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Hemosiderotic Fibrolipomatous Tumor (HFLT)



Karyotype

Near-diploid karyotype with balanced or unbalanced t(1;10)(p22-p31;q24-q25), loss of material from 3p, and amplification of 3p11-p12.

t(1;10) Translocation

Does not result in a fusion transcript but consistently targets TGFBR3 (1p22) and OGA (10q24), leading to transcriptional upregulation of FGF8 and NPM3.

Frequency of t(1;10) in HFLT

Found in up to 85% of cases.

3p Amplification

Contains VGLL3 and CHMP2B, both showing increased expression.

Pathogenetic Link Between Tumors

Similar cytogenetic abnormalities (TGFBR3 and OGA rearrangement, VGLL3 amplification) are shared among HFLT, PHAT, and MIFS, suggesting a common molecular pathway.

BRAF Fusions

Present in some Myxoinflammatory Fibroblastic Sarcomas (MIFS) but not detected in HFLT.


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