Pleural Malignant Solitary Fibrous Tumor With Sarcomatous Overgrowth Showing PDGFR β Mutation

Imatinib inhibits in vitro proliferation of cells derived from a pleural solitary fibrous tumor expressing platelet-derived growth factor receptor-beta

“…Mutational analysis, performed on formalin-fixed tissue, detected no mutations for KIT, PDGFRA, or PDGFRB, as reported by one of the authors (R.G.) [10]. Retrospective analysis of 29 pleuro-pulmonary SFTs showed some expression for PDGFR-alpha and PDGFR-beta.…”

Lack of a common or characteristic cytogenetic anomaly in solitary fibrous tumor

“…Structural rearrangements of 4q13, 9p22-23, 9q22, 9q31-32, and 12q24 have each been identified in two separate SFTs suggesting there may be SFT subgroups characterized by involvement of these breakpoints [16,17,20,22,23]. Additional cases must be studied to confirm the significance of this observation.…”

“…Trisomy 5 has also been observed in a subclone of a SFT of unknown primary site [13]. Recently, Rossi et al reported a solitary fibrous tumor of the pleura with low-grade and high-grade sarcomatous components, which both components expressed platelet derived growth factor receptor β (PDGFRβ) (17). Direct-sequencing polymerase chain reaction showed a missense mutation involving exon 18 of the PDGFRβ gene, only in the high-grade sarcomatous component.…”

“…Conventional cytogenetic studies have also revealed gain of chromosome 21 and complete loss or partial deletion of chromosomes 1,6,9,13,15,17,18, and X as occurring in more than one SFT [7,13,20,21]. Similarly, chromosomal imbalances have also been detected in SFT using comparative genomic hybridization (CGH).…”

Tumor fibroso solitario de la pleura

“…Esta lesión, tiene características citogenéticas muy similares a su contraparte benigna. Sin embargo, se han observado alteraciones como la mutación del receptor del factor ß de crecimiento derivado de las plaquetas 34,35 . Microscópicamente estas lesiones presentan un incremento de la celularidad con presencia de marcada atipia celular, prominente necrosis y alta actividad mitótica, lo cual permite su distinción 21,22,36 .…”