Fibrogenic tumors

2022-03-01   Paola Dal Cin , Judith VMG Bovée 

1.Brigham and Women's Hospital , Harvard Medical School, Boston , MA (USA)
2. Leiden University Medical Center, Leiden, The Netherlands



Fibrogenic tumors are extremely rare in bone and are characterized by the production of collagenous stroma and the absence of bone and cartilage formation. Desmoplastic fibroma of bone is locally aggressive and considered to be an osseous counterpart of desmoid-type fibromatosis.

Fibrogenic tumorsGenetic marker(s)
Desmoplastic fibroma of boneSingle cytogentic data. 1 Single cases with CTNNB1 mutation: p.Thr41Ala and p.Ser45Phe 2,3
Fibrosarcoma of boneSeveral chromsomal imbalances by microarray analysis , including CDKN2A (9p) homozygous loss and both STARD13 (13q) heterozygous and homozygous loss. 4 STRN::NTRK3 fusion in a single case 5


Reference NumberPubmed IDLast YearTitleAuthors
1228680022012Molecular genetic characterization of the 11q13 breakpoint in a desmoplastic fibroma of bone.Trombetta D et al
2301591382018Low-grade central fibroblastic osteosarcoma may be differentiated from its mimicker desmoplastic fibroma by genetic analysis.Song W et al
3319014372020A case of desmoplastic fibroma of bone with CTNNB1 point mutation.Kadowaki H et al
4198628222010Frequent deletion of CDKN2A and recurrent coamplification of KIT, PDGFRA, and KDR in fibrosarcoma of bone--an array comparative genomic hybridization study.Niini T et al
5305208182019Novel NTRK3 Fusions in Fibrosarcomas of Adults.Yamazaki F et al

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Paola Dal Cin ; Judith VMG Bovée

Fibrogenic tumors

Atlas Genet Cytogenet Oncol Haematol. 2022-03-01

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