摘要： Gardner综合征是一种以家族性腺瘤性息肉病（familial adenomatous polyposis，FAP）、多发骨瘤和软组织肿瘤为特点的常染色体显性遗传病。因为肠道腺瘤性息肉的恶变率高达100%，所以早期诊断、早期治疗具有重要意义。本文报道了1例于我院口腔科就诊的Gardner综合征患者，临床表现为直结肠腺瘤性息肉、副鼻窦及下颌骨多发骨瘤和颈部硬纤维瘤。此外，本文还就Gardner综合征的临床表现、诊断、治疗、并发症的预防等方面进行了文献回顾。

关键词: Gardner综合征, 家族性腺瘤性息肉病, 骨瘤, 软组织肿瘤

Abstract:  Gardner syndrome is featured by a combination of familial adenomatous polyposis (FAP), multiple osteomas and soft tissue tumors. The intestinal polyps have a 100% risk of undergoing malignant transformation, therefore early identification of this disease is very important. This article reported a case of a 53?鄄year old male patient with Gardner's syndrome, who had intestinal polyps, multiple osteomas in paranasal sinuses and mandible, as well as desmoid tumor of neck. Literatures regarding clinical features, diagnosis, treatment of Gardner syndrome and prevention of related complications were reviewed.

Key words: Gardner syndrome, familial adenomatous polyposis(FAP), osteomas, soft tissue tumors

中图分类号: 

• R782

• R739.82