Bibliographic Details
| Title: |
Gardner Syndrome with Extra and Intra-Abdominal Desmoid Tumors and Adrenal Involvement: PET/CT Findings. |
| Alternate Title: |
İntra-Abdominal ve Ekstra-Abdominal Dezmoid Tümörler ve Adrenal Tutulumla Birlikte Gardner Sendromu: PET/BT Bulguları. |
| Authors: |
Alshammari, Alshaima1 alshaima_97@hotmail.com, Ashkanani, Rasha1, Alabsi, Saker1, Ghanem, Mohammed1 |
| Source: |
Molecular Imaging & Radionuclide Therapy. 2015 Supplement, Vol. 24, p38-41. 4p. |
| Subject Terms: |
*GARDNER syndrome, *COMPUTED tomography, *DESMOID tumors, *POSITRON emission tomography, *ADRENAL cortex, *COLON polyps |
| Abstract (English): |
Gardner syndrome, also known as familial colorectal polyposis, is an autosomal dominant disease and is part of the spectrum of familial adenomatous polyposis (FAP) syndromes. It is characterized by the presence of multiple polyps in the colon together with neoplasms outside the colon. Gardner syndrome is also associated with desmoid tumors in approximately 15% of affected individuals. Desmoid tumors are rare benign slow-growing neoplasms, arising from connective tissue (musculoaponeurotic fibrous tissue). Due to their locally aggressive nature, the term aggressive fibromatosis have been used to describe these tumors. Seven percent of patients with FAP or its variants may also have adrenal masses, most being benign and asymptomatic. We report the PET/CT of a 42 year old patient who was diagnosed with Gardner syndrome, demonstrating the rare coexistence of the intestinal polyps, extra and intra-abdominal desmoids and adrenal adenoma by PET/CT. In addition, the characteristic cutaneous lesions and osteomas of the skull were also identified. [ABSTRACT FROM AUTHOR] |
| Abstract (Turkish): |
Ailesel kolorektal polipozis olarak da bilinen Gardner sendromu otozomal dominant bir hastalık olup ailesel adenomatöz polipozis (AAP) spektrumunun bir parçasıdır. Kolonda çok sayıda poliplerle birlikte kolon dışında neoplazmların varlığı ile karakterizedir. Gardner sendromu aynı zamanda, etkilenen bireylerin %15'inde dezmoid tümörlerle de ilişkilidir. Dezmoid tümörler nadir görülen, benign, yavaş büyüyen neplazmlar olup bağ dokudan (muküloaponevrotik fibröz doku) gelişmektedir. Lokal agresif karakterlerinden dolayı bu tümörleri tanımlamak için agresif fibromatözler terimi kullanılmıştır. AAP veya varyantlarına sahip hastaların %7'sinde adrenal kitle de bulunmakta olup çoğu benign ve asemptomatiktir. Burada, Gardner sendromu olan 42 yaşında bir hastanın intestinal polip, ekstra- ve intra- abdominal dezmoidler ve adrenal adenomun nadir görülen birlikteliğini gösteren PET/BT sonucunu bildirmekteyiz. Ayrıca, karakteristik kutanöz lezyonlar ve kafatası osteomları da bulunmaktadır. [ABSTRACT FROM AUTHOR] |
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