Metastatic NUT Carcinoma in a Child

Nuclear protein of the testis (NUT) carcinoma is a rare and aggressive malignancy associated with rearrangements of the NUT gene on chromosome 15q14 .This entity is often under diagnosed and there is limited literature regarding its biology and optimal management. We report a case of pediatric NUT carcinoma presenting as a mass involving the nasal septum with associated bilateral cervical lymphadenopathy. CT and MRI of PNS revealed soft tissue lesion involving cartilaginous and bony septum and tip of nose .Histopathology of cervical lymph node and IHC with NUT confirmed the diagnosis of metastatic NUT carcinoma. NUT carcinoma has a dismal prognosis despite aggressive multimodality management. As new NUT targeting drugs using BET inhibitors are emerging, the correct and prompt recognition of NC is the key to improve patient outcome.