Giant cervicothoracic malignant peripheral nerve sheath tumor in neurofibromatosis type I: a case report of integrated management with multimodal guidance.

Introduction: Neurofibromatosis type 1 (NF1) is a genetic disorder predisposing patients to malignant peripheral nerve sheath tumors (MPNSTs), which represent a leading cause of mortality with poor therapeutic outcomes. Large MPNSTs with NF1 are extremely rare, and only a few clinical reports are published on this disease. We describe a case of giant cervicothoracic MPNST with major vessels encased and present the clinical profiles, radiological findings, operative management, and follow-up data, along with a literature review. Case study: A 30-year-old male patient complained of an incidentally discovered painful mass in the right neck that had been progressively enlarging, accompanied by right hand weakness and intrinsic muscle atrophy. Contrast-enhanced MRI revealed a giant tumor closely related with the right brachial plexus and intervertebral foramina of C6-T2. The right subclavian artery and internal jugular vein were tightly encased by the tumor. A multidisciplinary approach incorporating advanced 3D imaging, preoperative vertebral artery embolization, and Conclusion: The successful long-term outcome provides a valuable template for managing similar high-risk cases, though long-term surveillance remains crucial due to the high recurrence potential of MPNSTs. This report contributes to the limited literature on radical surgical management of NF1-associated MPNSTs and emphasizes the critical balance between oncological control and functional preservation in these challenging cases.