Extraabdominalis desmoid tumorral megjeleno Gardner-syndromás beteg kezelésével szerzett hosszú távú tapasztalataink és irodalmi áttekintés.

Zoltán Mátrai, János Papp, Csaba Polgár, Erika Hitre, István Köves, Edit Oláh, Judit Andi, Andrea Kiss, István Vámosi Nagy, László Tóth, Zsolt Orosz

Research output: Review article

2 Citations (Scopus)

Abstract

Gardner's syndrome is a clinical subgroup of familial adenomatous polyposis, an autosomal dominant disease. It is characterized by gastrointestinal polyps and extra-intestinal manifestations including multiple osteomas, skin and soft tissue tumours. Aggressive desmoid tumours can be very difficult to manage in patients with Gardner's syndrome. We present a case of a 17-year-old female who presented with an aggressive desmoid tumor arising of the lumbar area as part of her Gardner's syndrome. She was treated with surgery, nonsteroidal anti-inflammatory drugs, tamoxifen and radiotherapy, and was followed up for 80 months. We conclude that desmoid tumors can precede gastrointestinal manifestations of familial adenomatous polyposis or Gardner's syndrome. Such patients should be evaluated with genetic testing followed by colonoscopy. Desmoid tumours should be managed in a multidisciplinary setting, as well.

Translated title of the contributionLong-term experience with therapy of a female patient with Gardner's syndrome, first presenting with extra-abdominal desmoid tumor, and review of the literature
Original languageHungarian
Pages (from-to)75-82
Number of pages8
JournalMagyar sebészet
Volume62
Issue number2
DOIs
Publication statusPublished - ápr. 2009

ASJC Scopus subject areas

  • Medicine(all)

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