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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)


Sparber-Sauer, Monika; Vokuhl, Christian; Seitz, Guido; Stegmaier, Sabine; Hallmen, Erika; von Kalle, Thekla; Scheer, Monika; Münter, Marc; Bielack, Stefan S; Ladenstein, Ruth; Niggli, Felix; Ljungman, Gustaf; Fuchs, Joerg; Klingebiel, Thomas; Koscielniak, Ewa; CWS Study Group, ? (2019). The impact of local control in the treatment of children with advanced infantile and adult-type fibrosarcoma: experience of the Cooperative Weichteilsarkom Studiengruppe (CWS). Journal of Pediatric Surgery:Epub ahead of print.

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 III) was present in 32/64 (50%) patients 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% CI) and 70% (±19, 95% CI) (p = 0.24); the 5-year overall survival (OS) was 98% (±3, 95% CI) and 82% (±16, 95% CI) (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.
TYPE OF STUDY AND LEVEL OF EVIDENCE: Treatment study: patients were enrolled in five prospective studies and one registry, prognosis study: retrospective study.
LEVEL OF EVIDENCE: II/ III.
MINI-ABSTRACT: Fibrosarcoma is a very rare malignant tumor. Little is known about differences of local treatment of advanced infantile and adult-type. Data of 89 patients registered in five prospective trials and one registry of the Cooperative Weichteilsarkom Studiengruppe (CWS) (1981-2016) were analyzed.

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 III) was present in 32/64 (50%) patients 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% CI) and 70% (±19, 95% CI) (p = 0.24); the 5-year overall survival (OS) was 98% (±3, 95% CI) and 82% (±16, 95% CI) (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.
TYPE OF STUDY AND LEVEL OF EVIDENCE: Treatment study: patients were enrolled in five prospective studies and one registry, prognosis study: retrospective study.
LEVEL OF EVIDENCE: II/ III.
MINI-ABSTRACT: Fibrosarcoma is a very rare malignant tumor. Little is known about differences of local treatment of advanced infantile and adult-type. Data of 89 patients registered in five prospective trials and one registry of the Cooperative Weichteilsarkom Studiengruppe (CWS) (1981-2016) were analyzed.

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Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > University Children's Hospital Zurich > Medical Clinic
Dewey Decimal Classification:610 Medicine & health
Language:English
Date:9 November 2019
Deposited On:12 Feb 2020 17:14
Last Modified:12 Feb 2020 17:14
Publisher:Elsevier
ISSN:0022-3468
OA Status:Closed
Publisher DOI:https://doi.org/10.1016/j.jpedsurg.2019.10.051
PubMed ID:31753608

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