title: Brief report: activity of imatinib in a patient with platelet-derived-growth-factor receptor positive malignant solitary fibrous tumor of the pleura.
abstract: Malignant solitary fibrous tumor (MSFT) of the pleura is a rare neoplasm, with unpredictable biologic behavior and a low sensitivity to chemotherapy. To the authors' knowledge, no other effective medical treatment is available for this disease. Imatinib mesylate is a tyrosine kinase inhibitor targeting the platelet-derived growth factor (PDGFR-alpha and PDGFR-beta), the BCR-ABL, and c-KIT receptors. We report the first evidence of the activity of imatinib in a symptomatic patient with a chemo- and radio-resistant advanced MSFT, who obtained a 21-months lasting major clinical benefit with a consistent reduction in tumor metabolism. Immunostaining of tumor cells demonstrated the positivity for PDGFR-alpha and PDGFR-beta and the absence of c-KIT over-expression, in the absence of c-KIT and PDGRFR mutations; all the cells strongly and diffusely expressed the ligand PDGF A in the cytoplasm. This profile suggests that the observed tumor response was mediated through the inhibition of the tyrosine kinase activity of PDGFR. Treatment with imatinib should be considered for patients with recurrent or unresectable MSFTs with PDGFR expression.
authors.affiliation: New Drugs Development Unit, European Institute of Oncology, Milan, Italy. tommaso.de-pas@ieo.it
authors.forename: Tommaso
authors.initials: T
authors.lastname: De Pas
authors.forename: Francesca
authors.initials: F
authors.lastname: Toffalorio
authors.forename: Piergiuseppe
authors.initials: P
authors.lastname: Colombo
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Trifirò
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Pelosi
authors.forename: Paolo Della
authors.initials: PD
authors.lastname: Vigna
authors.forename: Michela
authors.initials: M
authors.lastname: Manzotti
authors.forename: Monica
authors.initials: M
authors.lastname: Agostini
authors.forename: Filippo
authors.initials: F
authors.lastname: de Braud
mesh_terms: D000328:Adult; D001549:Benzamides; D005260:Female; D006801:Humans; D000068877:Imatinib Mesylate; D007124:Immunoenzyme Techniques; D010879:Piperazines / Q000627:therapeutic use*; D010997:Pleural Neoplasms / Q000188:drug therapy* / Q000378:metabolism / Q000601:surgery; D011013:Pneumonectomy; D047428:Protein Kinase Inhibitors / Q000627:therapeutic use*; D011505:Protein-Tyrosine Kinases / Q000037:antagonists & inhibitors; D019009:Proto-Oncogene Proteins c-kit / Q000378:metabolism; D011743:Pyrimidines / Q000627:therapeutic use*; D020796:Receptor, Platelet-Derived Growth Factor alpha / Q000037:antagonists & inhibitors / Q000378:metabolism*; D020797:Receptor, Platelet-Derived Growth Factor beta / Q000037:antagonists & inhibitors / Q000378:metabolism*; D054363:Solitary Fibrous Tumor, Pleural / Q000188:drug therapy* / Q000378:metabolism / Q000601:surgery; D014057:Tomography, X-Ray Computed
chemical_list: D001549:Benzamides; D010879:Piperazines; D047428:Protein Kinase Inhibitors; D011743:Pyrimidines; D000068877:Imatinib Mesylate; D011505:Protein-Tyrosine Kinases; D019009:Proto-Oncogene Proteins c-kit; D020796:Receptor, Platelet-Derived Growth Factor alpha; D020797:Receptor, Platelet-Derived Growth Factor beta