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Infantile fibrosarcoma: retrospective analysis of eleven patients
Canan Akyüz1, Serhan Küpeli, Ali Varan
1Hacettepe University, Institute of Oncology, Department of Pediatric Oncology, Ankara, Turkey.
Insights
Neoadjuvant chemotherapy is recommended for infantile fibrosarcomas, a type of congenital soft tissue sarcoma. This approach can prevent extensive surgery due to the tumor's sensitivity to chemotherapy and potential for spontaneous regression.
Area of Science:
- Pediatric Oncology
- Surgical Oncology
- Pediatric Pathology
Background:
- Infantile fibrosarcomas are congenital soft tissue sarcomas presenting at birth.
- These tumors require careful management to balance oncologic control and functional outcomes in infants.
Purpose of the Study:
- To retrospectively evaluate the clinical characteristics, treatment modalities, and outcomes of infantile fibrosarcoma.
- To assess the effectiveness of neoadjuvant chemotherapy in managing infantile fibrosarcoma.
Main Methods:
- Retrospective review of patients diagnosed with infantile fibrosarcoma between 1970-2008.
- Analysis of demographic data, presenting features, surgical interventions, chemotherapy, and survival rates.
Main Results:
- Eleven patients (9 males, 2 females) were analyzed.
- Most patients received neoadjuvant chemotherapy; 8 achieved no evidence of disease, while 3 died (1 from disease, 2 from therapy complications).
- No patients underwent amputation, and extensive surgery was avoided in most cases.
Conclusions:
- Infantile fibrosarcomas exhibit chemosensitivity and potential for spontaneous regression.
- Neoadjuvant chemotherapy is a viable strategy to prevent mutilating surgical procedures for infantile fibrosarcoma.
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.
