Pediatric Blood & Cancer · 2006 · 162 citations · 5 references
MedicineOncologyMolecular OncologyNeoplasiaPediatric OncologyEwing SarcomaMalignant NeoplasmsMalignant TumorsPediatric SarcomasPediatric TumorsClinical RemissionEwing Sarcoma Of BoneEwing Sarcoma TreatmentIliac Bone TumorsPediatric Ewing Sarcoma
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.
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BRD4-NUT fusion oncogene: a novel mechanism in aggressive carcinoma.
Christopher A. French, I Miyoshi, Ichiro Kubonishi et al. · PubMed · 2003 · 514 citations
Novel t(15;19)(q15;p13) chromosome abnormality in a thymic carcinoma.
Ichiro Kubonishi, N Takehara, J. Iwata et al. · PubMed · 1991 · 205 citations
Life Sciences, Genetics, Oncology +15