title: Periosteal Ewing sarcoma family of tumors of the femur confirmed by molecular detection of EWS-FLI1 fusion gene transcripts: a case report and review of the literature.
abstract: Ewing sarcoma family of tumors (ESFT) comprise common sarcomas in children and young adults. Such tumors usually occur in the medullary cavity of long bones and directly invade cortical bone and circumferential soft tissue, including periosteum. Periosteal ESFT is extremely rare and only a small numbers of cases, including cases not confirmed by molecular detection of chimeric fusion gene, have been reported. We describe herein a case of periosteal ESFT of the femur in a 13-year-old boy harboring EWS-FLI1 chimeric fusion gene transcripts detected by reverse transcription-polymerase chain reaction analysis. This report may provide an opportunity for further evaluation of the EWS-FLI1 type 1 fusion gene and detection of prognostic significance in periosteal ESFT.
authors.affiliation: First Department of Pathology, Fukushima Medical University School of Medicine, Fukushima, Japan.
authors.forename: Michiyuki
authors.initials: M
authors.lastname: Hakozaki
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Hojo
authors.forename: Takahiro
authors.initials: T
authors.lastname: Tajino
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Yamada
authors.forename: Atsushi
authors.initials: A
authors.lastname: Kikuta
authors.forename: Masaki
authors.initials: M
authors.lastname: Ito
authors.forename: Hideki
authors.initials: H
authors.lastname: Sano
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Mochizuki
authors.forename: Mitsuko
authors.initials: M
authors.lastname: Akaihata
authors.forename: Shinichi
authors.initials: S
authors.lastname: Kikuchi
authors.forename: Masafumi
authors.initials: M
authors.lastname: Abe
mesh_terms: D000293:Adolescent; D000328:Adult; D001859:Bone Neoplasms / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D002648:Child; D003937:Diagnosis, Differential; D005260:Female; D005269:Femur / Q000473:pathology; D006801:Humans; D008297:Male; D015514:Oncogene Proteins, Fusion / Q000235:genetics*; D010521:Periosteum / Q000473:pathology; D011379:Prognosis; D051789:Proto-Oncogene Protein c-fli-1 / Q000235:genetics*; D034802:RNA-Binding Protein EWS; D012512:Sarcoma, Ewing / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D014158:Transcription, Genetic
chemical_list: C106031:EWS-FLI fusion protein; D015514:Oncogene Proteins, Fusion; D051789:Proto-Oncogene Protein c-fli-1; D034802:RNA-Binding Protein EWS