Introduction: NUT carcinoma is a highly aggressive subtype of squamous cell carcinoma characterized by rearrangements and translocations involving the NUTM1 gene on chromosome 15q14. It primarily occurs along the midline structures of the body, such as the head, neck, and thorax. The disease carries a poor prognosis, with a median survival of approximately nine months. To date, no specific treatment protocol has demonstrated consistent efficacy in the management of NUT carcinoma. CasePresentation: We report the case of an adolescent patient presenting with a partially resected lesion in the nasal cavity. Histopathological examination confirmed the diagnosis of NUT carcinoma. The patient underwent a rhinectomy with supraomohyoid lymph node dissection and simultaneous nasal reconstruction. Postoperatively, the patient received cisplatin-based chemotherapy and adjuvant radiotherapy targeting the surgical bed and cervical region. Conclusion: This case emphasizes the critical need to recognize and understand this rare and highly aggressive malignancy, for which surgical resection remains the preferred treatment when feasible. It also underscores the importance of ongoing research to establish standardized treatment approaches.
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