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痣样基底细胞癌综合征:1例侵犯筛窦的侵袭性病例报告。

Nevoid basal cell carcinoma syndrome: report of an aggressive case with ethmoid sinus invasion.

作者信息

Tabuchi Keiji, Okubo Hideki, Takahashi Kazuhiko, Uemaetomari Isao, Shimoyamada Hiroaki, Nakayama Yoshio, Hara Akira

机构信息

Department of Otolaryngology, Institute of Clinical Medicine, University of Tsukuba, 1-1-1 Tennodai, 305-8575, Tsukuba, Japan.

出版信息

Auris Nasus Larynx. 2003 Dec;30(4):421-3. doi: 10.1016/s0385-8146(03)00092-0.

Abstract

Nevoid basal cell carcinoma syndrome, also referred to as Gorlin-Goltz syndrome, is a rare autosomal dominant disorder characterized by multiple basal cell carcinomas, jaw cysts, palmar or plantar pits, ectopic calcification of the falx cerebri, and various skeletal developmental abnormalities. A minority of basal cell carcinomas demonstrate aggressive behavior and involve the craniofacial bones in nevoid basal cell carcinoma syndrome. A non-familial case of nevoid basal cell carcinoma syndrome with a basal cell carcinoma of the eyelid invading to the ethmoid sinus is reported.

摘要

痣样基底细胞癌综合征,也称为戈林-戈尔茨综合征,是一种罕见的常染色体显性疾病,其特征为多发性基底细胞癌、颌骨囊肿、掌跖凹、大脑镰异位钙化以及各种骨骼发育异常。少数基底细胞癌表现出侵袭性,在痣样基底细胞癌综合征中累及颅面骨。本文报道了1例非家族性痣样基底细胞癌综合征病例,其眼睑基底细胞癌侵犯至筛窦。

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