Desmoid-type fibromatosis in a boy with Down syndrome

Hisashi Ishida, Kousuke Chayama, Kiichiro Kanamitsu, Kana Washio, Takehiro Tanaka, Akira Shimada

Research output: Contribution to journalArticle

Abstract

Patients with Down syndrome (DS) have a markedly higher incidence of childhood leukemia, but a lower incidence of most solid tumors, compared with age-matched euploid individuals. Trisomy 21 might be protective against tumorigenesis because of several tumor suppressive mechanisms. Desmoid-type fibromatosis (DF) is a rare monoclonal, fibroblastic proliferation characterized by a variable clinical course. In recent reports, almost all cases of DF involved genomic alterations associated with activation of the Wnt/β-catenin pathway. Here, we report the case of a boy with DS who developed DF without activation of the Wnt/β-catenin pathway. To the best of our knowledge, this is the first case of DS involving DF.

Original languageEnglish
Pages (from-to)624-626
Number of pages3
JournalPediatrics International
Volume59
Issue number5
DOIs
Publication statusPublished - May 1 2017

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Aggressive Fibromatosis
Fibroma
Down Syndrome
Catenins
Wnt Signaling Pathway
Incidence
Neoplasms
Carcinogenesis
Leukemia

Keywords

  • desmoid-type fibromatosis
  • Down syndrome

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Desmoid-type fibromatosis in a boy with Down syndrome. / Ishida, Hisashi; Chayama, Kousuke; Kanamitsu, Kiichiro; Washio, Kana; Tanaka, Takehiro; Shimada, Akira.

In: Pediatrics International, Vol. 59, No. 5, 01.05.2017, p. 624-626.

Research output: Contribution to journalArticle

Ishida, Hisashi ; Chayama, Kousuke ; Kanamitsu, Kiichiro ; Washio, Kana ; Tanaka, Takehiro ; Shimada, Akira. / Desmoid-type fibromatosis in a boy with Down syndrome. In: Pediatrics International. 2017 ; Vol. 59, No. 5. pp. 624-626.
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