| Summary: | Background: Nuclear protein of the testis (NUT) carcinoma (NC) is a rare type of poorly differentiated carcinoma. The median overall survival is 6.7–9.5 months. Surgery and radiotherapy are the main modalities of treatment. The efficacy of chemotherapy is often overlooked. Case report: A 7-year-old pediatric patient was diagnosed with recurrent NC of the submandibular gland, an uncommon site of NC. The tumor split and contracted following chemotherapy with cyclophosphamide, pirarubicin, cisplatin, ifosfamide, and etoposide. Subsequently, surgery was performed to excise the tumor with negative margins. Three months after the end of the multimodality treatment, the imaging did not show signs of recurrence or metastasis. Conclusion: The chemotherapy regimen of cyclophosphamide, pirarubicin, cisplatin, ifosfamide, and etoposide may be a new attempt to treat NC.
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