Rafiq Suhail, Manzoor Farzana, Dar Musaib Ahmad, Aslam Rassieq
Department of Radiodiagnosis, Government Medical College, Srinagar, Jammu and Kashmir, India.
Department of Pathology, Government Medical College, Srinagar, Jammu and Kashmir, India.
J Oral Maxillofac Pathol. 2021 May-Aug;25(2):373. doi: 10.4103/0973-029X.325261. Epub 2021 Aug 31.
Gorlin-Goltz syndrome (GGS) is a rare autosomal dominant disorder with multisystemic involvement. It is characterized by the triad of multiple baso-cellular epitheliomas, odontogenic keratocysts (OKC) in the jaws and skeletal anomalies. Later, it was found that calcification of falx is also highly specific. We present radiological findings in case series of two cases, one with multiple OKC, calcified falx, skin lesions, and fibrous dysplasia of sphenoid and second with multiple OKC, calcified falx, vertebral anomaly and medulloblastoma.
戈林-戈尔茨综合征(GGS)是一种罕见的常染色体显性疾病,累及多系统。其特征为三联征,即多发性基底细胞上皮瘤、颌骨牙源性角化囊肿(OKC)和骨骼异常。后来发现,大脑镰钙化也具有高度特异性。我们展示了两个病例系列的影像学表现,一例有多发性OKC、大脑镰钙化、皮肤病变和蝶骨纤维发育不良,另一例有多发性OKC、大脑镰钙化、椎体异常和髓母细胞瘤。