title: Primary paraesophageal Ewing's sarcoma: an uncommon case report and literature review.
abstract: Ewing's sarcoma is a rare and highly aggressive cancer most frequently arising in people under 20 years of age. We report an uncommon case of primary paraesophageal Ewing's sarcoma in a 25-year-old male harboring the infrequent EWSR1/ERG fusion transcript with multiple splice variants coexisting in the same tumor. The patient was totally refractory to chemotherapy and died 17 months after diagnosis. We underscore the need for better understanding of the molecular pathogenesis of the disease and improved systemic therapy options.
authors.affiliation: Department of Medicine, GI and Lymphoma Unit, The Royal Marsden NHS Foundation Trust, London and Surrey, UK.
authors.forename: Noelia
authors.initials: N
authors.lastname: Tarazona
authors.affiliation: Department of Pathology, University of Valencia, Valencia, Spain.
authors.forename: Lara
authors.initials: L
authors.lastname: Navarro
authors.affiliation: Department of Hematology and Medical Oncology, Biomedical Research Institute, INCLIVA, University of Valencia, Valencia, Spain.
authors.forename: Juan Miguel
authors.initials: JM
authors.lastname: Cejalvo
authors.affiliation: Department of Hematology and Medical Oncology, Biomedical Research Institute, INCLIVA, University of Valencia, Valencia, Spain.
authors.forename: Valentina
authors.initials: V
authors.lastname: Gambardella
authors.affiliation: Department of Hematology and Medical Oncology, Biomedical Research Institute, INCLIVA, University of Valencia, Valencia, Spain.
authors.forename: J Alejandro
authors.initials: JA
authors.lastname: Pérez-Fidalgo
authors.affiliation: Department of Pathology, University of Valencia, Valencia, Spain.
authors.forename: Alejo
authors.initials: A
authors.lastname: Sempere
authors.affiliation: Department of Pathology, University of Valencia, Valencia, Spain.
authors.forename: Samuel
authors.initials: S
authors.lastname: Navarro
authors.affiliation: Department of Hematology and Medical Oncology, Biomedical Research Institute, INCLIVA, University of Valencia, Valencia, Spain.
authors.forename: Andrés
authors.initials: A
authors.lastname: Cervantes
keywords: Ewing’s sarcoma; fusion genes; immunohistochemistry; recurrence