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Systemic therapy of aggressive fibromatosis in children and adolescents: Report of the Cooperative Weichteilsarkom Studiengruppe (CWS)
Klinikum Stuttgart Olgahosp, Stuttgart Canc Ctr, Zentrum Kinder Jugend & Frauenmed, Pediat Oncol Hematol & Immunol 5, Stuttgart, Germany..
Univ Childrens Hosp Marburg, Dept Pediat Surg, Marburg, Germany..
Klinikum Stuttgart Olgahosp, Inst Radiol, Zentrum Kinder Jugend & Frauenmed, Stuttgart, Germany..
Univ Kiel, Inst Paidopathol, Kiel, Germany..
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2018 (English)In: Pediatric Blood & Cancer, ISSN 1545-5009, E-ISSN 1545-5017, Vol. 65, no 5, article id e26943Article in journal (Refereed) Published
Abstract [en]

Background

Treatment algorithms for patients with aggressive fibromatosis (AF) are challenging. There are limited data available about the use of systemic therapy (ST) in pediatric patients with AF.

Methods

Patient-, tumor-, and treatment-related factors of 90 children and adolescents with AF treated on multiple prospective trials of the Cooperative Weichteilsarkom Studiengruppe (1981-2015) were analyzed with focus on response and outcome of ST.

Results

Median age was 9.48 years (0.02-18.05). Primary resection was performed in 54 patients and ST was administered in 29 of 54 patients because of disease progression or relapse. In 35 patients, ST was the initial treatment modality. A secondary resection was performed in 21 of 35 patients after ST. A total of 64 patients received ST, mainly methotrexate and vinblastine (40%) with a median duration of 380 days. The most frequent radiological response to ST was stable disease at 3 months (39%) and partial response at 6 months (53%). Radiotherapy was administered to 15 of 90 patients. One patient remained on observation only. The 5-year overall survival was 100% and the 5-year event-free survival (EFS) was 44%. Patients who had a primary resection showed a 5-year EFS of 35% versus 59% in patients who had received primary ST (P=0.08). Functional deficiencies as long-term sequelae following resection occurred in 11 patients. At a median follow-up of 5.05 years (0.25-14.88), complete remission was achieved in 51 patients and partial remission in 28 patients.

Conclusions

ST seems appropriate if a primary complete resection is not feasible and at relapse/progression after resection.

Place, publisher, year, edition, pages
2018. Vol. 65, no 5, article id e26943
Keywords [en]
aggressive fibromatosis, children and adolescents, CWS Group, systemic therapy
National Category
Hematology
Identifiers
URN: urn:nbn:se:uu:diva-351621DOI: 10.1002/pbc.26943ISI: 000428457100013PubMedID: 29316163OAI: oai:DiVA.org:uu-351621DiVA, id: diva2:1217534
Available from: 2018-06-13 Created: 2018-06-13 Last updated: 2018-06-13Bibliographically approved

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