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000177013 0247_ $$2doi$$aDOI: 10.1002/pbc.29403 
000177013 0247_ $$2doi$$aDOI: 10.1002/pbc.29403
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000177013 041__ $$aEnglish
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000177013 1001_ $$00000-0001-9551-2399$$aSparber-Sauer, Monika$$b0
000177013 245__ $$aInfantile myofibromatosis: Excellent prognosis but also rare fatal progressive disease. Treatment results of five Cooperative Weichteilsarkom Studiengruppe (CWS) trials and one registry.
000177013 260__ $$aNew York, NY$$bWiley$$c2022
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000177013 500__ $$aVolume69, Issue3, March 2022, e29403
000177013 520__ $$aInfantile myofibromatosis (IM) is a rare benign soft tissue tumor and often a self-limiting disease but rarely includes life-threatening complications. Little is known about optimal treatment of primary localized (LD) and multifocal disease (MFD).Treatment and outcome of 95 children with IM registered within five Cooperative Weichteilsarkom Studiengruppe (CWS) trials and one registry (1981-2016) were evaluated.LD was diagnosed in 71 patients at a median age of 0.4 years (range 0.0-17.7). MFD was present in 24 patients. The mainstay of treatment was watch-and-wait strategy (w&w) after initial biopsy or resection. Low-dose chemotherapy (CHT) was administered to 16/71 (23%) patients with LD and eight of 24 (33%) patients with MFD, imatinib was added in two. A delayed resection was possible in eight of 71 (11%) and five of 24 (21%) patients with LD and MFD, respectively. Overall, patients were alive in complete remission (n = 77) and partial remission (n = 10) at a median follow-up time of 3.4 years after diagnosis (range 0.01-19.4); no data available (n = 5). Three patients died of progressive disease (PD) despite CHT. Gender, tumor size, and location correlated with a favorable event-free survival (EFS) in patients with LD. The 5-year EFS and overall survival of patients with LD were 73% (±12, confidence interval [CI] 95%) and 95% (±6, CI 95%), respectively; for MFD 51% (±22, CI 95%) and 95% (±10, CI 95%).Prognosis is excellent in patients with LD and MFD. Targeted treatment needs to be evaluated for rare fatal PD.
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000177013 650_7 $$2Other$$aCWS Group
000177013 650_7 $$2Other$$ainfantile myofibromatosis
000177013 650_7 $$2Other$$ainfants and children
000177013 650_7 $$2Other$$alocalized and multifocal disease
000177013 7001_ $$00000-0002-4138-4536$$aVokuhl, Christian$$b1
000177013 7001_ $$00000-0001-9280-1451$$aSeitz, Guido$$b2
000177013 7001_ $$aSorg, Benjamin$$b3
000177013 7001_ $$0P:(DE-HGF)0$$aTobias, Möllers$$b4
000177013 7001_ $$avon Kalle, Thekla$$b5
000177013 7001_ $$aMünter, Marc$$b6
000177013 7001_ $$aBielack, Stefan S$$b7
000177013 7001_ $$aLadenstein, Ruth$$b8
000177013 7001_ $$aLjungman, Gustaf$$b9
000177013 7001_ $$aNiggli, Felix$$b10
000177013 7001_ $$aFrühwald, Michael$$b11
000177013 7001_ $$aLoff, Stefan$$b12
000177013 7001_ $$aKlingebiel, Thomas$$b13
000177013 7001_ $$00000-0002-1519-7569$$aKoscielniak, Ewa$$b14
000177013 773__ $$0PERI:(DE-600)2130978-4$$a10.1002/pbc.29403$$n3$$pe29403$$tPediatric blood & cancer$$v69$$x1545-5009$$y2022
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