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- Sparber-Sauer, Monika, et al.
(författare)
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The impact of local control in the treatment of children with advanced infantile and adult-type fibrosarcoma : Experience of the cooperative weichteilsarkom studiengruppe (CWS)
- 2020
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Ingår i: Journal of Pediatric Surgery. - : W B SAUNDERS CO-ELSEVIER INC. - 0022-3468 .- 1531-5037. ; 55:9, s. 1740-1747
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Tidskriftsartikel (refereegranskat)abstract
- Background and objectives: This study aims at examining the potential survival benefits of primary versus secondary surgery of children diagnosed with advanced infantile (iFS) and adult-type fibrosarcoma (aFS). Methods: Treatment and outcome of 89 children with FS treated within prospective Cooperative Studiengruppe (CWS) trials (1981-2016) were analyzed retrospectively. Results: Localized disease (LD) was diagnosed in 87 patients: 64/66 patients with iFS (<= 2 years) and 23 with aFS (>2 <= 18 years). Two patients (iFS) had metastatic disease. Resection was the mainstay of therapy of patients with LD resulting in microscopically complete (R0, IRS group I) (n = 29/87, 33%), microscopically incomplete (R1, IRS group II) (n = 17/87, 20%) and macroscopically incomplete (R2, IRS group III) (n = 41/87, 47%). Advanced LD (IRS group Ill) was present in 32/64 (50%) palieras with iFS and in 9/23 (39%) with aFS. Chemotherapy was added predominantly in patients with advanced disease and an assessable objective response to CHT was seen in 71% iFS and 75% aFS. The 5 -year event-free survival (EFS) of patients with iFS and aFS was 81% (+/- 10, 95% Cl) and 70% (+/- 19, 95% Cl) (p = 0.24); the 5 -year overall survival (OS) was 98% (+/- 3, 95% Cl) and 82% (+/- 16, 95% Cl) (p = 0.02). Primary resection was no prognostic factor. Secondary R0/R1 resection in patients with advanced disease improved 5-year EFS and OS in aFS (p = 0.002 and p = 0.000) but not in infants. Conclusions: Secondary resection improves outcome in advanced aFS but not in infants. Mutilating surgery in infants should be avoided. (C) 2019 Elsevier Inc. All rights reserved.
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