title: PDGFR expression in differential diagnosis between KIT-negative gastrointestinal stromal tumours and other primary soft-tissue tumours of the gastrointestinal tract.
abstract: OBJECTIVE
  To investigate the value of platelet-derived growth factor receptors (PDGFRs) by immunohistochemistry in discriminating KIT-negative gastrointestinal stromal tumours (GISTs) from other soft-tissue neoplasms of the digestive tract.
  
  
  RESULTS
  One-hundred and sixty-seven primary gastrointestinal mesenchymal tumours (125 GISTs, 15 intra-abdominal desmoids, 12 leiomyomas, eight leiomyosarcomas, three schwannomas, two solitary fibrous tumours, and one case each of inflammatory pseudotumour and fibroid polyp) were reclassified based on morphology and on the immunohistochemical panel recommended by the National Institutes of Health consensus on GIST. All cases were then tested with antibodies specific for PDGFR alpha and beta. Of 125 GISTs, 117 were KIT-positive (93.6%) and eight KIT-negative (6.4%). All the KIT-positive GISTs were negative for both PDGFRs, while all the eight KIT-negative GISTs expressed PDGFR-alpha, with two of them also coexpressing PDGFR-beta. Among the 42 non-GIST tumours, only a small percentage (26.6%) of desmoids immunostained for PDGFR-alpha, two of them coexpressing PDGFR-beta.
  
  
  CONCLUSIONS
  Immunostaining with PDGFR-alpha is a helpful marker in discriminating between KIT-negative GISTs and other gastrointestinal mesenchymal lesions: all KIT-negative GISTs were positive for PDFGR-alpha, while none of the other gastrointestinal mesenchymal tumours analysed, except a small subset of desmoids, was reactive with anti-PDGFRs. These preliminary data demonstrate the suitability of commercially available antibodies to detect immunohistochemically the mutually exclusive expression of KIT and PDGFR-alpha previously reported in GISTs by molecular biological techniques. Since PDGFR exists in the form of a homodimer (alphaalpha, betabeta) or heterodimer (alphabeta) and two of the KIT-negative GISTs coexpressed both PDGFR isoforms, further investigations are required to elucidate the role of PDGFR-beta in GISTs.
authors.affiliation: Department of Pathologic Anatomy and Legal Medicine, Section of Pathologic Anatomy, University of Modena and Reggio Emilia, Modena, Italy. rossi.giulio@unimo.it
authors.forename: G
authors.initials: G
authors.lastname: Rossi
authors.forename: R
authors.initials: R
authors.lastname: Valli
authors.forename: F
authors.initials: F
authors.lastname: Bertolini
authors.forename: A
authors.initials: A
authors.lastname: Marchioni
authors.forename: A
authors.initials: A
authors.lastname: Cavazza
authors.forename: C
authors.initials: C
authors.lastname: Mucciarini
authors.forename: M
authors.initials: M
authors.lastname: Migaldi
authors.forename: M
authors.initials: M
authors.lastname: Federico
authors.forename: G P
authors.initials: GP
authors.lastname: Trentini
authors.forename: A
authors.initials: A
authors.lastname: Sgambato
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000818:Animals; D018952:Antigens, CD34 / Q000032:analysis; D003937:Diagnosis, Differential; D005260:Female; D046152:Gastrointestinal Stromal Tumors / Q000378:metabolism / Q000473:pathology*; D041981:Gastrointestinal Tract / Q000737:chemistry / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D007889:Leiomyoma / Q000378:metabolism / Q000473:pathology*; D007890:Leiomyosarcoma / Q000378:metabolism / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D019009:Proto-Oncogene Proteins c-kit / Q000032:analysis; D020796:Receptor, Platelet-Derived Growth Factor alpha / Q000032:analysis; D020797:Receptor, Platelet-Derived Growth Factor beta / Q000032:analysis; D017479:Receptors, Platelet-Derived Growth Factor / Q000096:biosynthesis*
chemical_list: D018952:Antigens, CD34; D019009:Proto-Oncogene Proteins c-kit; D020796:Receptor, Platelet-Derived Growth Factor alpha; D020797:Receptor, Platelet-Derived Growth Factor beta; D017479:Receptors, Platelet-Derived Growth Factor