CONGENITAL - INFANTILE FIBROSARCOMA; EVALUATION OF CLINICAL BAHAVIOUR AND OUTCOME

Document Type : Original Article

Authors

1 Pediatric Surgery Unit, Mansoura Faculty of Medicine

2 Pediatric Hematology Oncology Unit, Mansoura Faculty of Medicine

3 Pathology Department, Mansoura Faculty of Medicine

Abstract

Congenital - infantile fibrosarcoma is a relatively rare spindle cell tumour of the soft tissues occurring in children 
younger than two years of age. Most cases of this tumour involve the extremities or shoulder region and are often 
misdiagnosed as vascular malformations. For evaluation of clinical behaviour and outcome of congenital–infantile 
fibrosarcoma, 13 newborns and infants having this tumour have been treated surgically, either alone or combined with 
chemotherapy and or radiotherapy. Patients were followed up for a period up to 8.5 years. In the present series, wide surgical excision of congenital - infantile fibrosarcoma with pathologically proven safety margin was successful in 10 patients with no recurrence (77%). Preoperative chemotherapy was of benefit in cases of extended tumour size with possible neurovascular invasion so as to avoid heroic amputation surgery. Postoperative chemotherapy and radiotherapy were adjuvant tools in incompletely resected or irresectable tumours. So, wide surgical excision of congenital - infantile fibrosarcoma with pathologically proven safety margin is the treatment of choice. In addition the prognosis of this tumour is much more favourable than the adulthood type. 

Keywords

The Egyptian Journal of Surgery
Volume 22, Issue 2
April 2003
Pages 115-121

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