Rapidly Growing Malignant Peripheral Nerve Sheath Tumors Arising From Neurofibromatosis Type 1: A Case Report by Rehabilitation Physicians

由1型神经纤维瘤病引起的快速生长恶性周围神经鞘瘤:康复医师的病例报告

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Abstract

We describe a case of a 40-year-old Japanese woman with rapidly growing malignant peripheral nerve sheath tumors (MPNSTs) arising from neurofibromatosis type 1 (NF-1). The patient presented numbness in both legs, back pain, and gait disturbances. Magnetic resonance imaging (MRI) revealed a spinal tumor at the thoracic level. To resolve her symptoms, a laminectomy and intradural tumor resection were performed. The tumor was diagnosed as a neurofibroma with no malignant characteristics. After the surgery, she participated in a rehabilitation program aimed at promoting independence in daily activities and enhancing muscle strength. Initially, her walking ability showed improvement; however, she soon experienced complications, including challenges with bowel movements and a gradual decline in her walking function. A follow-up MRI on the 67th day post-surgery showed tumor regrowth that necessitated reoperation. After the surgery, the neurological symptoms improved temporarily, but they worsened again, ultimately leading to a shift to palliative care and her demise several days later. This case underscores the challenges in pathological diagnosis and the aggressive nature of MPNSTs, emphasizing the need for vigilant monitoring and timely intervention. Despite initial surgical success, rapid tumor growth can occur during rehabilitation, highlighting the importance of a multidisciplinary approach for accurate diagnosis and treatment. Early detection of tumor progression through meticulous neurological monitoring and prompt surgical consultation are critical for optimal outcomes. Further research into more definitive diagnostic tools and effective treatment strategies for MPNSTs is crucial to improve patient care.

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