adenoid ameloblastoma

  • 文章类型: Systematic Review
    背景:最近,已经描述了一种新的牙源性肿瘤,所谓的腺样体成釉细胞瘤(AdAM)。这篇综述的目的是确定AdAM的临床和影像学特征,并描述其主要的组织病理学发现。
    方法:系统评价包括已发表的在颌骨中诊断为AdAM的病例,有足够的临床,成像,和组织病理学数据来证实其诊断。采用以下组织病理学诊断标准:成釉细胞瘤样成分的存在,管道状结构,螺旋细胞凝聚,和网状建筑。
    结果:15篇文章,对应于30例AdAM,被选中。大多数病例影响男性(63.3%),轻微偏爱下颌骨(16:14),颌骨的后部区域是最常见的受影响部位。诊断时的平均年龄为40.8岁。临床上,病变通常表现为肿胀(53.3%),射线照相,作为一个明确的射线可透性(33.4%)。手术切除(40%)是最常用的治疗方法,30%的病例复发。显微镜检查显示大多数AdAM病例(93.3%)的筛状区域;在100%的病例中可见导管状结构和螺旋细胞凝结。
    结论:报告病例数量少,错误诊断的存在,并且采用初始保守治疗使得难以确定AdAM是否比常规成釉细胞瘤具有更高的复发风险或更具侵袭性的生物学行为。
    BACKGROUND: Recently, a new odontogenic tumor has been described, the so-called adenoid ameloblastoma (AdAM). The aim of this review was to determine the clinical and imaging features of AdAM and to describe its main histopathological findings.
    METHODS: The systematic review included published cases with a diagnosis of AdAM in the gnathic bones, which had sufficient clinical, imaging, and histopathological data to confirm its diagnosis. The following histopathological diagnostic criteria were adopted: presence of ameloblastoma-like components, duct-like structures, spiral cellular condensations, and a cribriform architecture.
    RESULTS: Fifteen articles, corresponding to 30 cases of AdAM, were selected. Most cases affected men (63.3%), with a slight preference for the mandible (16:14) and the posterior region of gnathic bones was the most commonly affected site. The mean age at diagnosis was 40.8 years. Clinically, the lesions usually presented as a swelling (53.3%) and, radiographically, as a well-defined radiolucency (33.4%). Surgical resection (40%) was the most frequently adopted treatment and recurrence occurred in 30% of cases. Microscopic examination showed cribriform areas in most AdAM cases (93.3%); duct-like structures and spiral cellular condensations were seen in 100% of the cases.
    CONCLUSIONS: The small number of reported cases, the existence of erroneous diagnoses, and the adoption of initial conservative management make it difficult to determine whether AdAM has a higher risk of recurrence or more aggressive biological behavior than conventional ameloblastomas.
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  • 文章类型: Review
    背景:世界卫生组织(WHO)关于牙源性和颌面骨肿瘤的章节为这些肿瘤的诊断提供了全球参考。在第五版中,纳入共识定义和制定必要和理想的诊断标准有助于提高对不同实体的认识.这些是关键的增强,因为牙源性肿瘤的诊断主要基于组织形态学,并结合临床和影像学表现。
    方法:审查。
    结果:尽管有成釉细胞瘤的诊断标准,腺样成釉细胞瘤,和牙本质鬼细胞瘤,这些肿瘤的一部分继续显示重叠的组织学特征,这可能导致误诊.准确的分类可能是具有挑战性的小活检,但可能通过完善现有的诊断标准和在特定病例中使用免疫组织化学和/或分子技术来增强。很明显,钙化上皮牙源性肿瘤的非钙化朗格汉斯细胞丰富亚型和牙源性纤维瘤的淀粉样蛋白丰富变体的临床和组织学特征融合为单个肿瘤描述。此外,这个肿瘤显示出显著的临床,组织学上与位于上颌骨的硬化性牙源性癌的子集重叠。良性神经周受累与神经周受累是牙源性肿瘤中一个未充分开发的概念,需要澄清以减少与硬化性牙源性癌的诊断混淆。
    结论:虽然在WHO章节中讨论了围绕分类和离散肿瘤实体的有争议的问题,歧义不可避免地仍然存在。这篇综述将研究几组牙源性肿瘤,以突出持续的知识差距,未满足的需求和未解决的争议。
    BACKGROUND: The World Health Organization\'s (WHO) chapter on odontogenic and maxillofacial bone tumors provides a global reference for diagnosis of these tumors. In the fifth edition, the inclusion of consensus definitions and development of essential and desirable diagnostic criteria help improve recognition of distinct entities. These are key enhancements since the diagnosis of odontogenic tumors is largely based on histomorphology which is taken in combination with clinical and radiographic appearances.
    METHODS: Review.
    RESULTS: Despite delineation of diagnostic criteria for ameloblastoma, adenoid ameloblastoma, and dentinogenic ghost cell tumor, a subset of these tumors continues to show overlapping histological features that can potentially lead to misdiagnosis. Accurate classification may be challenging on small biopsies, but potentially enhanced by refining existing diagnostic criteria and utilization of immunohistochemistry and/or molecular techniques in a specific cases. It has become clear that the clinical and histologic features of the non-calcifying Langerhans cell-rich subtype of calcifying epithelial odontogenic tumor and the amyloid-rich variant of odontogenic fibroma converge into a single tumor description. In addition, this tumor shows remarkable clinical, histological overlap with a subset of sclerosing odontogenic carcinoma located in the maxilla. Benign perineural involvement vs perineural invasion is an underexplored concept in odontogenic neoplasia and warrants clarification to reduce diagnostic confusion with sclerosing odontogenic carcinoma.
    CONCLUSIONS: While controversial issues surrounding classification and discrete tumor entities are addressed in the WHO chapter, ambiguities inevitably remain. This review will examine several groups of odontogenic tumors to highlight persistent knowledge gaps, unmet needs and unresolved controversies.
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  • 文章类型: Case Reports
    背景:腺样性成釉细胞瘤(AdAM)是一种经常复发的肿瘤,具有成釉细胞瘤和腺瘤样牙源性肿瘤(AOT)的混合组织学特征。在世界卫生组织(WHO)牙源性肿瘤分类的下一次修订中,AdAM有望被归类为成釉细胞瘤的新亚型。然而,AdAM是否是成釉细胞瘤或AOT的组织学变异尚不清楚.为了建立一个新的类别,表明肿瘤类别的遗传证据是必要的。
    方法:我们介绍了一例23岁的日本女性AdAM患者,使用免疫组织化学染色对成釉细胞瘤相关突变进行了遗传/DNA分析,桑格测序,和下一代测序(NGS)分析具有可靠的临床病理证据。
    结果:BRAFp.V600E的免疫组织化学表达对成釉细胞瘤和AOT样成分均呈弥漫性阳性。Sanger测序和NGS分析显示BRAFp.V600E中存在错义突变(c.1799T>A),一种在成釉细胞瘤中通常改变但在KRAS中没有改变的基因,另一个与AOT相关的基因。
    结论:该病例报告首次提供了具有BRAFp.V600E突变的AdAM成釉细胞瘤起源的遗传学证据。需要进行更多的AdAM组分子检测,以恰当地对它们进行分类并预测最佳治疗方案。
    BACKGROUND: Adenoid ameloblastoma (AdAM) is a frequently recurrent tumor that shows hybrid histological features of both ameloblastoma and adenomatoid odontogenic tumor (AOT). AdAM is expected to be classified as a new subtype of ameloblastoma in the next revision of the World Health Organization (WHO) odontogenic tumor classification. However, whether AdAM is a histologic variant of ameloblastoma or AOT remains unclear. To establish a new category, genetic evidence indicating the tumor category is necessary.
    METHODS: We present a case of a 23-year-old Japanese woman with AdAM who underwent genetic/DNA analysis for ameloblastoma-related mutation using immunohistochemical staining, Sanger sequencing, and next-generation sequencing (NGS) analyses with reliable clinicopathological evidence.
    RESULTS: Immunohistochemical expression of BRAF p.V600E was diffusely positive for both ameloblastoma- and AOT-like components. Sanger sequencing and NGS analyses showed missense mutations in BRAF p.V600E (c.1799T > A), a gene that is commonly altered in ameloblastomas but not in KRAS, another gene associated with AOT.
    CONCLUSIONS: This case report is the first to provide genetic evidence on the ameloblastomatous origin of AdAM with a BRAF p.V600E mutation. A larger series of AdAM groups\' molecular testing is needed to aptly classify them and prognosticate the best treatment.
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  • 文章类型: Review
    背景:最近发表在《头颈部病理学》上的一项系统评价发现,3.8%的牙源性鬼细胞瘤具有导管样/筛状结构。在此,我们讨论有关该肿瘤与腺样成釉细胞瘤的鉴别诊断的这一发现。
    方法:对最近发表在《头颈部病理学杂志》上的一篇论文中报道的一些微观发现进行了严格审查。
    结果:尽管牙本质鬼细胞瘤有重叠的微观特征,腺样体成釉细胞瘤的特征在于导管样结构和螺纹/磨牙的组合。在我们看来,至少一些以前诊断为牙源性鬼细胞瘤的病例现在可以更准确地分类为腺样成釉细胞瘤。
    结论:我们得出结论,使用新的WHO头颈部肿瘤分类(2022)提出的诊断标准重新评估牙源性鬼细胞瘤病例是必要的。
    BACKGROUND: A recent systematic review published in Head and Neck Pathology found that 3.8% of dentinogenic ghost cell tumors harbor duct-like/ cribriform architecture. Herein we discuss this finding regarding the differential diagnosis of this tumor with adenoid ameloblastoma.
    METHODS: A critical review of some microscopic findings reported in a recent paper published in the Head and Neck Pathology Journal was done.
    RESULTS: Although there are overlapping microscopic features with dentinogenic ghost cell tumor, adenoid ameloblastoma is distinguished by the combination of duct-like structures and whorls/morules. In our opinion, at least some cases previously diagnosed as dentinogenic ghost cell tumors may now be more accurately classified as adenoid ameloblastoma.
    CONCLUSIONS: We conclude that a reassessment of dentinogenic ghost cell tumor cases using the diagnostic criteria proposed by the new WHO classification of Head and Neck Tumors (2022) is warranted.
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  • 文章类型: Journal Article
    腺样成釉细胞瘤是一种杂合牙源性肿瘤,显示成釉细胞瘤和腺瘤样牙源性肿瘤(AOT)的组织病理学特征,文献报道了大约40例。该报告的目的是使用三个新病例说明腺样体成釉细胞瘤的诊断挑战,并分析文献中的证据,将腺样体成釉细胞瘤视为成釉细胞瘤的新亚型。对关键词腺样体成釉细胞瘤进行了文献综述,杂合/复合牙源性肿瘤,杂合成釉细胞瘤和腺瘤样牙源性肿瘤,成釉细胞瘤具有诱导性变化,选择与牙本质和牙本质瘤诊断相符的病例。在文献报道的40例病例中,对31例资料充足的病例和3例新病例进行分析。在34例腺样成釉细胞瘤中,大多数肿瘤(76.5%)发生在年龄为25至55岁的成年人中。观察到轻度女性偏爱,男女比例为0.9:1。大约,64.7%发生在下颌骨。放射学上,82.4%的腺样成釉细胞瘤表现为射线可透性病变,而47.1%的患者在诊断时出现边缘不明确和皮质穿孔。组织病理学,70.8%的肿瘤表现为丛状成釉细胞瘤,而导管样结构/腺体结构是支持腺瘤样牙源性肿瘤的最常见特征,在95.9%的腺样成釉细胞瘤中观察到。91.6%的肿瘤显示出牙样形式的诱导性变化。Further,45.4%的肿瘤在手术切除后至少复发一次。该报告提供了基于文献综述的证据来显示腺样体成釉细胞瘤的存在,其在人口统计学上与常规成釉细胞瘤相似,但具有组织病理学差异,并表现为更高的发生率/多次复发,表明其生物攻击性。因此,我们建议在下一次修订的WHO牙源性肿瘤分类中,将腺样成釉细胞瘤作为成釉细胞瘤的亚型。
    Adenoid ameloblastoma is a hybrid odontogenic tumour showing histopathological features of both ameloblastoma and adenomatoid odontogenic tumour (AOT), with approximately 40 cases reported in the literature. The aims of the report are to illustrate the diagnostic challenges of adenoid ameloblastoma using three new cases and to analyze evidence in literature to consider adenoid ameloblastoma as a new sub type of ameloblastoma. A literature review was performed with the key words-adenoid ameloblastoma, hybrid/composite odontogenic tumours, hybrid ameloblastoma and adenomatoid odontogenic tumour, ameloblastoma with inductive changes, dentinoid and dentinoma to select the cases compatible with the diagnosis of adenoid ameloblastoma. Out of the 40 cases reported in literature, 31 cases with sufficient information and 3 new cases were analyzed. Out of the 34 adenoid ameloblastomas majority of tumours (76.5%) occurred in adults with age ranging from 25 to 55 years. Slight female predilection with a male:female ratio of 0.9:1 was observed. Approximately, 64.7% occurred in the mandible. Radiologically, 82.4% of adenoid ameloblastomas presented as radiolucent lesions while 47.1% occurred with ill-defined margins and cortical perforation at diagnosis. Histopathologically, 70.8% of tumours presented as plexiform ameloblastomas, while duct like structures/glandular structures were the commonest feature supportive of adenomatoid odontogenic tumour observed in overwhelming majority of 95.9% of adenoid ameloblastomas. 91.6% of tumours showed inductive change in the form of dentinoid. Further, 45.4% of the tumours developed at least one recurrence following surgical excision. The report presents literature review based evidence to show the existence of adenoid ameloblastoma, which is demographically similar to conventional ameloblastoma but with histopathological differences and presenting with higher rate/multiple recurrences, indicating its biological aggressiveness. Thus, we would like to propose the inclusion of adenoid ameloblastoma as a sub type of ameloblastoma in the next revision of the WHO odontogenic tumour classification.
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  • 文章类型: Journal Article
    Odontogenic tumors are rare entities, often derived from the epithelial remnants in the gnathic bones following odontogenesis. This brief manuscript will seek to address recent developments pertaining to odontogenic tumors as well as particularly uncommon odontogenic tumors and the difficulties in their diagnosis.
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