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Retroperitoneal infantile fibrosarcoma: clinical, molecular, and therapeutic aspects of an unusual tumor.

Abstract
The authors describe a patient with a large retroperitoneal infantile fibrosarcoma that responded well to preoperative chemotherapy, which subsequently facilitated the complete surgical resection of the mass. The patient had an unusual site of metastasis presumed to be to a regional lymph node. The histology on initial core biopsies was not classic but showed a round cell, myxoid pattern. The presence of the t(12;15)(p13;q25) and the fusion transcript ETV6-NTRK3 by RTPCR facilitated the diagnosis.
AuthorsRaveena Ramphal, David Manson, Sandra Viero, Maria Zielenska, Ted Gerstle, Alberto Pappo
JournalPediatric hematology and oncology (Pediatr Hematol Oncol) Vol. 20 Issue 8 Pg. 635-42 (Dec 2003) ISSN: 0888-0018 [Print] England
PMID14578034 (Publication Type: Case Reports, Journal Article)
Chemical References
  • ETV6-NTRK3 fusion protein, human
  • Oncogene Proteins, Fusion
Topics
  • Female
  • Fibrosarcoma (diagnosis, drug therapy, surgery)
  • Humans
  • Infant
  • Oncogene Proteins, Fusion (analysis, genetics)
  • Retroperitoneal Neoplasms (diagnosis, drug therapy, surgery)
  • Reverse Transcriptase Polymerase Chain Reaction (methods)
  • Translocation, Genetic

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