title: Comparison of morphological, immunohistochemical, and molecular genetic features of inflammatory fibroid polyps (Vanek's tumors).
abstract: Vanek's tumor (inflammatory fibroid polyp) is a rare benign lesion occurring throughout the digestive tract. Histologically, two patterns can be recognized. Classical Vanek's tumor contains concentric formations of proliferating spindle cells which are CD34 positive. Atypical, inflammatory pseudotumor-like Vanek's tumor lacks concentric formations and is CD34 negative. Recently, mutations in platelet-derived growth factor receptor alpha (PDGFRA) were reported in gastric and small intestinal Vanek's tumors. In this study, KIT exons 9, 11, 13, and 17, PDGFRA exons 12, 14, and 18, and a part of exon 15 BRAF for point mutation V600E were screened in 23 cases of Vanek's tumor, both classical (n = 16) and inflammatory pseudotumor-like (n = 7). No mutations in all analyzed exons of KIT and BRAF and in exon 14 of PDGFRA were detected. Six Vanek's tumors harbored activating mutations in PDGFRA exons 12 (n = 5) and 18 (n = 1) respectively: S566_E571delinsK (n = 1), S566_E571delinsR (n = 4), and D842 del (n = 1). The mutations were detected in the classical (n = 5), as well as inflammatory pseudotumor-like (n = 1) Vanek's tumors. The results of this study suggest that the two morphological patterns of Vanek's tumor more probably represent only variants of one type of tumor than two different lesions. Furthermore, BRAF mutations were not shown to drive growth of PDGFRA wild-type Vanek's tumors.
authors.affiliation: Department of Pathology, Medical Faculty Hospital,, Charles University in Prague, Medical Faculty in Plzen, Edvarda Benese 13, Plzen, Czech Republic. DAUM@fnplzen.cz
authors.forename: Ondrej
authors.initials: O
authors.lastname: Daum
authors.forename: Jana
authors.initials: J
authors.lastname: Hatlova
authors.forename: Vaclav
authors.initials: V
authors.lastname: Mandys
authors.forename: Petr
authors.initials: P
authors.lastname: Grossmann
authors.forename: Petr
authors.initials: P
authors.lastname: Mukensnabl
authors.forename: Zdenek
authors.initials: Z
authors.lastname: Benes
authors.forename: Michal
authors.initials: M
authors.lastname: Michal
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005091:Exons; D005260:Female; D041981:Gastrointestinal Tract / Q000473:pathology; D006801:Humans; D007410:Intestinal Diseases / Q000235:genetics / Q000473:pathology*; D007889:Leiomyoma / Q000235:genetics* / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009154:Mutation; D011127:Polyps / Q000235:genetics* / Q000473:pathology*; D048493:Proto-Oncogene Proteins B-raf / Q000235:genetics; D020796:Receptor, Platelet-Derived Growth Factor alpha / Q000235:genetics*; D013272:Stomach Diseases / Q000235:genetics / Q000473:pathology*
chemical_list: D020796:Receptor, Platelet-Derived Growth Factor alpha; C482119:BRAF protein, human; D048493:Proto-Oncogene Proteins B-raf
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