Gardner syndrome

Pharyngeal
desmoid-type fibromatosis in a pediatric patient with Gardner"s syndrome. Axial CT image shows an oropharyngeal well circumscribed, soft tissue mass, with similar attenuation than the muscles, and slight enhancement after contrast administration. The airway is almost completely occupied by the mass.
www.eurorad.org • CC by-nc-sa-4.0
Pharyngeal
desmoid-type fibromatosis in a pediatric patient with Gardner"s syndrome. Coronal CT image depicts the same oropharyngeal soft tissue mass.
www.eurorad.org • CC by-nc-sa-4.0
Pharyngeal
desmoid-type fibromatosis in a pediatric patient with Gardner"s syndrome. On T2-weighted axial image the mass is heterogeneous, most of it hyperintense.
www.eurorad.org • CC by-nc-sa-4.0
Pharyngeal
desmoid-type fibromatosis in a pediatric patient with Gardner"s syndrome. T1-weighted sagittal after contrast administration image manifests a solid, well-circumscribed mass with a homogeneous enhancement.
www.eurorad.org • CC by-nc-sa-4.0
Radiological
review of skull lesions. Gardner Syndrome (multiple osteomas). Axial head CT images (a–c) show multiple osteomas in the ethmoid air cells (dashed arrows), left ramus of the mandible (arrowhead) and right anterior wall of the maxillary sinus (thin arrow). This patient was also found to have a mesenteric desmoid tumour (thick arrow, d)
insightsimaging.springeropen.com • Open Access
Radiopaedia • CC-by-nc-sa 3.0 • de

Gardner syndrome is one of the polyposis syndromes. It is characterized by:

Other abnormalities include:

Pathology

There is an autosomal dominant inheritance in the FAP gene (chromosome 5q) in a majority of patients but with 20% of cases resulting from new mutations. Extracolonic features often precede the diagnosis of colonic polyps.

History and etymology

First described in 1953 by Gardner and Richards .

Siehe auch:
und weiter: