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Conservative strategy in infantile fibrosarcoma is possible: The European paediatric Soft tissue sarcoma Study
Daniel Orbach1, Bernadette Brennan2, Angela De Paoli3
1Department of Pediatric, Adolescent and Young Adult Oncology, Institut Curie, Paris, France.
Insights
Conservative therapy for infantile fibrosarcoma (IFS) is effective, minimizing mutilating surgery and avoiding intensive chemotherapy. The vincristine-actinomycin-D regimen is recommended as a first-line treatment for unresectable tumors to reduce long-term effects.
Area of Science:
- Pediatric Oncology
- Surgical Oncology
- Medical Genetics
Background:
- Infantile fibrosarcoma (IFS) is a rare pediatric malignancy with high local aggressiveness but favorable survival rates.
- Conservative therapeutic strategies are being developed to reduce treatment burden in infants.
- Initial resectability guides treatment recommendations in IFS management.
Purpose of the Study:
- To evaluate the efficacy of conservative therapeutic recommendations for infantile fibrosarcoma.
- To assess the impact of initial resectability on treatment strategies and outcomes.
- To determine the optimal first-line chemotherapy regimen for unresectable IFS.
Main Methods:
- Prospective registration of children with localized IFS between 2005 and 2012.
- Initial surgery was performed only if it could be achieved without mutilation.
- Vincristine-actinomycin-D (VA) chemotherapy was administered as first-line treatment for initially inoperable tumors (IRS-group III/R2).
Main Results:
- A total of 50 infants were included; 87.2% had the ETV6-NTRK3 transcript.
- Complete or microscopic incomplete resection (IRS-group I/II) required no further therapy.
- VA chemotherapy showed a 68.0% response rate in IRS-III/R2 patients, with only 3 cases requiring mutilating surgery.
Conclusions:
- Conservative therapy is feasible in IFS, significantly reducing the need for mutilating surgery.
- The vincristine-actinomycin-D (VA) regimen is effective as a first-line therapy for unresectable IFS.
- Avoiding alkylating or anthracycline-based chemotherapy in 71.0% of cases reduces potential long-term adverse effects.
Background:
Infantile fibrosarcoma (IFS) is a very rare disease occurring in young infants characterised by a high local aggressiveness but overall with a favourable survival. To try to reduce the total burden of therapy, the European pediatric Soft tissue sarcoma Study Group has developed conservative therapeutic recommendations according to initial resectability.
Material And Methods:
Between 2005 and 2012, children with localised IFS were prospectively registered. Initial surgery was suggested only if possible without mutilation. Patients with initial complete (IRS-group I/R0) or microscopic incomplete (group II/R1) resection had no further therapy. Patients with initial inoperable tumour (group III/R2) received first-line vincristine-actinomycin-D chemotherapy (VA). Delayed conservative surgery was planned after tumour reduction. Aggressive local therapy (mutilating surgery or external radiotherapy) was discouraged.
Results:
A total of 50 infants (median age 1.4 months), were included in the study. ETV6-NTRK3 transcript was present in 87.2% of patients where investigation was performed. According to initial surgery, 11 patients were classified as group I, 8 as group II and 31 as group III. VA chemotherapy was first delivered to 25 children with IRS-III/R2 and one with IRS-II/R1 disease. Response rate to VA was 68.0%. Mutilating surgery was only performed in three cases. After a median follow-up of 4.7 years (range 1.9-9.0), 3-year event-free survival and overall survival were respectively 84.0% (95% confidence interval [CI] 70.5-91.7) and 94.0% (95% CI 82.5-98.0).
Conclusions:
Conservative therapy is possible in IFS as only three children required mutilating surgery, and alkylating or anthracycline based chemotherapy was avoided in 71.0% of patients needing chemotherapy. VA regimen should be first line therapy in order to reduce long term effects.