Proteus综合征临床病理观察  

Proteus syndrome:a clinicopathological observation

作　　者：陆江阳[1] 王晓虹[1] 刘茜[1] 杨毅[1] 李韶然[1] 

机构地区：[1]解放军总医院第一附属医院病理科

出　　处：《诊断病理学杂志》2007年第5期339-343,共5页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨Proteus综合征(PS)的临床病理特点与鉴别诊断。方法结合文献对1例PS的临床表现与组织病理学特点进行观察和研究。结果本例患者自幼发病,随年龄增长进行性加重,具有多胚层的病理改变,表现为皮肤、骨骼及软组织的不对称过度生长和皮下多发良性肿瘤,本例特征性病变为巨颅和巨大毛囊皮脂腺囊性错构瘤。结论PS是一种罕见的多胚层错构增生综合征,病理检查须多处采取标本,而且要与临床表现密切结合才能做出诊断。Objective To study the clinicopathological characteristics and differential diagnosis of Proteus syndrome. Methods The clinical representations and histopathological characteristics were studied with review of to the literatures. Results The patient had been ill from a child and aggravated with ages. The pathological changes involved two embryolayers and included the asymmetrical overgrowth of skin, bone and soft tissue and several subcutaneous benign tumors. The representative changes of the case were macrocrania and huge folliculosebaceous cystic hamartoma. Conclusion Proteus syndrome is a rare disease, its pathological diagnosis depends on taking sufficient specimens and combination with clinical history and presentations.

关 键 词：变形综合征 巨颅 毛囊皮脂腺囊性错构瘤 病理学 

分 类 号：R739.5[医药卫生—肿瘤]