Lazaridou Maria Nikolaou, Dimitrakopoulos Ioannis, Tilaveridis Ioannis, Iliopoulos Christos, Heva Antigoni
Department of Oral and Maxillofacial Surgery, G. Papanikolaou Hospital, Exohi, Thessaloniki, Ioanni Hatzoudi 9, Neapoli, 56727 Thessaloniki, Greece.
Oral Maxillofac Surg. 2012 Mar;16(1):127-31. doi: 10.1007/s10006-011-0270-0. Epub 2011 Apr 6.
Gorlin-Goltz syndrome, also known as nevoid basal cell carcinoma syndrome, is an autosomal dominant inherited disorder which is characterized by the presence of multiple basal cell carcinomas, maxillary keratocysts, and musculoskeletal anomalies.
We present a case of a patient suffering from Gorlin-Goltz syndrome who developed an intraosseous basal cell carcinoma associated with a recurrent maxillary keratocyst. To our knowledge, this is the first case of malignant transformation of a keratocyst into a basal cell carcinoma described in the literature.
This case highlights the importance of careful histologic examination of keratocysts excised in patients suffering from Gorlin-Goltz syndrome.
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性疾病，其特征为存在多发性基底细胞癌、上颌角化囊肿和肌肉骨骼异常。
我们报告一例患有戈林-戈尔茨综合征的患者，该患者发生了与复发性上颌角化囊肿相关的骨内基底细胞癌。据我们所知，这是文献中描述的首例角化囊肿恶变为基底细胞癌的病例。
该病例突出了对戈林-戈尔茨综合征患者切除的角化囊肿进行仔细组织学检查的重要性。
