Conservative strategy in infantile fibrosarcoma is possible:
The European paediatric Soft tissue sarcoma Study Group
experience

J 2016

Conservative strategy in infantile fibrosarcoma is possible: The European paediatric Soft tissue sarcoma Study Group experience

ORBACH, Daniel, Bernadette BRENNAN, Angela DE PAOLI, Soledad GALLEGO, Peter MÚDRY et. al.

Základní údaje

Originální název

Conservative strategy in infantile fibrosarcoma is possible: The European paediatric Soft tissue sarcoma Study Group experience

Autoři

ORBACH, Daniel (250 Francie), Bernadette BRENNAN (826 Velká Británie a Severní Irsko), Angela DE PAOLI (380 Itálie), Soledad GALLEGO (724 Španělsko), Peter MÚDRY (203 Česká republika, garant, domácí), Nadine FRANCOTTE (56 Belgie), Max van NOESEL (528 Nizozemské království), Anna KELSEY (826 Velká Británie a Severní Irsko), Rito ALAGGIO (380 Itálie), Dominique RANCHERE (250 Francie), Gian Luca DE SALVO (380 Itálie), Michaela CASANOVA (380 Itálie), Christophe BERGERON (250 Francie), Johannes H.M. MERKS (528 Nizozemské království), Meriel JENNEY (826 Velká Británie a Severní Irsko), Michael C.G. STEVENS (826 Velká Británie a Severní Irsko), Gianni BISOGNO (380 Itálie) a Andrea FERRARI (380 Itálie)

Vydání

European Journal of Cancer, Oxford, Elsevier Science Inc. 2016, 0959-8049

Další údaje

Jazyk

angličtina

Typ výsledku

Článek v odborném periodiku

Obor

30200 3.2 Clinical medicine

Stát vydavatele

Velká Británie a Severní Irsko

Utajení

není předmětem státního či obchodního tajemství

Impakt faktor

Impact factor: 6.029

Kód RIV

RIV/00216224:14110/16:00092096

Organizační jednotka

Lékařská fakulta

DOI

http://dx.doi.org/10.1016/j.ejca.2015.12.028

UT WoS

000371780300001

Klíčová slova anglicky

Infantile fibrosarcoma; Newborn; Infant; Cancer; Chemotherapy; ETV6-NTRK3 transcript

Štítky

EL OK

Příznaky

Mezinárodní význam, Recenzováno

Změněno: 1. 12. 2016 14:14, Soňa Böhmová

Anotace

V originále

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. (C) 2016 Elsevier Ltd. All rights reserved.

Citovat

ORBACH, Daniel, Bernadette BRENNAN, Angela DE PAOLI, Soledad GALLEGO, Peter MÚDRY, Nadine FRANCOTTE, Max van NOESEL, Anna KELSEY, Rito ALAGGIO, Dominique RANCHERE, Gian Luca DE SALVO, Michaela CASANOVA, Christophe BERGERON, Johannes H.M. MERKS, Meriel JENNEY, Michael C.G. STEVENS, Gianni BISOGNO a Andrea FERRARI. Conservative strategy in infantile fibrosarcoma is possible: The European paediatric Soft tissue sarcoma Study Group experience. European Journal of Cancer. Oxford: Elsevier Science Inc., 2016, roč. 57, APR 2016, s. 1-9. ISSN 0959-8049. Dostupné z: https://dx.doi.org/10.1016/j.ejca.2015.12.028.

@article{1363098,
   author = {Orbach, Daniel and Brennan, Bernadette and De Paoli, Angela and Gallego, Soledad and Múdry, Peter and Francotte, Nadine and Noesel, Max van and Kelsey, Anna and Alaggio, Rito and Ranchere, Dominique and De Salvo, Gian Luca and Casanova, Michaela and Bergeron, Christophe and Merks, Johannes H.M. and Jenney, Meriel and Stevens, Michael C.G. and Bisogno, Gianni and Ferrari, Andrea},
   article_location = {Oxford},
   article_number = {APR 2016},
   doi = {http://dx.doi.org/10.1016/j.ejca.2015.12.028},
   keywords = {Infantile fibrosarcoma; Newborn; Infant; Cancer; Chemotherapy; ETV6-NTRK3 transcript},
   language = {eng},
   issn = {0959-8049},
   journal = {European Journal of Cancer},
   title = {Conservative strategy in infantile fibrosarcoma is possible: The European paediatric Soft tissue sarcoma Study Group experience},
   volume = {57},
   year = {2016}
}

TY  - JOUR
ID  - 1363098
AU  - Orbach, Daniel - Brennan, Bernadette - De Paoli, Angela - Gallego, Soledad - Múdry, Peter - Francotte, Nadine - Noesel, Max van - Kelsey, Anna - Alaggio, Rito - Ranchere, Dominique - De Salvo, Gian Luca - Casanova, Michaela - Bergeron, Christophe - Merks, Johannes H.M. - Jenney, Meriel - Stevens, Michael C.G. - Bisogno, Gianni - Ferrari, Andrea
PY  - 2016
TI  - Conservative strategy in infantile fibrosarcoma is possible: The European paediatric Soft tissue sarcoma Study Group experience
JF  - European Journal of Cancer
VL  - 57
IS  - APR 2016
SP  - 1-9
EP  - 1-9
PB  - Elsevier Science Inc.
SN  - 09598049
KW  - Infantile fibrosarcoma
KW  - Newborn
KW  - Infant
KW  - Cancer
KW  - Chemotherapy
KW  - ETV6-NTRK3 transcript
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. (C) 2016 Elsevier Ltd. All rights reserved.
ER  -

ORBACH, Daniel, Bernadette BRENNAN, Angela DE PAOLI, Soledad GALLEGO, Peter MÚDRY, Nadine FRANCOTTE, Max van NOESEL, Anna KELSEY, Rito ALAGGIO, Dominique RANCHERE, Gian Luca DE SALVO, Michaela CASANOVA, Christophe BERGERON, Johannes H.M. MERKS, Meriel JENNEY, Michael C.G. STEVENS, Gianni BISOGNO a Andrea FERRARI. Conservative strategy in infantile fibrosarcoma is possible: The European paediatric Soft tissue sarcoma Study Group experience. \textit{European Journal of Cancer}. Oxford: Elsevier Science Inc., 2016, roč.~57, APR 2016, s.~1-9. ISSN~0959-8049. Dostupné z: https://dx.doi.org/10.1016/j.ejca.2015.12.028.

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