Infantile fibrosarcoma: retrospective analysis of eleven patients

Tumori. 2011 Mar-Apr;97(2):166-9. doi: 10.1177/030089161109700206.

Abstract

Aims and background: Infantile fibrosarcomas are soft tissue sarcomas that are diagnosed at or soon after birth. In the present study, we retrospectively evaluated clinical characteristics, treatment modalities and outcome of patients diagnosed with infantile fibrosarcoma at our institution.

Methods: A retrospective review was conducted to evaluate demographic characteristics, presenting features, type and timing of surgery, other treatment modalities and survival characteristics.

Results: Nine males and 2 females were diagnosed with infantile fibrosarcoma between 1970-2008. The initial surgical procedure was subtotal resection in 4 patients, gross-total resection in 3 and biopsy in 4. Neoadjuvant chemotherapy was given to 10 patients. Three patients died, one for the disease and 2 from complications of therapy. Eight patients are under follow-up with no evidence of disease for 1.3 to 13.5 years. None of the patients in the series underwent amputation.

Conclusions: Owing to the chemosensitive nature of the tumor and possibility of spontaneous regression, neoadjuvant chemotherapy should be considered to prevent extensive or mutilating surgery.

MeSH terms

  • Antineoplastic Combined Chemotherapy Protocols / administration & dosage
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use*
  • Chemotherapy, Adjuvant
  • Child, Preschool
  • Cyclophosphamide / administration & dosage
  • Dactinomycin / administration & dosage
  • Doxorubicin / administration & dosage
  • Etoposide / administration & dosage
  • Female
  • Fibrosarcoma / diagnosis*
  • Fibrosarcoma / drug therapy
  • Fibrosarcoma / mortality
  • Fibrosarcoma / pathology
  • Fibrosarcoma / surgery
  • Fibrosarcoma / therapy*
  • Humans
  • Infant
  • Infant, Newborn
  • Male
  • Neoadjuvant Therapy / methods*
  • Neoplasm Regression, Spontaneous
  • Radiotherapy, Adjuvant
  • Retrospective Studies
  • Treatment Outcome
  • Vincristine / administration & dosage

Substances

  • Dactinomycin
  • Vincristine
  • Etoposide
  • Doxorubicin
  • Cyclophosphamide

Supplementary concepts

  • CAV protocol
  • VAC protocol