• Archives of Craniofacial Surgery
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• v.24 no.5
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• pp.244-249
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• 2023

Fibrous dysplasia (FD) is a rare skeletal disorder characterized by abnormal fibro-osseous connective tissue replacing normal bone. Despite its benign behavior, craniofacial FD can cause morphological disfigurement, headache, and even blindness as a result of the produced mass effect. Surgical resection is recommended when the patient shows apparent clinical symptoms or aggravating facial asymmetry. Postoperative complications have been reported, such as hematoma, surgical site infection, abscess formation, resorption of the bone graft used for reconstruction, and recurrence. An aneurysmal bone cyst (ABC) is a rare benign bony lesion that can occur secondary to preexisting bone tumor. Secondary ABCs in craniofacial FD are extremely rare in the literature, accounting for less than 30, all of which are either case reports or series. We report an extremely rare case of symptomatic secondary ABC arising from craniofacial FD that had been misdiagnosed with abscess formation or recurrence and was surgically removed. Notably, 17 years elapsed between the primary surgery and the complication of secondary ABC. The patient underwent total removal of secondary ABC. After surgery, symptoms were relieved, with no recurrence observed during a 6-month follow-up.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.35 no.5
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• pp.304-309
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• 2009

Purpose: Fibrous dysplasia (FD) is a fibro-osseous disease associated with activating missense mutations of the gene encoding the $\alpha$-subunit of stimulatory G protein. FD may affect a single bone (called monostotic form) or multiple bones (called polyostotic form). The extent of lesions reflects the onset time of mutation. In this study, cells from monostotic FD in maxilla of a patient were isolated and cultured in vitro for characterization. Materials and Methods: The single cells were released from FD lesion which was surgical specimen from 15 years-old boy. These isolated cells were cultured in vitro and tested their proliferation activity with MTT assay. In osteogenic media, these cells underwent differentiation process comparing with its normal counterpart i.e. bone marrow stromal cells. The proliferated FD cells were detached and transplanted into the dordsal pocket of nude mouse and harvested in 6 weeks and 12 weeks. Results and Summary: FD cells have an increased proliferation rate and poor differentiation. As a result, cells isolated from FD lesion decreased differentiation into osteoblast and increased proliferation capacity. MTT assay presented that proliferation rate of FD cells were higher than control. However, the mineral induction capacity of FD was lesser than that of control. Monostotic FD cells make fewer amounts of bone ossicles and most of them are woven bone rather than lamellar bone in vivo transplantation. In transplanted FD cells, hematopoietic marrow were not seen in the marrow space and filled with the organized fibrous tissue. Therefore, they were recapitulated to the original histological features of FD lesion. Collectively, these results indicated that the FD cells were shown that the increased proliferation and decreased differentiation potential. These in vitro and in vivo system can be useful to test FD cell's fate and possible.

• Archives of Craniofacial Surgery
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• v.24 no.2
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• pp.59-65
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• 2023

Background: Fibrous dysplasia (FD) is a localized bone disorder in which fibro-osseous tissue replaces the normal bone structure. Patients with craniofacial FD often present with gradual swelling, deformity, and compromised vision or hearing. We previously introduced "the core extirpation method," a novel surgical technique that is minimally invasive like traditional bone shaving but has longer-lasting effects. This study presents the long-term outcomes of our core extirpation method. Methods: We conducted a retrospective analysis of patients who underwent core extirpation for FD of the zygomaticomaxillary region from 2012 through 2021. Computed tomography (CT) scans were performed 6 to 12 months before the operation, immediately before and after the operation, and during follow-up visits. We performed all operations using the upper gingivobuccal approach, and we extirpated the core of the lesion while preserving the cortical structures of the zygoma and the maxilla to maintain symmetrical facial contour. Results: In 12 patients with lesions in the growth phase, anteroposterior/mediolateral (AP/ML) length discrepancies and the volume increased between preoperative and immediate postoperative CT scans. All patients' immediate postoperative AP/ML discrepancies were stable up to 12-17 months postoperatively. Postoperative volume showed continuous lesion growth; the median volume growth rate was 0.61 cc per month. Conclusion: In this article, we present our experiences managing FD using the minimally invasive core extirpation technique, which entails small expected blood loss and can be performed as day surgery. It provides similar cosmetic outcomes as traditional bone shaving but with longer-lasting results. Although there are some limitations with the study's retrospective nature and small sample size, our 4-year follow-up results show promising results of the core extirpation method in well-indicated patients.

• The Journal of the Korean bone and joint tumor society
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• v.8 no.1
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• pp.20-26
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• 2002

Fibrous dysplasia is a benign pathologic condition in which the normal cancellous bone is replaced by the fibro-osseous tissue. It is found mostly in the femur, tibia, skull, rib, and humerus. Clinically it may develops pain, progressive deformity, and pathologic fracture. Curettage with bone graft has been the most popular treatment method thesedays. A 17-year-old female who had fibrous dysplasia of the humerus combined with multiple cystic lesion was treated by intralesional steroid injection into the lesion total 2 times. The follow-up plain X-ray which was taken 11 months after steroid injection reveals decrease in size and increase in bone density and cortex thickness. The follow-up MRI reveals significant decrease in size and signal intensity. The signal intensity was decreased to that of normal bone marrow in T2 weighted image. She complains no pain and lives symptom free in last follow-up at 2 years and 8 months after steroid injection.

• Journal of the korean academy of Pediatric Dentistry
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• v.26 no.3
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• pp.492-498
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• 1999

Oculodentodigital syndrome(ODD) was first reported by Lohmann in 1920 and termed by Meyer Schwicketath, which they called "dysplasia oculo-dento-digitalis" in 1957. It is somewhat rare heritable disease. ODD is generally inherited in an autosomal dominant pattern with a complex phenotype. The characteristic features are : (1) unique facial features, (2) microphthalmos, (3) syndactyly and camptodactyly of 4th and 5th fingers, (4) osseous anomalies of the middle phalanges of 5th fingers and toes, (5) enamel hypoplasia, (6) dry lusterless hair. We found several occlusal wearing and yellow discoloration of succedaneous teeth, multiple caries lesions, premature loss and pulpal involvement of primary teeth with associated enamel abnormalities caused by generalized enamel hypoplasia in a fairly constant oral finding. Occasionally partial anodontia, microdontia and cleft lip and palate can be manifested. This case, a 9-year-old female with repaired bilateral syndactyly was referred to pediatric dental clinic, Pusan National University Hospital for evaluation of severe attrition of teeth and caries lesions. She had most of the above mentioned typical manifestations of the syndrome. Dental treatment including caries control, stainless steel crown were performed.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.34 no.3
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• pp.215-219
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• 2012

Cemento-ossifying fibroma (COF) is a benign fibro-osseous tumor with fibrous tissue, abnormal cement and bone, or a combination of such elements. These are slow-growing lesions and are more frequent in women. Here, we report the case of a 28-year-old Korean woman. The patient having no underlying disease complained about facial swelling and asymmetry. A firm mass with impacted molars and teeth deviation on the right maxilla was observed. A computed tomography scan was taken and an incisional biopsy was performed. Following this, COF was diagnosed. Complete surgical removal of the lesion was carried out. A post-operative follow-up was conducted and 3 months later the patient reported no discomfort or any sign of recurrence in regards to the lesion. Differential diagnosis with fibrous dysplasia and the COF is important because of the treatment choice. We report a case of COF and offer a review of the literature on this article.

• The Journal of the Korean bone and joint tumor society
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• v.2 no.1
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• pp.94-100
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• 1996

Ossifying fibroma is one of a group of fibro-osseous lesions which arises typically within the jaw bones and only rarely affects the long bones. Ossifying fibroma of the long bones almost involve exclusively the tibia but may also involve the fibula. Ossifying fibroma of the long bones is distinct from fibrous dysplasia, adamantinoma and nonossifying fibroma with regard to age of the patient, site, radiographic appearance, histological features, and clinical course. We are reporting the cases of seven patients with a tumor-like lesion that named osteofibrous dysplasia. It is most commonly found in the tibia and fibula of a child ten years of age or younger. Of the seven cases reported in this study, only one patient was younger than ten years. In all cases, the lesions were usually located in the tibial diaphysis. The average duration of clinical manifestation was 5.2 years. The clinical symptoms were anterior bowing of the tibia in 2 cases, buldging of the tibia in 2 cases, and mass overlying the tibia in 3 cases. On the roentgenography, it shows multiple radiolucent lesion with intervening sclerotic rim of the tibial diaphysis. In seven patients, 6 cases were confirmed with biopsy. We had done curettage and bone graft in three cases, VFG was done in one case. The other three cases underwent conservative management.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.27 no.5
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• pp.478-481
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• 2005

Ossifying fibroma is a rare, benign, fibro-osseous tumor of mesenchymal origin. It develops mainly in the mandible, where it is usually slow growing and asymptomatic, whereas in the maxilla and paranasal sinus, it is more aggressive. The vast majority are located in the posterior region of the mandible; however, ossifying fibroma involving the maxillary sinus is uncommon. It may reach a very large size and cause facial asymmetry. It shows a female predilection, and most cases are seen in the third and fourth decades of life. Radiographically, ossifying fibroma is radiolucent or radiopaque depending on the amount of calcification. Histologically, the tumor consists of a cementum-like or bony mass. Ossifying fibroma shares many histopathologic features with fibrous dysplasia. Their radiographic features may help to separate these entities when pathological differentiation is uncertain. It is important to differentiate ossifying fibroma from fibrous dysplasia. The treatment of ossifying fibroma involves its complete removal using curettage, enucleation, excision, or en bloc resection. This case report presents an unusual case of ossifying fibroma of the maxillary sinus. We describe a Caldwell-Luc operation with a lateral window approach for excision of an ossifying fibroma of the maxillary sinus.

• Journal of the korean academy of Pediatric Dentistry
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• v.32 no.2
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• pp.200-206
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• 2005

Cemento-ossifying fibroma of the jaws is well circumscribed, generally slow-growing, benign lesions which enlarge in an expansile manner. Clinically it presents as a slowly enlarging lesion commonly in the premolar-molar area of the mandible and only occasionally in the maxilla and other locations. It occurs twice as often in females and primarily in the 20 to 30 year age group. Differential diagnosis should be peformed, preferably with other fibro-osseous lesions such as fibrous dysplasia. A faster growing and more destructive variant of cemento-ossifying fibroma sometimes occurs in patients under age 15 and is termed juvenile (aggressive) ossifying fibroma. Treatment is surgical removal with the extent depending on the size and location of the individual lesion. Recurrence is considered rare. A case involving a 12-year-old male patient with delayed eruption of right mandibular canine is discussed. Following an incisional biopsy, the histopathologic diagnosis established was cemento-ossifying fibroma. After the surgical enucleation of the lesion, no sign of recurrence was detected.

• Imaging Science in Dentistry
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• v.48 no.4
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• pp.289-293
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• 2018

Odontomas are considered a type of odontogenic hamartoma, and are generally reported not to exceed 3 cm in diameter. Some authors have referred to odontomas with a diameter exceeding 3 cm as giant odontomas. As hamartomas, giant odontomas generally show no signs or symptoms, but if they perforate the mucosa to become exposed in the oral cavity, oral and maxillofacial infections can result. Surgical removal and a histopathological examination may also be required to differentiate them from osteomas, cemento-osseous dysplasia, or mixed odontogenic tumors. This report presents the case of a 28-year-old woman with a giant odontoma in the right mandibular third molar area. Based on a review of the literature published since 2010, only 11 cases of "giant" or "large" odontomas have been reported, most of which were of the complex odontoma type. It was confirmed that they tend to occur in the right posterior mandible.