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Published on: October 25, 2011
Unusual case of congenital/infantile fibrosarcoma in a new born
Eddiba Tarik1, Rouas Lamiae, Amrani Abdelouahed
1Department of Pediatric Orthopedic and Traumatology, Children's Hospital of Rabat and the Unity of Teaching and Research in Pediatric Surgery, Rabat, Morocco.
Insights
Congenital infantile fibrosarcoma (CIFS) is a rare pediatric tumor. This case highlights an unusual neonatal presentation mimicking hemangioma, successfully treated with amputation after chemotherapy failure.
Area of Science:
- Pediatric Oncology
- Surgical Pathology
- Neonatal Medicine
Background:
- Congenital infantile fibrosarcoma (CIFS) is a rare soft tissue tumor predominantly affecting infants under five years old.
- CIFS typically presents in distal extremities but can occur in unusual locations like the retroperitoneum or lungs.
- While generally having a good prognosis, CIFS in newborns is exceptionally rare.
Observation:
- A case of CIFS in a neonate presented atypically, mimicking a hemangioma and causing significant neonatal hemorrhage.
- The tumor was located in the left arm and axilla and was associated with a congenital hand malformation.
- Initial chemotherapy treatment for the infant failed to resolve the tumor.
Findings:
- Despite chemotherapy failure, the infant underwent a shoulder amputation for tumor removal.
- The infant is currently two years old and shows no signs of tumor recurrence.
- This case underscores the importance of considering rare diagnoses in neonatal presentations.
Implications:
- This case expands the known clinical spectrum of congenital infantile fibrosarcoma.
- It highlights the challenges in diagnosing and managing rare neonatal tumors.
- Early and aggressive surgical intervention may be crucial for favorable outcomes in complex CIFS cases.
Abstract:
Congenital infantile fibrosarcoma (CIFS) is a rare mesenchymal tumor that is primarily developed in the soft tissue of distal extremities and occasionally in unusual locations such as the lung and retroperitoneum. It occurs mainly in children below the age of 5 years. About 200 cases have been reported in the literature so far, very few of them in new-borns. The prognosis of this tumor is relatively good compared to adult forms. We report an unusual case of CIFS occurring in new-born mimicking an hemangioma and causing hemorrhage in the neonatal period. The tumor is located in the left arm and axilla and associated with a hand malformation. A shoulder amputation is performed after chemotherapy failure. The infant is now two-years old with no recurrence.
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