title: Successful treatment of a child with t(15;19)-positive tumor.
abstract: A subset of poorly differentiated carcinomas is characterized by the translocation t(15;19)(q13;p13), resulting in a BRD4/NUT fusion gene. Typically, this tumor affects children or young adults, with a predilection for midline head and neck or thoracic structures. The clinical course is invariably fatal, in spite of intensive chemotherapy and radiotherapy. We here present the successful treatment of a 10-year-old boy who presented with a BRD4/NUT-positive undifferentiated tumor in the iliac bone. The patient was selected for combined modality therapy, and has remained in complete continuous remission for close to 13 years. The findings show that t(15;19)-BRD4/NUT-positive tumors may arise in locations more typical for other pediatric tumors, such as Ewing sarcoma, and that they not always display epithelial differentiation. More importantly, our results also demonstrate that at least some patients with t(15;19)-positive tumors may be successfully treated.
authors.affiliation: Department of Clinical Genetics, Lund University Hospital, Lund, Sweden. fredrik.mertens@med.lu.se
authors.forename: Fredrik
authors.initials: F
authors.lastname: Mertens
authors.forename: Thomas
authors.initials: T
authors.lastname: Wiebe
authors.forename: Catharina
authors.initials: C
authors.lastname: Adlercreutz
authors.forename: Nils
authors.initials: N
authors.lastname: Mandahl
authors.forename: Christopher A
authors.initials: CA
authors.lastname: French
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage; D001859:Bone Neoplasms / Q000175:diagnosis / Q000235:genetics* / Q000628:therapy; D002648:Child; D002884:Chromosomes, Human, Pair 15 / Q000235:genetics*; D002888:Chromosomes, Human, Pair 19 / Q000235:genetics*; D003131:Combined Modality Therapy; D005500:Follow-Up Studies; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009687:Nuclear Proteins / Q000235:genetics*; D015514:Oncogene Proteins, Fusion / Q000235:genetics*; D012074:Remission Induction; D018228:Sarcoma, Small Cell / Q000175:diagnosis / Q000235:genetics* / Q000628:therapy; D014178:Translocation, Genetic / Q000235:genetics*; D016896:Treatment Outcome
chemical_list: C471990:BRD4-NUT fusion oncogene protein, human; D009687:Nuclear Proteins; D015514:Oncogene Proteins, Fusion