Maxillofacial Surgery and Diagnostic Sciences Department, College of Dentistry, King Saud Bin Abdulaziz University for Health and Sciences, P.O Box 22490, Riyadh, 11426, Saudi Arabia.
King Abdulaziz International Medical Research Centre, Riyadh, Saudi Arabia.
Head Neck Pathol. 2022 Jun;16(2):550-559. doi: 10.1007/s12105-021-01354-0. Epub 2021 Jul 5.
Primordial odontogenic tumor (POT) is a rare, mixed odontogenic neoplasm composed of spindled and stellate-shaped cells in myxoid stroma resembling dental papilla, surfaced by cuboidal-to-columnar odontogenic epithelium. Most POTs present in the posterior mandible as a well-demarcated radiolucency associated with a developing tooth in children and adolescents. POT is treated conservatively with no recurrences documented to-date. To describe the clinicopathological features of a recurrent POT. A 19-year-old female presented with an asymptomatic swelling, and panoramic radiograph revealed a multiloculated radiolucency in the mandibular body and ramus, with buccal and lingual perforation. The tumor was composed of plump spindle and stellate cells in a delicately collagenous and myxoid stroma, surfaced by columnar epithelial cells with reverse nuclear polarization. There was extensive epithelial proliferation forming invaginations within the tumor mass and organoid/enamel organ-like structures with enameloid-like deposits, dentinoid, and dystrophic calcifications. This was similar to the POT that had been excised four years prior from the same location. The patient underwent hemi-mandibulectomy and currently is free of disease at a thirteen-month follow-up. This report describes the first recurrent POT exhibiting extensive epithelial proliferation.
原发性牙源性肿瘤 (POT) 是一种罕见的混合性牙源性肿瘤,由在黏液样基质中呈纺锤形和星状的细胞组成,类似于牙乳头,表面覆盖有立方体形至柱状的牙源性上皮。大多数 POT 在后颌骨中呈现为界限清楚的放射性透光区,伴有儿童和青少年发育中的牙齿。POT 采用保守治疗,迄今为止未记录到复发。描述复发性 POT 的临床病理特征。一名 19 岁女性出现无症状肿胀,全景片显示下颌体和支部有多房放射性透光区,伴有颊侧和舌侧穿孔。肿瘤由丰满的纺锤形和星状细胞组成,存在精细的胶原和黏液样基质,表面覆盖有柱状上皮细胞,具有反向核极化。广泛的上皮增殖形成肿瘤内的内陷和类器官/牙器样结构,伴有釉质样沉积物、牙本质和营养不良性钙化。这与四年前在同一部位切除的 POT 相似。患者接受了半下颌切除术,目前在 13 个月的随访中无疾病。本报告描述了首例表现出广泛上皮增殖的复发性 POT。