Successful treatment of a child with t(15;19)-positive tumor.
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.
Mertens
Fredrik
F
Department of Clinical Genetics, Lund University Hospital, Lund, Sweden. fredrik.mertens@med.lu.se
Wiebe
Thomas
T
Adlercreutz
Catharina
C
Mandahl
Nils
N
French
Christopher A
CA
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
C471990:BRD4-NUT fusion oncogene protein, human; D009687:Nuclear Proteins; D015514:Oncogene Proteins, Fusion