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Low-grade fibromyxo...
Low-grade fibromyxoid sarcoma : A report of the Cooperative Weichteilsarkom Studiengruppe (CWS)
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- Scheer, Monika (author)
- Olgahospital, Klinikum Stuttgart, Pediat Oncol 5, Hematol,Immunol, Kriegsbergstr 62, D-70174 Stuttgart, Germany.
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- Vokuhl, Christian (author)
- Univ Hosp Schleswig Holstein, Kiel Paediat Tumor Registry, Dept Paediat Pathol, Kiel, Germany.
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- Veit-Friedrich, Iris (author)
- Olgahospital, Klinikum Stuttgart, Pediat Oncol 5, Hematol,Immunol, Kriegsbergstr 62, D-70174 Stuttgart, Germany.
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- Münter, Marc (author)
- Olgahospital, Klinikum Stuttgart, Dept Radiat Oncol, Stuttgart, Germany.
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- von Kalle, Thekla (author)
- Katharinenhospital, Klinikum Stuttgart, Radiol Inst, Pediat Radiol, Stuttgart, Germany.
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- Greulich, Michael (author)
- Olgahospital, Klinikum Stuttgart, Pediat Surg, Stuttgart, Germany.
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- Loff, Steffan (author)
- Olgahospital, Klinikum Stuttgart, Pediat Surg, Stuttgart, Germany.
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- Stegmaier, Sabine (author)
- Olgahospital, Klinikum Stuttgart, Pediat Oncol 5, Hematol,Immunol, Kriegsbergstr 62, D-70174 Stuttgart, Germany.
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Sparber-Sauer, Monika (author)
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- Niggli, Felix (author)
- Univ Zurich, Dept Pediat Oncol, Zurich, Switzerland.
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- Ladenstein, Ruth (author)
- St Anna Childrens Hosp, Vienna, Austria.;St Anna Kinderkrebsforsch eV, Vienna, Austria.
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- Kazanowska, Bernarda (author)
- Univ Wroclaw, Dept Pediat Oncol, Wroclaw, Poland.
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- Ljungman, Gustaf, 1958- (author)
- Uppsala universitet,Barnneurologi/Barnonkologi
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- Jahnukainen, Kirsi (author)
- Univ Helsinki, Cent Hosp, Dept Pediat, Helsinki, Finland.
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- Fuchs, Jörg (author)
- Univ Childrens Hosp, Dept Pediat Surg & Urol, Tubingen, Germany.
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- Bielack, Stefan S. (author)
- Olgahospital, Klinikum Stuttgart, Pediat Oncol 5, Hematol,Immunol, Kriegsbergstr 62, D-70174 Stuttgart, Germany.;Univ Childrens Hosp Muenster, Dept Pediat Hematol & Oncol, Munster, Germany.
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- Koscielniak, Ewa (author)
- Olgahospital, Klinikum Stuttgart, Pediat Oncol 5, Hematol,Immunol, Kriegsbergstr 62, D-70174 Stuttgart, Germany.;Goethe Univ Frankfurt Main, Hosp Children & Adolescents, Frankfurt, Germany.
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- Klingebiel, Thomas (author)
- Univ Tubingen, Dept Pediat Oncol, Tubingen, Germany.
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Olgahospital, Klinikum Stuttgart, Pediat Oncol 5, Hematol,Immunol, Kriegsbergstr 62, D-70174 Stuttgart, Germany Univ Hosp Schleswig Holstein, Kiel Paediat Tumor Registry, Dept Paediat Pathol, Kiel, Germany. (creator_code:org_t)
- 2019-11-17
- 2020
- English.
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In: Pediatric Blood & Cancer. - : WILEY. - 1545-5009 .- 1545-5017. ; 67:2
- Related links:
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https://urn.kb.se/re...
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https://doi.org/10.1...
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Abstract
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- Background: Low-grade fibromyxoid sarcoma (LGFMS) is a rare soft-tissue tumor with benign histologic appearance, though fully malignant behavior is possible.Methods: Patients with LGFMS<21 years registered in Cooperative Weichteilsarkom Studiengruppe trials until 2017 were analyzed. Firstline treatment consisted of complete surgical resection whenever possible.Results: Median age of 31 patients was 10.9 years (first month to 17.1 years). Twenty-six tumors were confirmed to the tissue of origin (T1), four invaded contiguous structures (T2), one was TX. Eight were >5 cm. The best surgical result was resection with free margins (R0) in 24 and microscopic residuals (R1) in seven. Five-year event-free (EFS), 5-year local-relapse-free (LRFS), and 5-year overall-survival were 71 +/- 18.6% confidence interval (CI) 95%, 76 +/- 17.6% CI 95%, and 100%, respectively. Six patients suffered local relapse in a median of 1 year, one combined within 1.3 year and one metastatic relapse with lesions in the lung, back muscles, and thigh discovered in whole-body imaging 6 years after the first diagnosis. In univariate analysis, T status correlated with EFS (T1 79.6 +/- 18.6%, T2 50.0 +/- 49.0%, P = .038). Resection with free margins tends to be associated with better LRFS (R0 82.4 +/- 18.6%, R1 53.6 +/- 39.4%, P = .053). Among 24 patients with R0 resection, five (21%) suffered relapse, thereof three local, one metastatic, and one combined. Among seven patients with R1-resection, three (43%) suffered local relapse.Conclusion Special caution is advisable in T2 tumors. The metastatic potential with lesions in unusual sites indicates that affected patients need to be informed. If long-term follow-up with whole-body imaging is beneficial, it may be addressed in larger intergroup analyses. Further research in disease biology is essential for optimal treatment and follow-up care.
Subject headings
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Cancer och onkologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Cancer and Oncology (hsv//eng)
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Hematologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Hematology (hsv//eng)
Keyword
- low-grade fibromyxoid sarcoma
- pediatric oncology
- pediatric sarcoma
- rare tumors
- soft-tissue sarcoma
Publication and Content Type
- ref (subject category)
- art (subject category)
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Scheer, Monika
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Vokuhl, Christia ...
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Veit-Friedrich, ...
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Münter, Marc
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von Kalle, Thekl ...
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Greulich, Michae ...
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Loff, Steffan
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Stegmaier, Sabin ...
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Sparber-Sauer, M ...
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Niggli, Felix
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Ladenstein, Ruth
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Kazanowska, Bern ...
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Ljungman, Gustaf ...
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Jahnukainen, Kir ...
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Fuchs, Jörg
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Bielack, Stefan ...
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Koscielniak, Ewa
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Klingebiel, Thom ...
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- MEDICAL AND HEALTH SCIENCES
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Pediatric Blood ...
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Uppsala University