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Infantile myofibrom...
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Sparber-Sauer, Monika
(author)
Infantile myofibromatosis : Excellent prognosis but also rare fatal progressive disease. Treatment results of five Cooperative Weichteilsarkom Studiengruppe (CWS) trials and one registry
- Article/chapterEnglish2022
Publisher, publication year, extent ...
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2021-10-11
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John Wiley & Sons,2022
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LIBRIS-ID:oai:DiVA.org:uu-459712
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https://urn.kb.se/resolve?urn=urn:nbn:se:uu:diva-459712URI
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https://doi.org/10.1002/pbc.29403DOI
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Language:English
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Summary in:English
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Subject category:ref swepub-contenttype
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Subject category:art swepub-publicationtype
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Background: Infantile 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).Methods: Treatment and outcome of 95 children with IM registered within five Cooperative Weichteilsarkom Studiengruppe (CWS) trials and one registry (1981-2016) were evaluated.Results: 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%).Cconclusion: Prognosis is excellent in patients with LD and MFD. Targeted treatment needs to be evaluated for rare fatal PD.
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Vokuhl, Christian
(author)
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Seitz, Guido
(author)
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Sorg, Benjamin
(author)
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Tobias, Möllers
(author)
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von Kalle, Thekla
(author)
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Münter, Marc
(author)
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Bielack, Stefan S
(author)
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Ladenstein, Ruth
(author)
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Ljungman, Gustaf,1958-Uppsala universitet,Institutionen för kvinnors och barns hälsa(Swepub:uu)gustaflm
(author)
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Niggli, Felix
(author)
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Frühwald, Michael
(author)
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Loff, Stefan
(author)
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Klingebiel, Thomas
(author)
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Koscielniak, Ewa
(author)
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Uppsala universitetInstitutionen för kvinnors och barns hälsa
(creator_code:org_t)
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In:Pediatric Blood & Cancer: John Wiley & Sons69:31545-50091545-5017
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Sparber-Sauer, M ...
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Vokuhl, Christia ...
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Seitz, Guido
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Sorg, Benjamin
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Tobias, Möllers
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von Kalle, Thekl ...
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Münter, Marc
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Bielack, Stefan ...
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Ladenstein, Ruth
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Ljungman, Gustaf ...
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Niggli, Felix
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Frühwald, Michae ...
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Loff, Stefan
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Klingebiel, Thom ...
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Koscielniak, Ewa
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Pediatric Blood ...
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Uppsala University