TY - JOUR
T1 - Conservative strategy in infantile fibrosarcoma is possible
T2 - The European paediatric Soft tissue sarcoma Study Group experience
AU - Orbach, Daniel
AU - Brennan, Bernadette
AU - De Paoli, Angela
AU - Gallego, Soledad
AU - Mudry, Peter
AU - Francotte, Nadine
AU - van Noesel, Max
AU - Kelsey, Anna
AU - Alaggio, Rita
AU - Ranchère, Dominique
AU - De Salvo, Gian Luca
AU - Casanova, Michela
AU - Bergeron, Christophe
AU - Merks, Johannes H M
AU - Jenney, Meriel
AU - Stevens, Michael C G
AU - Bisogno, Gianni
AU - Ferrari, Andrea
N1 - Copyright © 2016 Elsevier Ltd. All rights reserved.
PY - 2016/4
Y1 - 2016/4
N2 - 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.
AB - 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.
KW - Antineoplastic Agents/therapeutic use
KW - Chemotherapy, Adjuvant/methods
KW - Combined Modality Therapy/methods
KW - Cost of Illness
KW - Dactinomycin/administration & dosage
KW - Disease-Free Survival
KW - Feasibility Studies
KW - Female
KW - Fibrosarcoma/drug therapy
KW - Follow-Up Studies
KW - Humans
KW - Infant
KW - Lymphatic Metastasis
KW - Male
KW - Neoplasm Staging
KW - Prospective Studies
KW - Reoperation
KW - Soft Tissue Neoplasms/drug therapy
KW - Treatment Outcome
KW - Vincristine/administration & dosage
KW - Watchful Waiting
UR - http://www.scopus.com/inward/record.url?scp=84957598526&partnerID=8YFLogxK
U2 - 10.1016/j.ejca.2015.12.028
DO - 10.1016/j.ejca.2015.12.028
M3 - Article
C2 - 26849118
SN - 1879-0852
VL - 57
SP - 1
EP - 9
JO - European Journal of Cancer
JF - European Journal of Cancer
ER -