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A giant congenital fibrosarcoma of the leg
Cemil Ertürk1, Raci Yarmpapuç, Mehmet Akif Altay
1Faculty of Medicine, Department of Orthopaedic Surgery, Harran University Sanliurfa, Turkey. erturkc@yahoo.com
Insights
Congenital fibrosarcoma, a rare pediatric soft-tissue tumor, often recurs locally despite initial wide surgical excision. This case highlights the challenge of local recurrence, necessitating amputation in a young patient.
Area of Science:
- Pediatric Oncology
- Surgical Pathology
- Soft Tissue Tumors
Background:
- Congenital fibrosarcoma is a rare malignant soft-tissue neoplasm affecting infants and children.
- While generally having a more favorable clinical course than adult fibrosarcomas, local recurrence is a significant clinical challenge.
Purpose of the Study:
- To report a case of congenital fibrosarcoma in a pediatric patient.
- To illustrate the clinical presentation, management, and challenges associated with local recurrence of this rare tumor.
Main Methods:
- A case study of a 1-year-old girl with a large posterior right leg mass.
- Diagnosis confirmed via histological examination.
- Initial wide surgical excision followed by observation.
Main Results:
- Histological findings confirmed congenital fibrosarcoma.
- Despite initial wide excision, the tumor recurred locally after 1.5 years.
- Femoral supracondylar amputation was required due to tumor recurrence.
Conclusions:
- Congenital fibrosarcoma, though rare, poses a risk of local recurrence even after adequate initial resection.
- Aggressive local recurrence may necessitate further surgical intervention, such as amputation, in pediatric patients.
- Continued vigilance and potentially novel therapeutic strategies are warranted for managing recurrent congenital fibrosarcoma.
Abstract:
Congenital fibrosarcoma is a rare, malignant, soft-tissue tumor in infants and the pediatric age group. The clinical behavior of congenital fibrosarcoma is more favorable, and metastatic spread is rare, but local recurrence is a common problem. The patient was a 1-year-old girl at the time of operation, and there was a giant mass in the posterior region of the right leg. A wide surgical excision was performed and histological findings showed the diagnosis of congenital fibrosarcoma. After one and a half years, femoral supracondylar amputation was performed because of the recurrence of the local tumor.
