SwePub
Sök i LIBRIS databas

  Extended search

id:"swepub:oai:DiVA.org:uu-459712"
 

Search: id:"swepub:oai:DiVA.org:uu-459712" > Infantile myofibrom...

  • 1 of 1
  • Previous record
  • Next record
  •    To hitlist
  • 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 ...

  • 2021-10-11
  • John Wiley & Sons,2022
  • printrdacarrier

Numbers

  • LIBRIS-ID:oai:DiVA.org:uu-459712
  • https://urn.kb.se/resolve?urn=urn:nbn:se:uu:diva-459712URI
  • https://doi.org/10.1002/pbc.29403DOI

Supplementary language notes

  • Language:English
  • Summary in:English

Part of subdatabase

Classification

  • Subject category:ref swepub-contenttype
  • Subject category:art swepub-publicationtype

Notes

  • 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.

Subject headings and genre

Added entries (persons, corporate bodies, meetings, titles ...)

  • Vokuhl, Christian (author)
  • Seitz, Guido (author)
  • Sorg, Benjamin (author)
  • Tobias, Möllers (author)
  • von Kalle, Thekla (author)
  • Münter, Marc (author)
  • Bielack, Stefan S (author)
  • Ladenstein, Ruth (author)
  • Ljungman, Gustaf,1958-Uppsala universitet,Institutionen för kvinnors och barns hälsa(Swepub:uu)gustaflm (author)
  • Niggli, Felix (author)
  • Frühwald, Michael (author)
  • Loff, Stefan (author)
  • Klingebiel, Thomas (author)
  • Koscielniak, Ewa (author)
  • Uppsala universitetInstitutionen för kvinnors och barns hälsa (creator_code:org_t)

Related titles

  • In:Pediatric Blood & Cancer: John Wiley & Sons69:31545-50091545-5017

Internet link

Find in a library

To the university's database

  • 1 of 1
  • Previous record
  • Next record
  •    To hitlist

Kungliga biblioteket hanterar dina personuppgifter i enlighet med EU:s dataskyddsförordning (2018), GDPR. Läs mer om hur det funkar här.
Så här hanterar KB dina uppgifter vid användning av denna tjänst.

 
pil uppåt Close

Copy and save the link in order to return to this view