First evidence of treatment efficacy in metastatic carcinoma of the parotid gland with BRD4/NUT translocation

Vulsteke, Christof; Lurquin, Eveline; Debiec-Rychter, Maria; Gheysens, Olivier; Nuyts, Sandra; Schoenaers, Joseph; Politis, Constantinus; Mebis, Jeroen; Hauben, Esther; Clement, Paul

doi:10.1179/1973947815Y.0000000046

First evidence of treatment efficacy in metastatic carcinoma of the parotid gland with BRD4/NUT translocation

Author:

Vulsteke, Christof

Lurquin, Eveline ; Debiec-Rychter, Maria ; Gheysens, Olivier ; Nuyts, Sandra ; Schoenaers, Joseph ; Politis, Constantinus ; Mebis, Jeroen ; Hauben, Esther ; Clement, Paul

Keywords:

Science & Technology, Life Sciences & Biomedicine, Oncology, Infectious Diseases, Pathology, Pharmacology & Pharmacy, Nut midline carcinoma, Chemotherapy, BRD4/NUT fusion gene, NUT MIDLINE CARCINOMA, REARRANGEMENT, DIFFERENTIATION, TUMOR, Adult, Antineoplastic Combined Chemotherapy Protocols, Bone Neoplasms, Carcinoma, Cisplatin, Doxorubicin, Humans, Ifosfamide, In Situ Hybridization, Fluorescence, Male, Nuclear Proteins, Oncogene Proteins, Fusion, Parotid Neoplasms, Radiotherapy, Treatment Outcome, 1115 Pharmacology and Pharmaceutical Sciences, Microbiology, 3202 Clinical sciences, 3214 Pharmacology and pharmaceutical sciences

Abstract:

Nuclear protein in testis (NUT) midline carcinomas (NMC) are characterized by rearrangements of the gene NUT. In the majority of NMCs, a translocation t(15;19), resulting in a BRD4/NUT fusion gene, is present. Nuclear protein in testis midline carcinomas is a rare, but probably underdiagnosed entity due to misdiagnosis. Most cases have been reported in the mediastinum and upper aero-digestive tract. The clinical course of a NMC is extremely aggressive, in spite of intensive chemotherapy and radiotherapy, with an average survival < / 1 year.