Desmoid tümör ve rektum karsinomunun eşlik ettiği familyal adenomatöz polipozis: Olgu sunumu

Şükrü Taş; Mustafa Duman; Ali Atıcı; Feza Ekiz; Tahsin Dalgıç; İlter Özer; Erdal Bostancı; Musa Akoğlu

Desmoid tümör ve rektum karsinomunun eşlik ettiği familyal adenomatöz polipozis: Olgu sunumu

Year 2010, Volume: 32 Issue: 4, 371 - 376, 23.12.2010

Şükrü Taş Mustafa Duman , Ali Atıcı Feza Ekiz Tahsin Dalgıç İlter Özer , Erdal Bostancı Musa Akoğlu

Abstract

Özet

Familiyal adenomatöz polipozis (FAP) gastrointestinal sistemde özellikle kolon ve rektumda çok sayıda adenomatöz poliplerin varlığı ile karakterize otozomal dominant geçiş gösteren bir sendromdur. Bu sendromda; adrenal adenomlar, osteomalar, desmoid tümörler gibi bening tümörler, tiroid ve pankreatik kanserler, hepaoblastomalar, santral sinir sistemi tümörleri ile diş anomalilerinin görülme olasılığı artmıştır. Burada 28 yaşında karın duvarında kitle şikayeti ile başvuran ve FAP zemininde gelişen rektum kanseri ve desmoid tümör tespit edilen hasta sunuldu.

Anahtar sözcükler: Desmoid tümör, familyal adenomatöz polipozis, rektum kanseri

Abstract

Familial adenomatous polyposis (FAP) is an autosomal dominant syndrome, where polyps develop throughout the gastrointestinal tract, particularly in the colon and the rectum. FAP-related complications, for which medical attention is essential, are not rare and their estimated lifetime risk presumably exceeds 30%. Affected individuals can develop thyroid and pancreatic cancer, hepatoblastomas, CNS tumors (especially medulloblastomas), and various benign tumors such as adrenal adenomas, osteomas, desmoid tumors and dental abnormalities.A 28 year old patient who applied with a mass in abdominal wall and found to have rectum cancer and desmoid tumor in the background of FAP is presented in this case report.

Key words: Desmoid tumor, familial adenomatous polyposis, rectal carcinoma

Keywords

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