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痣样基底细胞癌综合征中子宫和卵巢纤维瘤病的影像学特征

Imaging features of uterine and ovarian fibromatosis in Nevoid Basal Cell Carcinoma Syndrome.

作者信息

Scalia Ambra Cassar, Farulla Antonino, Fiocchi Federica, Alboni Carlo, Torricelli Pietro

机构信息

Department of Radiology, Azienda Ospedaliero-Universitaria Policlinico di Modena, Italy.

Department of Obstetrics and Ginecology, Azienda Ospedaliero-Universitaria Policlinico di Modena, Italy.

出版信息

J Radiol Case Rep. 2018 Sep 30;12(9):21-30. doi: 10.3941/jrcr.v12i9.3390. eCollection 2018 Sep.

Abstract

Gorlin-Goltz Syndrome also known as Nevoid Basal Cell Carcinoma Syndrome is an autosomal dominant multisystem disorder. It is characterized by basal cell carcinomas, odontogenic keratocysts, skeletal abnormalities and in a minority of female patients bilateral calcified ovarian fibromas. It is challenging to radiologically assess ovarian fibromas as they have similar imaging patterns to some malignant ovarian lesions. However, it is vitally important to differentiate between benign and malignant lesions to determine patients' suitability for fertility-sparing surgery. This report describes a case of a 25 year-old patient with Gorlin-Goltz Syndrome and bilateral ovarian fibromas.

摘要

戈林-戈尔茨综合征,也称为痣样基底细胞癌综合征,是一种常染色体显性遗传的多系统疾病。其特征为基底细胞癌、牙源性角化囊肿、骨骼异常,少数女性患者还伴有双侧钙化性卵巢纤维瘤。对卵巢纤维瘤进行放射学评估具有挑战性,因为它们的影像学表现与一些恶性卵巢病变相似。然而,区分良性和恶性病变对于确定患者是否适合保留生育功能的手术至关重要。本报告描述了一名25岁患有戈林-戈尔茨综合征和双侧卵巢纤维瘤的患者的病例。

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