European Journal of Pediatrics

, Volume 155, Issue 5, pp 373–376

Mobilization of tumour cells during biopsy in an infant with Ewing sarcoma

Authors

  • A. Zoubek
    • Children's Cancer Research InstituteSt. Anna Children's Hospital
  • H. Kovar
    • Children's Cancer Research InstituteSt. Anna Children's Hospital
  • M. Kronberger
    • Children's Cancer Research InstituteSt. Anna Children's Hospital
  • G. Amann
    • Institute of Clinical PathologyUniversity of Vienna
  • R. Windhager
    • Department of OrthopaedicsUniversity of Vienna
  • B. Gruber
    • Children's Cancer Research InstituteSt. Anna Children's Hospital
  • H. Gadner
    • Children's Cancer Research InstituteSt. Anna Children's Hospital
Hematology/Oncology

DOI: 10.1007/BF01955264

Cite this article as:
Zoubek, A., Kovar, H., Kronberger, M. et al. Eur J Pediatr (1996) 155: 373. doi:10.1007/BF01955264
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Abstract

Ewing sarcoma and the closely relate peripheral primitive neuroectodermal tumour, recently referred to as Ewing tumour (ET), are characterised by unique gene rearrangements on chromosome 22. The breakpoints have been cloned and shown to fuse the Ewing sarcoma gene to one of two closely related ETS proto-oncogens, FLI-1 or ERG, which reside on chromosomes 11 and 21, respectively. The rearrangement results in the expression of specific hybrid transcripts which can be detected with high sensitivity by the reverse transcriptase polymerase chain reaction technique (RT-PCR) in primary tumours, blood and bone marrow. We report on a 7-month-old boy with a pelvic Ewing sarcoma in whom circulating tumour cells were identified in the peripheral blood during open tumour biopsy by RT-PCR. However, before and 6 days after surgery no tumour cells could be detected in the peripheral blood.

Conclusion

The application of RTPCR to monito shedding of tumour cells during surgical intervention will nelp to evaluate if open biopsy potentially contributes to metastatic tumour cell spread.

Key words

Ewing tumoursEWS-FLI-1 fusion transcriptsCirculating tumour cellsTumour biopsyMinimal metastatic disease

Abbreviations

cDNA

complementary desoxyribonucleic acid

ET

Ewing tumours

ETS E26

avian erythroblastosis virus transformation-specific oncogen

EWS gene

Ewing sarcoma gene

FLI-1 gene

Friend leukaemia virus integration site 1 gene

M1C2/CD99

cell surface sialoglycoprotein

mRNA

messanger ribonucleic acid

RT-PCR

reverse transcriptasepolymerase chain reaction

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Copyright information

© Springer-Verlag 1996