A case report of giant neurofibromatosis of maxillofacial, neck and chest was treated by multidisciplinary cooperation
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摘要: 1型神经纤维瘤病(neurofibromatosis type 1，NF1)是一种常染色体显性遗传性神经系统疾病。神经纤维瘤作为NF1中一种典型表现，体积可逐渐发展，当面积大于100 cm2时称为巨大NF1，是神经纤维鞘中施万细胞的瘤样增生。广东医科大学附属医院耳鼻咽喉中心收治了1例少见的颌面颈胸部巨大神经纤维瘤病患者，并在不同学科的协助下为其成功开展了手术治疗。Abstract: Neurofibromatosis Type 1 (NF1) is an autosomal dominant hereditary neurological disorder. One of the typical manifestations of NF1 is neurofibroma, which can develop gradually over time. When the volume exceeds 100 cm2, it is referred to as giant neurofibroma, representing a tumor-like proliferation of Schwann cells within the nerve fiber sheath. The Department of Otolaryngology at the Affiliated Hospital of Guangdong Medical University received a rare case involving a patient with giant neurofibromatosis affecting the maxillofacial region, neck, and chest. The patient underwent successful surgical treatment with the collaboration of various medical disciplines.
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Key words:
- neurofibromatosis type 1 /
- cutaneous neurofibroma /
- surgical treatment
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