Orvosi Hetilap
Volume/Issue:
Volume 150: Issue 41
Long-term experiences with multidisciplinary therapy of twenty-six patients with dermatofibrosarcoma protuberans and review of the literature
Authors: Zoltán Mátrai 1 , Gabriella Liszkay 2 , Vanda Plotár 3 , Zsolt Orosz 3 , Judit Székely 4 , Erika Hitre 5 , Alexandra Bartal 6 , Zoltán Langmár 8 , Katalin Bőcs 7 , Ferenc Rényi Vámos 1 , Ákos Sávolt 1 , and László Tóth 1
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  • 1 Országos Onkológiai Intézet Általános és Mellkassebészeti Osztály Budapest Ráth György u. 7–9. 1122
  • 2 Országos Onkológiai Intézet Onkodermatológiai Osztály Budapest
  • 3 Országos Onkológiai Intézet Sebészi és Molekuláris Daganatpatológiai Centrum Budapest
  • 4 Országos Onkológiai Intézet Sugárterápiás Osztály Budapest
  • 5 Országos Onkológiai Intézet Kemoterápia B Belgyógyászati-Onkológiai és Klinikai Farmakológiai Osztály Budapest
  • 6 Országos Onkológiai Intézet Intézeti Gyógyszertár Budapest
  • 7 Országos Onkológiai Intézet Radiológiai Diagnosztika Budapest
  • 8 Semmelweis Egyetem, Általános Orvostudományi Kar II. Szülészeti és Nőgyógyászati Klinika Budapest
DOI:
https://doi.org/10.1556/oh.2009.28702
Page Count:
1894–1902
Publication Date:
01 Oct 2009
Online Publication Date:
02 Oct 2009
Article Category:
Research Article
Open access

A dermatofibrosarcoma protuberans alacsony vagy közepes malignitású, ritka, rosszindulatú daganat. A tumort a lassú, de agresszív lokális növekedés, az alacsony áttétképzési és a magas helyi kiújulási arány jellemzi. Az elsődleges kezelés a radikális sebészi eltávolítás hagyományos kimetszéssel vagy Mohs-féle sebészi technikával. Pozitív sebészi szél, recidív tumor esetén radio-, kemoterápia, illetve újabban imatinib mesylat alkalmazható. Célkitűzés: A szerzők 26, dermatofibrosarcoma protuberans miatt onkológiai centrumban multidiszciplinárisan kezelt beteg hosszú távú klinikopatológiai utánkövetését végezték. Módszer és eredmények: A betegek átlagéletkora 44,7 év volt. Az utánkövetés átlagideje 60,57 hónap volt. Tizenöt betegnél (57,7%) sikerült R0 eltávolítást végezni, míg 11 betegnél (42,3%) csak R1 reszekciót. Az R0 reszekciók eléréséhez átlagosan 1,87 kimetszésre volt szükség. Adjuváns kezelésként a primer tumor eltávolítását követően 6 beteg (23%) részesült radioterápiában és 2 (7,6%) beteg kemoterápiában. Tizenhat betegnél nem észleltünk kiújulást. Tíz betegnél (38,4%) alakult ki recidíva, amely miatt további kezeléseket folytattunk. Egy beteg távoli áttétek miatt elhunyt. Statisztikai módszerekkel vizsgáltuk az irodalomban prognosztikai faktornak számító 50 év feletti életkor és a sebészi radikalitás hatását a helyi kiújulásra. Következtetések: A dermatofibrosarcoma protuberans multidiszciplináris kezeléssel eredményesen kezelhető daganat. Pontosabb következtetések levonásához nagyobb esetszám és multicentrikus randomizált vizsgálatok szükségesek.

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Cover Orvosi Hetilap
Orvosi Hetilap
Print ISSN:
0030-6002
Online ISSN:
1788-6120
Keywords:
dermatofibrosarcoma protuberans; Mohs-féle mikrográfikus sebészet; radioterápia; imatinib mesylat; dermatofibrosarcoma protuberans; Mohs micrographic surgery; radiotherapy; imatinib mesylate

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