Bello Ibrahim Olajide
Department of Oral Medicine and Diagnostic Sciences, College of Dentistry, King Saud University, Riyadh, Saudi Arabia.
Saudi Dent J. 2024 Jan;36(1):38-43. doi: 10.1016/j.sdentj.2023.10.012. Epub 2023 Oct 18.
Odontogenic keratocysts (OKCs) are a common presentation in almost all patients with nevoid basal cell carcinoma syndrome (NBCCS) or Gorlin syndrome, irrespective of race. In most patients with NBCCS, OKC presents as multiple lesions affecting the jaws which makes it a signpost for the investigation of patients with the syndrome. In approximately 40% of pediatric patients, the initial presentation is that of a single OKC, which may often result in missing the diagnosis of NBCCS. This is particularly common in patients without clinically apparent NBCCS-related manifestations. This review examines the clinicopathological features that clinicians and oral pathologists may look for in pediatric patient with OKC and OKC surgical specimens that may serve as indicators for the diagnosis of NBCCS. Although these features do not diagnose NBCCS by themselves, they may significantly help in initiating the diagnostic process at an early stage with an obvious benefit to the child and relatives.
牙源性角化囊肿（OKCs）在几乎所有患有痣样基底细胞癌综合征（NBCCS）或戈林综合征的患者中都很常见，无论种族如何。在大多数NBCCS患者中，OKC表现为影响颌骨的多个病变，这使其成为该综合征患者检查的一个标志。在大约40%的儿科患者中，最初表现为单个OKC，这可能常常导致漏诊NBCCS。这在没有明显临床NBCCS相关表现的患者中尤为常见。本综述探讨了临床医生和口腔病理学家在患有OKC的儿科患者以及OKC手术标本中可能寻找的临床病理特征，这些特征可作为NBCCS诊断的指标。虽然这些特征本身不能诊断NBCCS，但它们可能显著有助于在早期启动诊断过程，对儿童及其亲属有明显益处。
