dentinogenic ghost cell tumour

  • 文章类型: Journal Article
    最近,在世界卫生组织(WHO)对头颈部肿瘤的最新分类中,腺样体成釉细胞瘤(AA)被认为是一种单独的肿瘤类型。这个决定被几个团体认为是有争议的,他们将AA描述为成釉细胞瘤的一种亚型,杂合牙源性肿瘤或属于其他公认的牙源性肿瘤,包括牙源性鬼细胞瘤和腺瘤样牙源性肿瘤。在这里,我们回顾了WHO决定将AA分类为单独肿瘤类型的原因。我们还批评了自WHO分类以来发表的最新报告的分子和组织学发现。虽然承认肿瘤的分类在不断发展,当前证据的平衡表明AA应该仍然是一种独特的肿瘤类型,而不是成釉细胞瘤的亚型,等待进一步的分子表征。
    Adenoid ameloblastoma (AA) was recently recognised as a separate tumour type in the most recent World Health Organisation (WHO) classification of head and neck tumours. This decision has been considered controversial by several groups, who have described AA as a subtype of ameloblastoma, a hybrid odontogenic tumour or to fall within the spectrum of other recognised odontogenic tumours, including dentinogenic ghost cell tumour and adenomatoid odontogenic tumour. Here we review the reasons for the WHO decision to classify AA as a separate tumour type. We also critique molecular and histological findings from recent reports published since the WHO classification. While acknowledging that the classification of tumours is constantly evolving, the balance of current evidence suggests that AA should remain a distinct tumour type, and not a subtype of ameloblastoma, pending further molecular characterisation.
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  • 文章类型: Journal Article
    尽管不常见的牙源性鬼细胞瘤(DGCT)是一种良性实体,它具有引起颌骨广泛破坏并在保留骨治疗后复发的能力。因此,手术后应达到清晰的边缘,重建技术必须经常用于修复骨缺损。然而,在涉及颞下颌关节(TMJ)的情况下,这可能是具有挑战性的,尤其是儿童。
    我们介绍了一个12岁男孩的DGCT病例,下颌骨和TMJ广泛浸润。进行了两阶段的重建方法。在初次手术时,获得了无瘤边缘,并使用a骨移植物和同种异体髁植入物修复了下颌骨解剖结构,以进行临时TMJ重建。在5个月后的第二步中,收到了由窝和髁组件组成的定制TMJ假体,TMJ被完全替换为最终的重建。
    定制的TMJ假体可能是儿童TMJ重建的解决方案。然而,关于生长障碍的进一步过程必须在短期随访中进行评估,并且可能需要额外的纠正干预措施。
    UNASSIGNED: Although the uncommon dentinogenic ghost cell tumour (DGCT) is a benign entity, it possesses the ability to cause widespread destruction of the jaws and to recur after bone-preserving therapy. Hence, clear margins should be achieved upon surgery, and reconstruction techniques must often be used to restore osseous defects. However, this can be challenging in cases with involvement of the temporomandibular joint (TMJ), and especially in children.
    UNASSIGNED: We present a case of a DGCT in a 12-year-old boy with wide infiltration of the mandible and the TMJ. A two-staged reconstructive approach was performed. Upon primary surgery, tumour-free margins were obtained and mandibular anatomy was restored using an iliac crest graft and an alloplastic condyle implant for temporary TMJ reconstruction. In a second step 5 months later, having received a customized TMJ prosthesis consisting of a fossa and a condyle component, the TMJ was completely replaced for definitive reconstruction.
    UNASSIGNED: A customized TMJ prosthesis could be a solution for reconstruction of the TMJ in children. However, the further course with respect to growth disturbances must be evaluated upon short-term follow-ups and might require additional corrective interventions.
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  • 文章类型: Case Reports
    异位牙源性肿瘤罕见且难以诊断。因此,他们偶尔被误诊为其他肿瘤和过度治疗。牙本质鬼细胞肿瘤(DGCT)是以CTNNB1突变为特征的牙源性肿瘤,鬼细胞外观,牙本质样钙化.在这里,我们介绍了一例患者口腔底部的异位DGCT,首次为其牙源性提供可靠的临床病理和遗传证据。
    一名72岁男子表现为无痛性舌下肿胀。成像显示一个多叶,他嘴底的囊性肿块.细胞学评估显示由基底细胞组成的折叠上皮簇,角化材料,和钙化。组织学分析显示多囊性,冠状到坚固的巢,有牙源性卫星状网状上皮,包括鬼影细胞和牙质基质沉积。免疫组织化学分析发现CK19、CK5/6、bcl-2、p63为弥漫性阳性,β-连环蛋白在细胞核中呈局灶性阳性,牙本质基质中的细胞对DMP1呈阳性。检测到CTNTTB1突变,导致最终诊断为异位DGCT。随访6个月无复发。
    总的来说,我们对DGCT进行了全面的临床概述,并确定了其病理和遗传特征。该报告将有助于将来认识这种罕见疾病,并有助于避免误诊和过度治疗。
    Ectopic odontogenic tumours are rare and difficult to diagnose. Consequently, they are occasionally misdiagnosed as other tumours and overtreated. Dentinogenic ghost cell tumours (DGCTs) are odontogenic neoplasms characterised by a CTNNB1 mutation, ghost cell appearance, and dentinoid-like calcification. Herein, we present a case of ectopic DGCT on the floor of a patient\'s mouth, providing reliable clinicopathological and genetic evidence of its odontogenicity for the first time.
    A 72-year-old man presented with painless sublingual swelling. Imaging revealed a multi-lobulated, solid-cystic mass on the floor of his mouth. Cytological evaluation showed folded epithelial clusters composed of basaloid cells, keratinised material, and calcification. Histological analysis revealed a multi-cystic, cribriform to solid nest, with an odontogenic satellate reticulum-like epithelium, including ghost cells and dentinoid matrix deposition. Immunohistochemical analysis found that CK19, CK5/6, bcl-2, and p63 were diffuse positive, β-catenin was focal positive in the nuclei, and the cells in the dentinoid matrix were positive for DMP1. The CTNTTB1 mutation was detected, leading to the final diagnosis of ectopic DGCT. There was no recurrence during the 6-month follow-up.
    Overall, we have presented a comprehensive clinical overview of DGCT and identified its pathological and genetic features. This report will aid in the recognition of this rare disease in the future and help to avoid misdiagnosis and overtreatment.
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