title: Treatment of infantile fibrosarcoma with chemotherapy and surgery: results from the Dana-Farber Cancer Institute and Children's Hospital, Boston.
abstract: OBJECTIVE
  To retrospectively evaluate the treatment and outcome of patients diagnosed with infantile fibrosarcoma at the Dana-Farber Cancer Institute and Children's Hospital, Boston.
  
  
  METHODS
  Between 1982 and 1998, a total of 11 infants were diagnosed pathologically with infantile fibrosarcoma. A retrospective chart review was conducted to determine the extent of surgical therapy and chemotherapy required for a favorable clinical outcome. Cytogenetic findings were reviewed and archived tumor specimens were analyzed, when available, for the presence of the TEL/TRKC fusion gene.
  
  
  RESULTS
  Three patients had primary surgical resection with negative pathologic margins and have been lost to follow-up. Two patients received chemotherapy only after limited biopsy or subtotal resection and are alive with no evidence of disease 8 and 18 years from diagnosis. Four patients had limited biopsies followed by chemotherapy with delayed resection. One of these four patients had negative margins and received no further chemotherapy. The other three of these patients had positive microscopic margins; two of them received postoperative chemotherapy while the third did not. All four are currently alive with no evidence of disease. Two patients had progressive disease within 7 and 10 months of diagnosis while on chemotherapy after subtotal resections. One of these two patients is dead of disease; the other is alive after palliative radiotherapy. Seven patients had archived or frozen tissue available for molecular analysis. All seven had evidence of TEL gene rearrangement; six exhibited the TEL/TRKC fusion. Six patients had characteristic trisomies previously reported to be associated with infantile fibrosarcoma.
  
  
  CONCLUSIONS
  Previously reported series of treatment outcomes in infantile fibrosarcoma have been limited to very few patients due to the rare occurrence of this tumor. In our experience, initial chemotherapy combined with surgery has been successful for most cases. When disease progression occurred, it was within one year of diagnosis. There was no development of distant metastases in the patients with progressive disease. The role of additional chemotherapy for microscopic margins after local control is not clear. We found a high incidence of the TEL/TRKC fusion gene, confirming its utility in diagnosis. We propose a uniform approach to treatment to gather clinical and biologic information about this rare and curable disease.
authors.affiliation: Department of Pediatric Hematology-Oncology, University of California-San Francisco, San Francisco, CA 94143-0519, USA. lohm@itsa.ucsf.edu
authors.forename: Mignon L
authors.initials: ML
authors.lastname: Loh
authors.forename: Peter
authors.initials: P
authors.lastname: Ahn
authors.forename: Antonio R
authors.initials: AR
authors.lastname: Perez-Atayde
authors.forename: Mark C
authors.initials: MC
authors.lastname: Gebhardt
authors.forename: Robert C
authors.initials: RC
authors.lastname: Shamberger
authors.forename: Holcombe E
authors.initials: HE
authors.lastname: Grier
mesh_terms: D001900:Boston; D002648:Child; D003131:Combined Modality Therapy; D018450:Disease Progression; D018572:Disease-Free Survival; D005260:Female; D005354:Fibrosarcoma / Q000188:drug therapy* / Q000601:surgery*; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D012042:Registries; D012189:Retrospective Studies; D013997:Time Factors