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左上肢罕见巨大神经纤维瘤1例

One case report of a rare giant neurofibroma in the left upper extremity
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摘要 患者男,51岁,因"左上肢及全身多处皮下肿物50余年"收入湖北医药院附属人民医院,诊断为神经纤维瘤病Ⅰ型(NF1)合并左上肢罕见巨大神经纤维瘤。患者自幼出现皮下肿物及咖啡斑,左上肢瘤体随年龄增长而增大,近年增长迅速,达56 cm×38 cm,伴出血、破溃。术前MRI显示左上肢软组织包块,结合家族史及临床表现符合NF1诊断标准。行分次手术切除瘤体,总重10.204 kg,术中采用自体血回输以避免大量输血相关资源消耗,剥离创面周围形成皮瓣,减轻张力后直接缝合。术后病理证实为神经纤维瘤。术后患者恢复良好,左上肢血运正常,皮瓣存活。术后2个月随访,患者左上肢功能改善,生活质量显著提升。 A 51-year-old male patient was admitted to People’s Hospital,Hubei University of Medicine due to multiple subcutaneous masses in the left upper limb and other body regions for over 50 years and was diagnosed as neurofibromatosis typeⅠ(NF1)combined with a rare giant neurofibroma in the left upper limb.The patient had developed subcutaneous masses and café-au-lait spots since childhood,with the left upper limb mass rapidly enlarged to 56 cm×38 cm over time,accompanied by hemorrhage and ulceration.Preoperative MRI revealed a soft tissue mass in the left upper limb.NF1 was diagnosed based on family history and clinical manifestations.Tumor resection was performed in stages,and the tumor tissue weighed 10.204 kg.During the operation,autologous blood reinfusion was adopted to avoid the consumption of a large amount of resources related to blood transfusion.The area around the wound was stripped to reduce the tension.The flap around the wound was sutured directly to close the wound.Neurofibroma was confirmed by postoperative pathology.The patient recovered well postoperatively,with normal blood supply to the left upper limb,survival of the skin flap,and significant functional improvement at the 2-month follow-up,leading to enhanced quality of life.
作者 李欣临 洪城 李少茹 张赛圣 Li Xinlin;Hong Cheng;Li Shaoru;Zhang Saisheng(Department of Burns and Plastic Surgery,People’s Hospital,Hubei University of Medicine,Shiyan 442000,China)
出处 《中华整形外科杂志》 北大核心 2025年第7期705-709,共5页 Chinese Journal of Plastic Surgery
关键词 神经纤维瘤病 巨大神经纤维瘤 手术治疗 Neurofibromatoses Giant neurofibroma Surgical resection
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