• Clinical and Experimental Pediatrics
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• v.54 no.6
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• pp.241-245
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• 2011

Tuberous sclerosis complex (TSC) is a genetic multisystem disorder that results from mutations in the TSC1 or TSC2 genes, and is associated with hamartomas in several organs, including subependymal giant cell tumors. The neurological manifestations of TSC are particularly challenging and include infantile spasms, intractable epilepsy, cognitive disabilities, and autism. The TSC1- and TSC2-encoded proteins modulate cell function via the mammalian target of rapamycin (mTOR) signaling cascade, and are key factors in the regulation of cell growth and proliferation. The mTOR pathway provides an intersection for an intricate network of protein cascades that respond to cellular nutrition, energy levels, and growth factor stimulation. In the brain, TSC1 and TSC2 have been implicated in cell body size, dendritic arborization, axonal outgrowth and targeting, neuronal migration, cortical lamination, and spine formation. The mTOR pathway represents a logical candidate for drug targeting, because mTOR regulates multiple cellular functions that may contribute to epileptogenesis, including protein synthesis, cell growth and proliferation, and synaptic plasticity. Antagonism of the mTOR pathway with rapamycin and related compounds may provide new therapeutic options for TSC patients.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.45 no.6
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• pp.316-323
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• 2019

Objectives: This study aimed to determine the prevalence of odontogenic cysts, tumors, and other lesions among reports in the archives of the Department of Oral and Maxillofacial Surgery at the Faculty of Dentistry affiliated with Kocaeli University collected over a four-year period. Materials and Methods: In this retrospective study, patient records from the archive of the Department of Oral and Maxillofacial Surgery from 2014 to 2018 were reviewed. Patient demographic information (age and sex) and lesion location were recorded and analyzed. Results: From a total of 475 files reviewed, odontogenic cyst was confirmed in 340 cases (71.6%), and odontogenic tumor was confirmed in 52 cases (10.9%). Regarding odontogenic cyst type, the most common was radicular cyst (216 cases), followed by dentigerous cyst (77 cases) and odontogenic keratocyst (23 cases). Among odontogenic tumors, the most frequent was odontoma (19 cases), followed by ossifying fibroma (18 cases) and ameloblastoma (9 cases). Giant cell granuloma was also reported in 35 cases. Conclusion: The distribution pattern of odontogenic cysts and tumors in our retrospective study is relatively similar to that reported in the literature. Complete clinical reports for final diagnosis of these lesions and routine follow-up examinations are very important for treatment.

• The Journal of the Korean bone and joint tumor society
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• v.1 no.1
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• pp.77-83
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• 1995

Malignant fibrous histiocytoma was classified as fibrosarcoma, liposarcoma, rhabdomyosarcoma in times past and it was first introduced in 1963 to refer to a group of soft tissue tumors and reported in bone in 1972. It was postulated that the origin of tumor cell was derived from histiocytes that could assume the appearance and function of fibroblasts("facultative fibroblasts") at first. But, recently the immunohistochemical studies suggested origin from a primitive mesenchymal cell. The malignant fibrous histiocytoma were commonly misdiagnosed as other tumors such as myxoid liposarcoma, pleomorphic rhabdomyosarcoma, osteogenic sarcoma, leiomyosarcoma, malignant schwannoma, giant cell tumor, hemangiopericytoma, dermatofibrosarcoma protuberans due to the broad morphologic spectrum. The seventeen cases of the malignant fibrous histiocytomas diagnosed at Hanyang University Hospital since 1979 were reviwed, and the suggestions are as follows : 1. No fundamental histologic differences between the MFHs of soft tissues and bones. 2. The other tumors must be excluded in order to diagnose MFH due to wide histologic variety of MFH. 3. The review of the initial tissue slide was necessary for adequate diagnosis because the recurrent MFHs showed different histologic findings from original tumors.

• Journal of Korean Neurosurgical Society
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• v.58 no.3
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• pp.248-253
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• 2015

Objective : The treatment of giant cell tumor (GCT) is mainly performed surgically. However, GCT in spine seems difficult to treat because of the limited surgical accessibility and proximity. In this report, we analyzed the outcome of GCT treatment in spine. Methods : Between 2000 and 2012, 19 patients received treatment for GCT in spine. Median age at their first diagnosis was 31 years, 10 patients were male, and 9 female. Fourteen tumors were located in the sacrum, 1 in cervical, 1 in thoracic and 3 in lumbar spine. As primary treatment, gross total removal (GTR) was done in 6 patients, and subtotal removal (STR) in 13 patients. Radiation therapy (RT) as an adjuvant therapy was performed in 2 cases in GTR group and 10 cases in STR group. Results : During the follow-up, 7 patients had local recurrence (36.8%). The average period until recurrence after primary treatment was 14 months. No recurrence was detected in GTR group. Recurrence was noted in 7 out of 13 patients who underwent STR. These differences were statistically significant (p=0.024). A median of recurrence free period (RFP) was 84 months. Also average RFP of the RT group was 112 months, and non-RT group was 65 months. These differences were statistically significant (p=0.041). Conclusion : Treatment of choice for GCT in spine is a complete removal of tumor without neurological deficits. In case of incomplete removal, radiation therapy may be a useful adjuvant treatment modality.

• Archives of Craniofacial Surgery
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• v.13 no.1
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• pp.76-79
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• 2012

Purpose: Sebaceous epithelioma (sebaceoma) is a benign tumor with sebaceous differentiation. It presents primarily as a yellowish papule or nodule on the face and scalp. It must be differentiated from basal cell carcinoma and other appendageal tumors. We report a giant sebaceous epithelioma on the scalp and describe the immunohistochemical character of the cells in sebaceous epithelioma to epithelial membrane antigen (EMA). Methods: A 55-year-old-man who presented with 5-cm-diameter 2-cm-height, round shape exophytic ulcerated tumor on his head presented for treatment. The patient had noticed the lesion 40 years prior as a small yellowish plaque and 18 months ago, the plaque started to grow progressively larger. We excised the lesion with 1 cm resection margin, considering the possibility of malignancy because this lesion grossly resembled basal cell carcinoma (BCC). The defect was repaired with the use of a splitthickness skin graft. Results: When we excised the lesion, the margin was clear. Histology showed nodules that consisted of an admixture of basaloid cells and mature adipocytes lacking an organized lobular architecture. Strong expression of EMA on mature adipose cells confirmed the differential diagnosis from BCC with sebaceous differentiation because of the absence of a nuclear palisade pattern and cleft-like spaces on the hematoxylin and eosin (H&E) section. Conclusion: We treated the giant sebaceous epithelioma on the scalp with surgical excision and a split-thickness skin graft. It is important to know that the diagnosis of sebaceous epithelioma should be made based on the histologic pattern of the H&E section. Immunohistochemistry with EMA can help to confirm the differential diagnosis between sebaceous epithelioma and BCC.

• The Journal of the Korean bone and joint tumor society
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• v.4 no.1
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• pp.37-43
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• 1998

Between 1992 and 1996, 5 patients with the giant-cell tumor of the spine were treated. Four were female and one was male. The mean age was 34 years old, and the mean follow-up time was 36 months. The locations of the lesions were the cervical spine in 1, the thoracic spine in 3, and the lumbar spine in 1. Pain was the predominant presenting symptom in all cases and four had a neurological deficit. A combined anterior and posterior surgical approach wds as performed in all cases, which were also treated with AIF(anterior interbody fusion) and anterior and/or posterior instrumentation. Adjuvant radiation therapy was performed in 1 case of cervical spine. At the final follow-up, the pain and neurologic symptoms were improved. Radiologic examination showed no evidence of local recurrence and no failure of instrumentation of the spine.

• Imaging Science in Dentistry
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• v.47 no.4
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• pp.269-273
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• 2017

Mandibular bone depression, also known as Stafne bone cavity, is defined as a bone depression filled mainly with salivary gland tissue. Parotid gland bone defects are infrequently observed. We report the case of a 52-year-old male patient who underwent radiographic examinations due to temporomandibular joint dysfunction, and a radiolucent area was detected in the mandibular ramus, with a provisional diagnosis of traumatic bone cyst or parotid mandibular bone defect. The patient was then referred for magnetic resonance imaging, which demonstrated a hyperintense area eroding the mandibular ramus, which corresponded to glandular tissue. Although the defect was a benign lesion, radiolucencies in the mandibular ramus lead to concerns among professionals, because their radiographic features can resemble various intrabony neoplastic lesions, such as giant cell tumors or benign tumors of the parotid gland.

• The Korean Journal of Cytopathology
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• v.8 no.2
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• pp.164-169
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• 1997

A case of plasmacytoma of the ovary and breast, which developed in a patient with a solitary plasmacytoma in the lumbar vertebra for nine months, was diagnosed cytologically and histologically. Enlargement of the right ovary and multiple palpable masses in the right and left breast were already present at six months after the diagnosis of vertebral solitary plasmacytoma. At eight months, plasma cell leukemia developed, and nine months the enlarged both ovaries, replaced by yellowish-gray solid tumors showed infiltration of immature plasma cells. The cytologic features of the ovarian tumors were same with those of the breast tumor. The tumor cells were of predominantly immature plasma cells with one or more nuclei. Some mature plasma cell had an eccentric nucleus with single nucleolus and peripherally clumped chromatin. Binucleated or multinucleated giant cells were often present. Histologically, sheets of poorly differentiated plasmacytoid tumor cells were separated by strands of hyaline fibrous tissue. On immunohistochemical stains, the tumor cells showed strong reactivity for lambda-light chain but no reaction for kappa-light chain, cytokeratin, or leukocyte common antigen.

• The Journal of the Korean bone and joint tumor society
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• v.13 no.2
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• pp.67-74
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• 2007

Purpose: To evaluate association of giant cell tumors recurrence between markers of proliferation cells (MCM3, Ki-67 and HH3) Materials and Methods: Ten case of giant cell tumor of bone were reviewed. The patients were six males and four females (mean age: 33 yrs). All patients were done operation after biopsy. The radiologic grading was determined according to Enneking grading system. The immunohistochemical stains of MCM3, HH3, and Ki-67 were done with Microarray block. Results: The three cases of 10 cases (30%) were recurred at same sites. Two case of recurrence was grade II according to radiologic features. The remaining case was grade I. The expression rate of immunohistochemical markers in radiologic grade 2 and 3 were more increased than grade 1. But there was not association between radiologic grading and proliferation of tumor cells because result data was not coherence. Mean MCM3 labeling index of non-recurred case was 11.2%, recurred case was 7.2%. Ki-67 was 12% vs. 8.9%, respectively and HH3 was 66.9 % vs. 75.4%, respectively. Thus there was no association between local recurrence and immunohistochemical Ki-67, MCM3 expression rate. But HH3 marker expression rate was increased in recurred cases compared to non-recurred cases. Conclusion: Our study suggests that HH3 immunohistochemical marker can be a useful prognostic factor.

• Korean Journal of Head & Neck Oncology
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• v.14 no.2
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• pp.220-227
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• 1998

The clinical and pathological features of 35 cases of anaplastic thyroid cancer were studied. These tumors occurred in 12 men and 23 women ranging in age from 19 to 83 years(mean age; 61.7 years). A rapidly enlarging thyroid mass was the most common presentation. The duration of the presence of mass varied from 20 days to 12 months with an average of 2.7 months. Systemic metastasis at the time of initial examination was found in 14 patients(40.0%) and the lung was the most common site of involvement. The overall rate of distant metastasis was about 65.7%. The tumors were subdivided morphologically into giant cell type of 10 cases, spindle cell type of 7 cases, epidermoid cell type of 1 cases, and mixed giant cell and spindle cell type of 5 cases. The mean survival period of 6 among 35 patients who had biopsy alone was 1.4 months. The 22 patients underwent the incomplete combined treatment modalities (palliative surgery with or without chemotherpy or radiation therapy) survived for a mean period of 3.0 months, among them, 7 patients who had surgery combined with chemotherapy and radiation therapy showed mean survival period of 3.7 months. The mean survival of 7 patients who had complete combined treatment modality(curative surgery combined with chemotherapy and hyperfractionated radiation therapy) was 6.6 months, only one patient survived for 21 months and one patient has been alive for 1 month after operation, and the others survived for a mean period of 4.8 months. So far, as of July 31, 1998, 34 patients among 35 were died(one has been survived for 1 month) despite the various treatment modalities, and the main cause of death were failure of local control and systemic metastasis. None of the various treatment modalities gave consistently favourable results. However, a combination of surgery, radiation therapy and chemotherapy seemed to have a slight positive effect on survival. Furthermore, the aggressive treatment modalities will be indicated only in the early diagnosed and minimal cases.