Abbas Rezhat, Javaid Nazifa
Department of Oral & Maxillofacial Pathology and Microbiology, Maulana Azad Institute of Dental Sciences, BSZ Marg, New Delhi, India.
Department of Oral & Maxillofacial Pathology and Microbiology, Institute of Dental studies and Technologies, Ghaziabad, Uttar Pradesh, India.
J Stomatol Oral Maxillofac Surg. 2025 Jun 19:102438. doi: 10.1016/j.jormas.2025.102438.
Sclerosing odontogenic carcinoma is a rare and locally invasive malignant tumor of odontogenic origin. Due to its low incidence, the literature is limited to isolated case reports, making comprehensive understanding of its clinicopathologic features and management strategies challenging.
This systematic review aims to synthesize the existing case reports on SOC to elucidate its clinical, radiographic, histopathological, and immunohistochemical characteristics, and to provide insights into current diagnostic and therapeutic approaches.
A comprehensive literature search was conducted across three major databases: PubMed, Scopus, and Embase. A total of 95 records were initially identified (PubMed: n = 36; Scopus: n = 30; Embase: n = 29). After screening and eligibility assessment, 16 studies meeting inclusion criteria were selected for final analysis.
SOC predominantly involved the mandible. The most commonly reported symptom was swelling, either painless or painful. Radiographically, lesions often appeared as ill-defined or poorly marginated radiolucencies, reflecting their infiltrative nature. Frequent cortical bone involvement included thinning, resorption, and perforation. Histopathologically, SOC was characterized by cords, nests, and strands of bland epithelial cells in a densely sclerotic stroma. Immunohistochemically, tumors showed strong expression of high molecular weight cytokeratins (CK5/6, CK19, p63, AE1/AE3). The primary treatment modality was wide local excision with clear margins, commonly involving segmental mandibulectomy or maxillectomy based on tumor extent.
SOC is an exceptionally rare odontogenic malignancy with consistent clinical and pathological features across reported cases. Its locally aggressive nature underscores the importance of early diagnosis and wide surgical excision. This review consolidates existing case data to enhance understanding and aid clinicians in recognizing and managing this challenging neoplasm.
硬化性牙源性癌是一种罕见的、具有局部侵袭性的牙源性恶性肿瘤。由于其发病率低，文献限于孤立的病例报告，全面了解其临床病理特征和治疗策略具有挑战性。
本系统评价旨在综合现有的关于硬化性牙源性癌的病例报告，以阐明其临床、影像学、组织病理学和免疫组化特征，并深入了解当前的诊断和治疗方法。
在三个主要数据库（PubMed、Scopus和Embase）中进行了全面的文献检索。最初共识别出95条记录（PubMed：n = 36；Scopus：n = 30；Embase：n = 29）。经过筛选和纳入标准评估，选择了16项符合纳入标准的研究进行最终分析。
硬化性牙源性癌主要累及下颌骨。最常报告的症状是肿胀，可为无痛性或疼痛性。在影像学上，病变常表现为边界不清或边缘模糊的透射区，反映其浸润性。常见的皮质骨受累包括变薄、吸收和穿孔。组织病理学上，硬化性牙源性癌的特征是在致密的硬化性间质中有条索状、巢状和束状的温和上皮细胞。免疫组化方面，肿瘤显示高分子量细胞角蛋白（CK5/6、CK19、p63、AE1/AE3）的强表达。主要治疗方式是广泛的局部切除且切缘清晰，通常根据肿瘤范围进行节段性下颌骨切除术或上颌骨切除术。
硬化性牙源性癌是一种极其罕见的牙源性恶性肿瘤，各报告病例具有一致的临床和病理特征。其局部侵袭性突出了早期诊断和广泛手术切除的重要性。本综述整合了现有的病例数据，以加强理解并帮助临床医生识别和处理这种具有挑战性的肿瘤。
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