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.
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.
Hakozaki
Michiyuki
M
First Department of Pathology, Fukushima Medical University School of Medicine, Fukushima, Japan.
Hojo
Hiroshi
H
Tajino
Takahiro
T
Yamada
Hitoshi
H
Kikuta
Atsushi
A
Ito
Masaki
M
Sano
Hideki
H
Mochizuki
Kazuhiro
K
Akaihata
Mitsuko
M
Kikuchi
Shinichi
S
Abe
Masafumi
M
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
C106031:EWS-FLI fusion protein; D015514:Oncogene Proteins, Fusion; D051789:Proto-Oncogene Protein c-fli-1; D034802:RNA-Binding Protein EWS