Ünsal Gürkan, Cicciù Marco, Ayman Ahmad Saleh Rand, Riyadh Ali Hammamy Mohammed, Amer Kadri Anwer, Kuran Bilge, Minervini Giuseppe
Near East University, Department of Dentomaxillofacial Radiology, Cyprus.
Department of General Surgery and Medical-Surgical Specialties, School of Dentistry, University of Catania, 95131 Catania, Italy.
Saudi Dent J. 2023 Sep;35(6):614-624. doi: 10.1016/j.sdentj.2023.05.023. Epub 2023 May 30.
Nevoid Basal Cell Carcinoma Syndrome (NBCCS) is an autosomal dominant syndrome that has various expressions in each patient. Generally; NBCCS is followed by multiple nevoid basal cell carcinoma of the skin, orbital anomalies, skeletal anomalies, central nervous system anomalies and multiple odontogenic keratocysts (OK). NBCCS is usually diagnosed between the ages of 5-30 years, with multiple basal cell carcinomas of the skin and OKs in the jaws as the initial findings. The purpose of this paper is to describe and compare the radiographic findings of the OKs in NBCCS patients in the literature with additional cases.
In this study, we evaluated the OKs of the patients with NBCCS in PubMed Database with 5 additional cases from our database. A total of 305 articles were found and the articles in English with full-text access were evaluated.
Despite all limitations for a fair discussion; we would like to state that among 59 cases that specified whether a 3D or 2D imaging modality was used, 29 cases were only interpreted with 2D data which should be avoided in OK evaluation.
According to the World Health Organization's Classification of Head and Neck Tumours Book which was published in 2017, OKs in NBCCS has a higher chance to have small satellite cystic lesions which increase their recurrence possibility post-operatively, thus, a thorough clinical and 3D radiographic evaluation should be performed both to NBCCS patients and non-syndromic OK patients to avoid any recurrence.
High recurrence rates of OKs should be reminded all the time. Radiographic examinations with 3D imaging modalities should be done in patients with NBCCS in order to provide a concise diagnosis and optimum treatment.
痣样基底细胞癌综合征（NBCCS）是一种常染色体显性综合征，在每位患者中表现各异。一般来说，NBCCS会伴有皮肤多发性痣样基底细胞癌、眼眶异常、骨骼异常、中枢神经系统异常以及多发性牙源性角化囊肿（OK）。NBCCS通常在5至30岁之间被诊断出来，最初的表现为皮肤多发性基底细胞癌和颌骨OK。本文旨在描述并比较文献中NBCCS患者的OK的影像学表现以及其他病例。
在本研究中，我们评估了PubMed数据库中NBCCS患者的OK，并加入了来自我们数据库的另外5个病例。共找到305篇文章，并对可获取全文的英文文章进行了评估。
尽管进行公正讨论存在诸多限制，但我们想说的是，在59例明确说明了使用3D还是2D成像方式的病例中，有29例仅通过2D数据进行解读，而在OK评估中应避免这种情况。
根据2017年出版的世界卫生组织《头颈部肿瘤分类》一书，NBCCS中的OK更有可能出现小的卫星囊性病变，这会增加术后复发的可能性，因此，应对NBCCS患者和非综合征性OK患者进行全面的临床和3D影像学评估，以避免任何复发。
应时刻提醒注意OK的高复发率。对于NBCCS患者，应采用3D成像方式进行影像学检查，以便做出准确诊断并提供最佳治疗。
