Department of Oral Pathology, Oral Medicine and Periodontology, Faculty of Dentistry, University of Malaya, Kuala Lumpur, Malaysia.
Eur J Med Res. 2010 Apr 8;15(4):180-4. doi: 10.1186/2047-783x-15-4-180.
Squamous odontogenic tumor (SOT) is a rare benign odontogenic epithelial neoplasm. A slow-growing painless expansive swelling is the common presenting symptom. Histopathologically, SOT can be easily misdiagnosed as an acanthomatous ameloblastoma. Although Notch receptors and ligands have been shown to play a role in cell fate decisions in ameloblastomas, the role of these cell signaling molecules in SOT is unknown.
This paper describes a case of SOT affecting the anterior mandible of a 10-year-old Indian female. The patient was treated by local surgical excision and there has been no follow-up clinical record of recurrence 5 years after primary treatment. Histo?pathological examination revealed a solid, locally-infiltrative neoplasm composed of bland-looking squamatoid islands scattered in a mature fibrous connective tissue stroma and the diagnosis was SOT. Immunohistochemical evaluation showed positive reactivity of varying intensity in the neoplastic epithelial cells for Notch1, Notch3, Notch4, and their ligands Jagged1 and Delta1. Expression patterns showed considerable overlap. No immunoreactivity was detected for Notch2 and Jagged2.
Present findings suggest that Notch receptors and their ligands play differential roles in the cytodifferentiation of SOT.
鳞状牙源性肿瘤（SOT）是一种罕见的良性牙源性上皮性肿瘤。常见的表现症状是生长缓慢、无痛性膨胀性肿胀。组织病理学上，SOT 很容易误诊为棘皮瘤型成釉细胞瘤。虽然 Notch 受体及其配体已被证明在成釉细胞瘤的细胞命运决定中发挥作用，但这些细胞信号分子在 SOT 中的作用尚不清楚。
本文描述了一例发生于 10 岁印度女性下颌前牙的 SOT 病例。患者接受了局部手术切除，在初次治疗后 5 年的随访中，未发现临床复发的记录。组织病理学检查显示，一种由外观温和的鳞片状岛状结构组成的实性、局部浸润性肿瘤，散布在成熟的纤维结缔组织基质中，诊断为 SOT。免疫组织化学评估显示，肿瘤上皮细胞对 Notch1、Notch3、Notch4 及其配体 Jagged1 和 Delta1 的反应呈不同强度的阳性。表达模式显示出相当大的重叠。Notch2 和 Jagged2 均无免疫反应。
目前的研究结果表明，Notch 受体及其配体在 SOT 的细胞分化中发挥不同的作用。
