯一确诊方法, 手术为其主要治疗方法, 由于其侵袭性的生长特点和生长位置的特殊, 术后易复发, 常引起上肢功能障碍, 新的治疗方法有待实施。Abstract:Objective To discuss the pathology, clinical manifestations, diagnosis, and treatment of plexiform neurofibromas in the upper arm of pediatric patients.Methods We analyzed three cases of the plexiform neurofibromas in the upper arm of pediatric patients by reviewing related literature.Results Plexiform neurofibromas are peripheral nerve sheath tumors associated with neurofibromatosis type 1.Two types ofplexiform neurofibromas have been recognized, diffuse type and nodular type. The disease is often associated with a family history of the disease. The risk of malignancy is 5% to 10%. This report includes three cases of nodular plexiform neurofibromas. All patients had functional disturbances after their surgical operations. In one of the case, the patient had family history of neurofibroma and one case reported recurrence of the disease.Conclusion Diagnosis of the plexiform neurofibromas in the upper arm of pediatric patients is difficult because they are easily confused with plexiform schwannomas. Imaging using B ultrasound, CT, and MRI would provide helpful information for diagnosing plexiform neurofibromas; however, histopathology is the only definitive diagnostic method. Surgical resection remains the main treatment. However, functional disturbances are almost inevitable. The location and infiltrative nature of plexiform neurofibromas can preclude complete resection. Therefore, new treatment needs to be imnlemented.
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