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  • 1 Általános és Mellkassebészeti Osztály, Országos Onkológiai Intézet, 1122, Budapest, Ráth György u. 7–9.
  • | 2 Molekuláris Genetikai Osztály, Országos Onkológiai Intézet, Budapest
  • | 3 Sugárterápiás Osztály, Országos Onkológiai Intézet, Budapest
  • | 4 Kemoterápia B Belgyógyászati-Onkológiai és Klinikai Farmakológiai Osztály, Országos Onkológiai Intézet, Budapest
  • | 5 Radiológiai Diagnosztikus Osztály, Országos Onkológiai Intézet, Budapest
  • | 6 Sebészi és Molekuláris Daganatpatológiai Centrum, Országos Onkológiai Intézet, Budapest
  • | 7 Budapest XII. kerület Hegyvidéki Önkormányzat Fogorvosi Rendelőintézete, Budapest
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Absztrakt

A Gardner-syndroma a familiaris adenomatosus polyposis klinikai altípusa, autosomalis dominánsan öröklődő betegség, amelyet a gastrointestinalis traktus polyposisa és extraintestinalis elváltozások jellemeznek, mint multiplex osteomák, valamint bőr- és lágyrésztumorok. A Gardner-syndromához társuló desmoid tumorok terápiás kihívást jelenthetnek. A szerzők egy 17 éves nőbeteg esetét mutatják be, akinél a Gardner-syndroma a lumbalis régió agresszív desmoid tumorával jelent meg. A beteget a 80 hónapos utánkövetés során sebészileg, non-steroid gyulladáscsökkentőkkel, tamoxifennel és sugárterápiával kezeltük. Következtetésként elmondhatjuk, hogy familiaris adenomatosus polyposisban vagy Gardner-syndromában a desmoid tumorok megjelenése megelőzheti a betegség gastrointestinalis manifesztációját. Az ilyen betegeknél genetikai vizsgálatot és colonoscopiát kell végezni, megelőzendő a későbbi colorectalis rosszindulatú daganat kialakulását. A desmoid tumor multidisciplinaris kezelést igényel.

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