Nuclear protein in testis midline carcinoma of nasal cavity: a case report and review of the literature

Nuclear protein in testis (NUT) midline carcinoma (NMC) is an extremely rare, highly aggressive, and poorly differentiated malignant neoplasm that predominantly arises from the midline epithelial structures of the head and neck. It is driven by genetic rearrangements of the NUTM1 gene, with the most common aberration being a translocation that forms a fusion gene with BRD4 on chromosome 19p13.1. The diagnosis of NMC is challenging due to its non-specific clinical and histological features, and it typically requires a combination of immunohistochemical detection of NUT protein and molecular assays to confirm NUTM1 gene rearrangement. The prognosis of NMC is dismal, with a median overall survival of less than one year. To date, no standard treatment regimen has been established, and only individualized therapeutic strategies have been reported in the literature. Herein, we present a case of a 40-year-old female patient with NMC of the left nasal cavity who presented with nasal mass, nasal obstruction, rhinorrhea, mild epistaxis, and hyposmia. The patient was diagnosed with NMC based on pathological and immunohistochemical findings, and was staged as T2N0M0. She received multidisciplinary treatment including neoadjuvant chemotherapy, radical surgery, adjuvant chemoradiotherapy, and subsequent immune checkpoint inhibitor (ICI) maintenance therapy. Up to the latest follow-up in February 2026, the patient had achieved complete remission with no evidence of tumor recurrence. We conclude that comprehensive and intensive first-line multidisciplinary therapy is crucial for improving clinical outcomes in patients with NMC, a disease with inherently high risks of recurrence and progression and a poor natural prognosis.