• Maxillofacial Plastic and Reconstructive Surgery
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• 제31권5호
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• pp.429-434
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• 2009

Basal cell nevus syndrome is a rare inherited disorder characterized by mulitple cutaneous basal cell carcinoma, pits of the palms and soles, cysts of the jaws, skeletal abnormalities and ectopic calcifications. Currently there are new lines of investigation based on biomolecular studies, which aim at identifying the molecules responsible for these cysts and thus early allowing an early diagnosis of these patients. We report a case of a 9-year-old boy with the various manifestation of basal cell nevus syndrome, which are multiple odontogenic keratocysts, pits of the soles, bifid ribs, ectopic calcification, macrocephaly, and hypertelorism, etc. Total five odontogenic keratocysts were found. For the reduction of the size of the odon-togenic keratocysts, following preoperative marsupialization, there were surgically enucleated. And the impacted upper right lateral incisor and canine are tracted orthodontically.

• 대한소아치과학회지
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• 제41권2호
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• pp.166-173
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• 2014

기저세포 모반 증후군은 상염색체 우성 방식으로 유전되는 질환으로 다발성 각화낭성 치성종양, 대두증 및 두정골 융기, 골격계 기형 등을 보이는 질환이다. 다발성 각화낭성 치성 종양, 두정골 융기, 양안격리증을 가진 만 11세 환자의 하악과 상악에서 발생한 다양한 크기의 낭종에 대한 적출술과 이종골 이식술을 시행 후 교정적 견인을 시행하였다. 낭종에 의한 매복 영구치를 가진 성장중인 기저세포 모반 증후군 환자에서의 보존적인 치료를 하였기에 문헌고찰과 더불어 보고하고자 한다.

• Restorative Dentistry and Endodontics
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• 제48권2호
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• pp.18.1-18.10
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• 2023

Objectives: This study aimed to determine whether collagen triple helix repeat containing-1 (CTHRC1), which is involved in vascular remodeling and bone formation, can stimulate odontogenic differentiation and angiogenesis when administered to human dental pulp stem cells (hDPSCs). Materials and Methods: The viability of hDPSCs upon exposure to CTHRC1 was assessed with the WST-1 assay. CTHRC1 doses of 5, 10, and 20 ㎍/mL were administered to hDPSCs. Reverse-transcription polymerase reaction was used to detect dentin sialophosphoprotein, dentin matrix protein 1, vascular endothelial growth factor, and fibroblast growth factor 2. The formation of mineralization nodules was evaluated using Alizarin red. A scratch wound assay was conducted to evaluate the effect of CTHRC1 on cell migration. Data were analyzed using 1-way analysis of variance followed by the Tukey post hoc test. The threshold for statistical significance was set at p < 0.05. Results: CTHRC1 doses of 5, 10, and 20 ㎍/mL had no significant effect on the viability of hDPSCs. Mineralized nodules were formed and odontogenic markers were upregulated, indicating that CTHRC1 promoted odontogenic differentiation. Scratch wound assays demonstrated that CTHRC1 significantly enhanced the migration of hDPSCs. Conclusions: CTHRC1 promoted odontogenic differentiation and mineralization in hDPSCs.

• Imaging Science in Dentistry
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• 제34권1호
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• pp.49-54
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• 2004

Primary intraosseous carcinoma (PIOC) is a rare odontogenic carcinoma defined as a squamous cell carcinoma arising within a jaw having no initial connection with the oral mucosa, and presumably developing from residues of the odontogenic epithelium. A 56-year-old patient who complained of delayed healing after extraction of upper left central incisor visited our department. The conventional radiographs showed a bony destructive lesion with ill-defined margin and moth-eaten appearance. On the computed tomographic images, the lesion perforated the labial cortex of alveloar bone, elevated the left nasal floor superiorly, and perforated partially both nasal floor. The magnetic resonance images showed low signal intensity at T2 and Tl weighted images at the area and adjacent soft tissue. Histologically, there were irregular epithelial islands with cell atypia, nuclear hyperchromatism, pleomorphism, atypical mitosis. The final diagnosis was PIOC.

• Imaging Science in Dentistry
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• 제42권1호
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• pp.55-60
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• 2012

Gorlin-Goltz syndrome is an autosomal dominant disorder principally characterized by cutaneous basal cell carcinomas, multiple keratocystic odontogenic tumors, and skeletal anomalies. This syndrome may be diagnosed early by dentist because keratocystic odontogenic tumors are usually one of the first manifestations of the syndrome. Early diagnosis and treatment are of utmost importance in reducing the severity of long term sequelae of this syndrome. This report presents a rare event of Gorlin-Goltz syndrome occurring in a 39-year-old male and his 8-year-old daughter. The clinical and investigative features of this familial disorder has been described in detail.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• 제48권6호
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• pp.386-389
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• 2022

Multiple odontogenic keratocysts (OKC) are a distinguishing feature of nevoid basal cell carcinoma syndrome (NBCCS). Owing to the high recurrence rate of syndromes associated OKCs, complete surgical resection is generally recommended as a definitive treatment. Herein, we report the management of multiple OKCs with marsupialization followed by excision with peripheral ostectomy in an NBCCS patient. We then discuss lesion progression over 11 years of annual follow-ups.

• International Journal of Oral Biology
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• 제37권4호
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• pp.167-173
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• 2012

This study investigated the genes involved in the differentiation of odontoblasts derived from human dental pulp stem cells (hDPSCs). hDPSCs isolated from human tooth pulp were validated by fluorescence activated cell sorting (FACS). After odontogenic induction, hDPSCs were analyzed investigated by Alizaline red-S staining, ALP assay, ALP staining and RT-PCR. Differential display-polymerase chain reaction (DD-PCR) was performed to screen differentially expressed genes involved in the differentiation of hDPSCs. By FACS analysis, the stem cell markers CD24 and CD44 were found to be highly expressed in hDPSCs. When hDPSCs were treated with agents such as ${\beta}$-glycerophosphate (${\beta}$-GP) and ascorbic acid (AA), nodule formation was exhibited within six weeks. The ALP activity of hDPSCs was found to elevate over time, with a detectable up-regulation at 14 days after odontogenic induction. RT-PCR analysis revealed that dentin sialophosphoprotein (DSPP) and osteocalcin (OC) expression had increased in a time-dependent manner in the induction culture. Through the use of DD-PCR, several genes were differentially detected following the odontogenic induction. These results suggest that these genes may possibly be linked to a variety of cellular process during odontogenesis. Furthermore, the characterization of these regulated genes during odontogenic induction will likely provide valuable new insights into the functions of odontoblasts.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제19권2호
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• pp.200-208
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• 1997

The WHO separates odontogenic carcinomas into three groups : malignant ameloblastoma, primary intraosseous carcinoma(PIOC), and carcinomas arising from odontogenic epithelium including those arising from odontogenic cysts. In WHO criteria, primary intraosseous carcinoma is defined as a squamous cell carcinoma arising within the jaw, having no connection with the oral mucosa, and no developing from residues of odontogenic epithelium. This is a case of 52-year old man who had prolonged jaw pain and final diagnosis was primary intraosseous carcinoma(PIOC) on mandible. We obtained successful result after composite resection combined with hemimandibulectomy, RND, following reconstruction with latissmus dorsi myocutaneous flap, and postoperative radiation therapy.

• 대한기관식도과학회지
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• 제5권1호
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• pp.68-72
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• 1999

Odontogenic keratocyst is a central destructive lesion of the jaws characterized by a thin, fragile layer of orthokeratinizing or parakeratinizing stratified squamous epithelium. Correlation between the histologic type and the recurrence ratio remains a subject of controversy, and multiple cysts are known to be associated with the nevoid basal cell syndrome. We experienced a case of multiple odontogenic keratocyst in a 25 year-old male patient involving bilateral mandible and maxilla. The cystic mass of the right maxilla was removed by Caldwell-Luc's approach and the right mandibular mass was removed by intraoral approach but the teeth that were impacted in the mandibular bone were remained in order to prevent an iatrogenic fracture.

• International Journal of Oral Biology
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• 제44권3호
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• pp.77-80
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• 2019

Clear cell odontogenic carcinoma (CCOC), a very rare neoplasm located mostly in the mandible, has been regarded as a benign tumor. However, due to the accumulation of case reports, CCOC has been reclassified as a malignant entity by the World Health Organization. Patients with CCOC present with regional swelling and periodontal indications with variable pain, often remaining misdiagnosed for a long period. CCOC has slow growth but aggressive behavior, requiring radical resection. Histologic analysis revealed the monophasic, biphasic, and ameloblastic types of CCOC with clear cells and a mixed combination of polygonal and palisading cells. At the molecular level, CCOC shows the expression of cytokeratin and epithelial membrane antigen, along with markers that assign CCOC to the sarcoma family. At the genetic level, Ewing sarcoma breakpoint region 1-activating transcription factor 1 fusion is regarded as the key feature for identification. Nevertheless, the scarcity of cases and dependence on histological data delay the development of an efficient therapy. Regarding the high recurrence rate and the potential of distant metastasis, further characterization of CCOC is necessary for an early and accurate diagnosis.