Figueredo Junior José Martins, Vanini Jaqueline Vaz, Carvalho Giovanna Lopes, Ribeiro Ramon, Heguedusch Daniele, Pinto Junior Décio Dos Santos, Shinohara Elio Hitoshi, Nunes Fabio Daumas
Department of Pathology, School of Dentistry, Universidade de São Paulo, Av. Prof. Lineu Prestes, 2227 - Cidade Universitária, São Paulo, SP, 05508-000, Brazil.
Department of Cariology, Restorative Sciences & Endodontics, School of Dentistry, University of Michigan, Ann Arbor, MI, USA.
Head Neck Pathol. 2025 May 8;19(1):58. doi: 10.1007/s12105-025-01796-w.
The primordial odontogenic tumor (POT) is a rare mixed odontogenic tumor believed to develop during early odontogenesis. It is composed of a fibromyxoid stroma with proliferation of mesenchymal cells surrounded by a layer of columnar cells exhibiting reverse polarity and pale cytoplasm. This article presents a case of primordial odontogenic tumor in the anterior region of the mandible of a 10-year-old male Caucasian patient who presented with an expansive intraosseous lesion in the anterior portion of the mandible and a delay in tooth eruption, with a 1-year history of asymptomatic swelling. An incisional biopsy was performed and sent to an external pathology service that diagnosed as ameloblastoma. The clinician sent the paraffin block and hematoxylin/eosin (H&E) slides for diagnosis review to the University of São Paulo, Oral Pathology Service. The H&E slides revealed an abundant myxoid cell-rich stroma containing a few islands of ameloblastic-like epithelium and areas of loosely arranged stellate/spindle-shaped cells resembling stellate reticulum. The odontogenic-like epithelium was also noted at the periphery of the specimen, as well as discrete areas of hyalinization. Cellular pleomorphism and mitotic figures were not observed, supporting the diagnosis of ameloblastic fibroma. The clinician proceeded with surgical excision and sent us the whole specimen. After reviewing the slides and discussing the case, the final diagnosis was primordial odontogenic tumor. This report is relevant to provide more information for clinicians and pathologists about this rare entity.
原始牙源性肿瘤（POT）是一种罕见的混合性牙源性肿瘤，被认为在早期牙胚发生过程中形成。它由纤维黏液样基质组成，其间充质细胞增生，周围有一层柱状细胞，这些柱状细胞呈现反向极性且细胞质淡染。本文报告了一例10岁白种男性患者下颌前部原始牙源性肿瘤的病例。该患者下颌前部出现骨内膨胀性病变，伴有牙齿萌出延迟，有1年无症状肿胀病史。进行了切开活检，并将标本送至外部病理科，诊断为成釉细胞瘤。临床医生将石蜡块和苏木精/伊红（H&E）切片送至圣保罗大学口腔病理科进行诊断复核。H&E切片显示富含黏液样细胞的丰富基质，其中含有一些成釉细胞样上皮岛以及类似星网状层的松散排列的星状/梭形细胞区域。在标本周边还可见牙源性样上皮以及散在的玻璃样变区域。未观察到细胞多形性和有丝分裂象，支持成釉细胞纤维瘤的诊断。临床医生进行了手术切除，并将整个标本送交给我们。在复查切片并讨论该病例后，最终诊断为原始牙源性肿瘤。本报告旨在为临床医生和病理学家提供更多关于这种罕见疾病的信息。
