Numéro |
Med Sci (Paris)
Volume 34, October 2018
Cancer biomarkers
|
|
---|---|---|
Page(s) | 20 - 25 | |
DOI | https://doi.org/10.1051/medsci/201834f104 | |
Publié en ligne | 7 novembre 2018 |
Bone and dental abnormalities as first signs of familial Gardner’s syndrome in a Chinese family: a literature review and a case report
1
M.S., D.D.S., Attending doctor, Department of Oral and Maxillofacial Surgery, First Affiliated Hospital, College of Medicine, Zhejiang University. 79# Qingchun Road, Hangzhou 310003, People’s Republic of China
2
B.D.S., Resident, Department of Oral and Maxillofacial Surgery, National Dental Centre Singapore. Second Hospital Avenue, 168938, Singapore
3
M.D., Ph.D., Professor, Department of Oral and Maxillofacial Surgery, First Affiliated Hospital, College of Medicine, Zhejiang University. 79# Qingchun Road, Hangzhou 310003, People’s Republic of China
4
M.D., Ph.D., Professor and Chairman, Department of Oral and Maxillofacial Surgery, First Affiliated Hospital, College of Medicine, Zhejiang University. 79# Qingchun Road, Hangzhou 310003, People’s Republic of China
5
M.S., D.D.S., Attending doctor, Department of Oral and Maxillofacial Surgery, First Affiliated Hospital, College of Medicine, Zhejiang University. 79# Qingchun Road, Hangzhou 310003, People’s Republic of China
* Corresponding author: Yi Lin 1507155@zju.edu.cn
Gardner’s syndrome (GS) is an autosomal dominant disease characterized by the presence of familial adenomatous polyposis (FAP) as well as extraintestinal manifestations such as osteomas, dental anomalies, epidermoid cysts and ocular abnormalities. These intestinal polyps carry a 100% risk of malignant change, so early diagnosis is crucial. As craniofacial osteomas and dental anomalies of GS usually precede gastrointestinal symptoms, otolaryngologists, oral surgeons and dentists play an important role in the diagnosis of GS. GS is extensively reported in literature in the Caucasian race but not in the Mongoloid race. We report a case of a 22-year-old patient with a manifestation of three features of GS - multiple osteomas, soft tissue tumors and dental anomalies in the craniofacial region, with no intestinal polyps at the time of reporting. A family pedigree with our patient as the proband was constructed and revealed 3 consecutive generations in his lineage with GS.
Key words: Familial adenomatous polyposis / Gardner’s syndrome / osteomas
© 2018 médecine/sciences – Inserm
Les statistiques affichées correspondent au cumul d'une part des vues des résumés de l'article et d'autre part des vues et téléchargements de l'article plein-texte (PDF, Full-HTML, ePub... selon les formats disponibles) sur la platefome Vision4Press.
Les statistiques sont disponibles avec un délai de 48 à 96 heures et sont mises à jour quotidiennement en semaine.
Le chargement des statistiques peut être long.