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Malignant melanotic nerve sheath tumors (MMNSTs) are rare, aggressive tumors with significant potential for distant metastasis. This case report describes an atypical presentation of MMNST in a middle-aged patient, arising in the carotid space rather than in the more commonly reported paraspinal regions. The report details clinical presentation, diagnostic evaluation, and the planned course of adjuvant radiation therapy after surgical excision. Comprehensive genomic profiling and multidisciplinary treatment planning were key components of management. Given the rarity of MMNST and its potential for recurrence and metastasis, vigilant, long-term follow-up is essential. This case underscores the importance of recognizing uncommon anatomical presentations that may pose unique diagnostic and therapeutic challenges.
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Ship et al. (2025) studied this question.
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