关键词: Hamartoma Hamartome Hand Main Median nerve Nerf Nerf médian Nerve Neurofibrome plexiforme Plexiform neurofibroma Tumeur Tumor

Mesh : Male Humans Middle Aged Child Neurofibroma, Plexiform / diagnostic imaging surgery Median Nerve / surgery Hamartoma Carpal Tunnel Syndrome / surgery Peripheral Nervous System Neoplasms / diagnostic imaging surgery pathology Upper Extremity / surgery pathology

来  源:   DOI:10.1016/j.hansur.2023.08.007

Abstract:
Plexiform neurofibroma is a benign peripheral nerve-sheath tumor, rarely involving major nerves of the extremities. In the literature, there are no clear treatment strategies for plexiform neurofibroma of major peripheral nerves. Our experience encountered two patients with plexiform neurofibroma of the median nerve, presenting with a palmar mass and symptoms of carpal tunnel compression. Preoperatively, plexiform neurofibroma was diagnosed on MRI and clinical examination. Both patients also experienced significant neurological deterioration, with finger numbness and increased nerve/tumor size. Potential malignant transformation was also considered. For these reasons, resection of the involved area of the nerve and repair were indicated. In both patients, intraoperative pathological diagnosis was plexiform neurofibroma. The 45-year-old male patient refused further surgery after carpal tunnel release, which was performed under axillary block. One year postoperatively, nerve compression symptoms decreased moderately. In the other patient, a 7-year-old boy, a significantly enlarged area of the median nerve was resected, and neurorrhaphy was performed. One year postoperatively, median nerve motor-sensory functions recovered completely. Four years postoperatively, no enlargement of the residual tumor was observed.
摘要:
丛状神经纤维瘤是一种良性外周神经鞘肿瘤,很少涉及四肢的主要神经。在文学中,对于主要周围神经丛状神经纤维瘤,目前尚无明确的治疗策略。我们的经验遇到了两名正中神经丛状神经纤维瘤患者,表现为手掌肿块和腕管压迫症状。术前,MRI和临床检查均诊断为丛状神经纤维瘤。两名患者也经历了显著的神经系统恶化,手指麻木和神经/肿瘤大小增加。还考虑了潜在的恶性转化。由于这些原因,涉及的神经区域的切除和修复被认为是。在这两个病人中,术中病理诊断为丛状神经纤维瘤。这位45岁的男性患者在腕管松解术后拒绝进一步手术,在腋窝阻滞下进行。术后一年,神经压迫症状中度减轻。在另一个病人身上,一个7岁的男孩,切除了一个明显扩大的正中神经区域,并进行了神经吻合术。术后一年,正中神经运动感觉功能完全恢复。术后四年,未观察到残余肿瘤的扩大。
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