PDF(Pubmed)
Abstract：
Keratoameloblastoma (KA) and solid variant of odontogenic keratocyst (SOKC) are rare odontogenic lesions, and their relationship and differences are unclear. The present study described a case that started as an odontogenic keratocyst (OKC) and transformed to SOKC/KA upon recurrence. Briefly, a 26‑year‑old man presented with swelling in the right cheek and was referred to the Department of Oral and Maxillofacial surgery, Hiroshima University Hospital (Hiroshima, Japan). At the initial visit, unicystic bone permeation was observed extending from the right canine to the molar, maxillary sinus and nasal cavity. After the biopsy, the patient underwent excisional surgery and was diagnosed with OKC. Thereafter, the lesion recurred six times over a period of 13 years and showed different histopathological features from those of the primary lesion, all consisting of numerous cysts with keratinization, which were diagnosed as SOKC/KA. The Ki‑67 positivity rate was ~10%, which was higher than that of the primary lesion, but there was no atypia. Genetic analysis of the recurrent lesion revealed mutations in adenomatous polyposis coli and Kirsten rat sarcoma viral oncogene homolog. This case originated from OKC, and the morphological features of OKC and KA were mixed upon recurrence, supporting the commonality and association between the two. However, multiple mutations different from those of OKC and ameloblastoma were detected, suggesting an association of SOKC/KA with increased proliferative activity and a high recurrence rate.
摘要：
角膜成纤维细胞瘤（KA）和牙源性角膜囊肿（SOKC）的实体变体是罕见的牙源性病变，他们的关系和差异尚不清楚。本研究描述了一个病例，该病例始于牙源性角化囊肿（OKC），并在复发后转化为SOKC/KA。简而言之，一名26岁的男子出现右脸颊肿胀，被转诊至口腔颌面外科，广岛大学医院(广岛,日本）。在初次访问时,观察到单囊骨渗透从右犬延伸到磨牙，上颌窦和鼻腔。活检后,患者接受了切除手术,并被诊断为OKC.此后,该病变在13年的时间内复发了6次，并显示出与原发病变不同的组织病理学特征，全部由许多角质化囊肿组成，被诊断为SOKC/KA。Ki‑67阳性率约为10%，高于原发病变，但是没有非典型。复发性病变的遗传分析显示，腺瘤性息肉病大肠杆菌和Kirsten大鼠肉瘤病毒癌基因同源物发生突变。这个案子起源于OKC,复发时OKC和KA的形态学特征混合，支持两者之间的共同性和关联性。然而,检测到与OKC和成釉细胞瘤不同的多个突变，提示SOKC/KA与增殖活性增加和高复发率相关。
