• IMMUNE NETWORK
• /
• v.20 no.5
• /
• pp.43.1-43.11
• /
• 2020

Capicua (CIC) is a transcriptional repressor that regulates several developmental processes. CIC deficiency results in lymphoproliferative autoimmunity accompanied by expansion of CD44^hiCD62L^lo effector/memory and follicular Th cell populations. Deletion of Cic alleles in hematopoietic stem cells (Vav1-Cre-mediated knockout of Cic) causes more severe autoimmunity than that caused by the knockout of Cic in CD4⁺CD8⁺ double positive thymocytes (Cd4-Cre-mediated knockout of Cic). In this study, we compared splenic CD4⁺ T cell activation and proliferation between whole immune cell-specific Cic-null (Cic^f/f;Vav1-Cre) and T cell-specific Cic-null (Cic^f/f;Cd4-Cre) mice. Hyperactivation and hyperproliferation of CD4⁺ T cells were more apparent in Cic^f/f;Vav1-Cre mice than in Cic^f/f;Cd4-Cre mice. Cic^f/f;Vav1-Cre CD4⁺ T cells more rapidly proliferated and secreted larger amounts of IL-2 upon TCR stimulation than did Cic^f/f;Cd4-Cre CD4⁺ T cells, while the TCR stimulation-induced activation of the TCR signaling cascade and calcium flux were comparable between them. Mixed wild-type and Cic^f/f;Vav1-Cre bone marrow chimeras also exhibited more apparent hyperactivation and hyperproliferation of Cic-deficient CD4⁺ T cells than did mixed wild-type and Cic^f/f;Cd4-Cre bone marrow chimeras. Taken together, our data demonstrate that CIC deficiency at the beginning of T cell development endows peripheral CD4⁺ T cells with enhanced T cell activation and proliferative capability.

• Journal of the korean academy of Pediatric Dentistry
• /
• v.43 no.1
• /
• pp.51-59
• /
• 2016

The purpose of the present retrospective study was to analyze gender, age, awareness, etiology, and treatment choice in patients with failed eruption of the mandibular first molar. Radiographic material and dental records from 67 patients with 74 mandibular first molars with failed eruption treated during the years 2001-2014 were evaluated. As results, the prevalence of mandibular first molar with failed eruption was higher in males than females, and the average awareness age was 9.19 years old. The most common etiological cause was unknown, and the next most common causes were abnormal eruption pathway and odontogenic tumor in order. In this study, it is suggested that more dental education to children about the time of eruption of permanent tooth is required, and the importance of regular dental check-ups is emphasized during mixed dentition.

• Asian Pacific Journal of Cancer Prevention
• /
• v.15 no.19
• /
• pp.8395-8400
• /
• 2014

In Saudi Arabia, cancer of breast is ranked the most frequent neoplasm and second source of cancer death in the female population. Breast cancer (BC) fast diagnosis, prognosis and medication management necessitate, these days, immunohistochemistry (IHC) assessment of hormone receptors and HER2 expression profile. The present report defines the IHC profile of ER, PR and HER2 in Saudi female breast neoplasms of ductal and lobular types and associations ER, PR and HER2 expression patterns with various clinicopathological factors (age, type of tumor, size, laterality, histological grade, and involvement of axillaries lymph nodes). Ninety nine cases of breast tumors were recruited from the pathology department archive of King Abdulaziz University Hospital, Kingdom of Saudi Arabia. ER, PR and HER2 expression was assessed using IHC staining. Ductal carcinomas with a variety of histological grades constituted 88 (88.8%) of total cases. Seventy four (77.8%), 59 (62.1%), and 35 (36.8%) of ductal carcinomas showed positive staining for ER, PR and HER2, in that order. Remaining breast cancer cases were four (4%) lobular carcinomas and two (2%) mixed form of ductal and lobular types, which were ER+, PR+, and HER2-. Breast cancer expression pattern of ER, PR and HER2 in Saudi female is different from that of Tunisian and Jordanian female populations and closer to the expression pattern of Egyptian, Lebanese, Iraqi and western country females. Furthermore, the present study found two IHC patterns of breast cancer ER+/PR-/HER2+ (5%) and ER+/PR-/HER2- (11.1%), which had not been reported in other Arabic studies. Thus the rates of IHC expression patterns in breast cancer show some variation among Arabic female populations.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
• /
• v.43 no.5
• /
• pp.312-317
• /
• 2017

Objectives: In order to assess clinical behavior, response to treatment, and factors influencing prognosis of Korean patients with osteosarcoma of the jaws (OSJ). Materials and Methods: A retrospective study of clinical, and pathological records of 26 patients with OSJ treated at the Department of Oral and Maxillofacial Surgery in Yonsei University Dental Hospital from 1990 to March 2017. Results: Of 26 patients, there were 9 men (34.6%) and 17 women (65.4%). Twenty-one of 26 patients had osteosarcoma of the mandible, and 5 of 26 patients had osteosarcoma of the maxilla. The histopathology of OSJ is highly variable, ranging from chondroblastic type (6 out of 26), osteoblastic type (10 out of 26), fibroblastic type (2 out of 26), to the rare variants like mixed type, small cell osteosarcoma types and more. All patients underwent gross total excision and only a few patients underwent neoadjuvant chemotherapy. Postoperative chemotherapy was given to most of the patients as adjuvant treatment or in combination with radiotherapy. The overall survival rate was 73.1% with an overall 2-year survival rate of 83.3%. The overall 5-,10-,15-year survival rates in this study were 73.5%, 73.5%, 49%, respectively. Using Kaplan-Meier analysis with log rank tests, the size of tumor (T-stage), and resection margins were found to affect the survival rate significantly. The chemotherapy was not significantly associated with improved survival rate. Conclusion: Surgical resection with a clear margin is the most important factor in disease survival. The role of chemotherapy and radiotherapy in OSJ remains controversial, and deserves further studies.

• Asian Pacific Journal of Cancer Prevention
• /
• v.17 no.5
• /
• pp.2679-2681
• /
• 2016

Background: Gastro-entero-pancreatic neuroendocrine neoplasms (GEP-NEN) are relatively rare tumors, not equally distributed in gastro-intestinal system. In 2010, a revised version of the WHO classification of GEP-NENs was published. This study reports for the first time the distribution and characteristics of GEP-NEN in a Lebanese population. Materials and Methods: This descriptive retrospective study concerns all the digestive neuroendocrine tumors with their characteristics diagnosed in $H\hat{o}tel$ Dieu de France in Beirut, Lebanon from 2001 to 2012, all the pathology reports being reanalyzed according to the latest WHO 2010 classification. The characteristics and features of GEP-NEN analyzed in this study were age, gender, grade and site. Results: A total of 89 GEP-NENs were diagnosed, representing 28.2% of all neuroendocrine tumors. The mean age of GEP-NEN patients was 58.7 years and the M/F sex ratio was 1.2. The primary localization was as follows: 21.3%(19) pancreatic, 18% (16) gastric, 15.7% (14) duodenal, 11.2% (10) appendix, 10.1% (9) intestinal, 10.1% (9) colorectal (7.9% colonic and 2.2% rectal), 5.6% (4) hepatic, 2.2% (2) ampulla, 1.1% (1) esophageal and 7.9%(5) NOS digestive (metastatic with unknown primary). Of the 89 patients with GEP-NEN, 56.2% (50) were diagnosed as grade I, 11.2% (10) as grade II, 20.2% (18) as grade III and 12.4% (11) were considered as mixed adeno-neuroendocrine carcinomas (MANEC). Conclusions: This study, one of the rare examples based on the 2010 WHO classification of neuroendocrine tumors in the literature, indicates that in the Lebanese population, all duodenal and appendicular tumors are G1 and the majority of MANEC tumors are gastric and pancreatic tumors. Moreover, more duodenal tumors and fewer rectal tumors were encountered in our study compared to European reports.

• Archives of Craniofacial Surgery
• /
• v.11 no.2
• /
• pp.107-110
• /
• 2010

Purpose: Chondroid syringoma is a rare mixed tumor of the skin that was first described by Hirsch and Helwig (1961). Characteristically, it is composed of the proliferation of epithelial cells in a myxoid and chondroid matrix. Most lesions occur on the head and neck region, and their size may range from 0.5~3 cm. Since a chondroid syringoma presents similar characteristics to other masses on the head and neck region, it is significantly important to distinguish with other masses by a surgical biopsy. Methods: A 51-year old woman presented with a painless nodular mass ($0.5{\times}0.5{\times}0.5cm$) on the philtrum, which appeared during the previous year. The mass was treated with a laser without a surgical biopsy. However, the size of the mass showed no changes. Surgical excision under local anesthesia was performed and sent for histopathology. Results: Gross examination showed a mass surrounded by a well developed capsule within the dermal layer. After complete excision without injury to the orbicularis oris muscle, the wound was covered with a local flap. The histology examination revealed numerous cuboid epithelial cells with tuboalveolar structures and keratinous cysts within a chondroid stroma. No recurrence or metastasis was observed at the follow-up visits. Conclusion: Chondroid syringoma is a rare mass on the face. An accurate diagnosis is essential for optimal treatment. This paper reports a case of a chondroid syringoma on the philtrum with a brief review of the relevant literature.

• Korean Journal of Head & Neck Oncology
• /
• v.11 no.1
• /
• pp.56-62
• /
• 1995

Branchial cleft cyst is rarely encountered congenital neck disease. It is commonly believed that the branchial anomaly is persistance of remnant of the embryologic branchial apparatus. Among the patients visited Kyung Hee Medical Center with neck mass and inflammatory sign from January, 1980 to Aprial, 1994, we reviewed 26 cases of branchial cleft cysts confirmed by histopathologic findings with retrospective study on clinical aspects. The results are as follows: 1) There was no sex difference(14 male and 12 female), and most common between 2nd and 4th decade(21 cases, 80%). 2) Palpable mass was most common complaint(21 cases). In physical exam, the mass was non-tender, mobile and soft in most cases(over 80%). 3) The most common lesion site was anterior triangle in 15 cases. Along the SCM level, 21 cases were in upper 1/3, 2 cases in middle 1/3 and 3 cases in lower 1/3. 4) According to Bailey's classification, type II were 20 cases(76.9%), type I 5 cases(19.2%) and type III 1 case. 5) Among 28 cases, fistulous tract was found in 6 cases: one was complete type and 5 were incomplete type. 6) Among 26 cases before operation, 10 cases were diagnosed as branchial cleft cyst, 6 cases tuberculosis, 3 cases parotid tumor and 2 cases thyroglossal duct cyst. 7) Type of lining epithelium in histopathologic finding was stratified squamous epithelium in 22 cases(84.6%), mixed type in 4 cases(15.4%).

• Korean Journal of Head & Neck Oncology
• /
• v.31 no.1
• /
• pp.14-17
• /
• 2015

Castleman's disease is an uncommon lymphoproliferative disorder. The disorder can be classified based on histological subtype, such as hyaline vascular type, plasma cell type, and mixed type, and can also be clinically divided into either unicentric or multicentric type. Its exact pathophysiology is not clearly identified. The unicentric type is able to be treated by surgical resection. However, there is no standard treatment modlity for the multicentric type. Treatment of multicentric type includes anti-cancer chemotherapy and radiation therapy. Recently, authors have experienced a rare case of unicentric type of Castleman's disease accompanying a mucoepidermoid carcinoma of parotid gland and report a case which is discussed with references.

• Journal of Korean Neurosurgical Society
• /
• v.59 no.6
• /
• pp.584-589
• /
• 2016

Objective : A rare subtype of meningioma, sclerosing meningioma is not included in the current World Health Organization classification of meningiomas and is classified into the category of other morphological variation subtypes. Sclerosing meningioma is often misdiagnosed to other non-benign meningioma or malignant neoplasm, so it is important to diagnose sclerosing type correctly. We analyzed the radiological and clinical characteristics of a series of sclerosing meningiomas. Methods : Twenty-one patients who underwent surgery in one institute with a histopathologically proven sclerosing meningioma were included from 2006 to 2014. Eighteen tumors were diagnosed as a pure sclerosing-type meningioma, and 3 as mixed type. Magnetic resonance image was taken for all patients including contrast enhancement image. Computed tomography (CT) scan was taken for 16 patients. One neuroradiologist and 1 neurosurgeon reviewed all images retrospectively. Results : In the all 16 patients with preoperative CT images, higher attenuation was observed in the meningioma than in the brain parenchyma, and calcification was observed in 11 (69%). In 15 of the 21 patients (71%), a distinctive very low signal intensity appeared as a dark color in T2-weighted images. Nine of these 15 tumors (60%) exhibited heterogeneous enhancement, and 6 (40%) exhibited homogeneous enhancement that was unlike the homogeneous enhancing pattern shown by conventional meningiomas. Ten patients had a clear tumor margin without peritumoral edema. Conclusion : Although these peculiar radiological characteristics are not unique to sclerosing meningioma, we believe that they are distinctive features that may be helpful for distinguishing sclerosing meningioma from other subtypes.

• Clinical and Experimental Pediatrics
• /
• v.61 no.2
• /
• pp.53-58
• /
• 2018

Purpose: Although the prognosis is generally good in patients with intermediate-risk neuroblastoma, no consensus has been reached on the ideal treatment regimen. This study analyzed treatment outcomes and toxicities in patients younger than 18 months with stage 4 MYCN nonamplified neuroblastoma. Methods: We retrospectively analyzed 20 patients younger than 18 months newly diagnosed with stage 4 MYCN nonamplified neuroblastoma between January 2009 and December 2015. Patients received 9 cycles of chemotherapy and surgery, with or without local radiotherapy, followed by 12 cycles of differentiation therapy with 13-cis-retinoic acid. Chemotherapy consisted of alternating cycles of cisplatin, etoposide, doxorubicin, and cyclophosphamide (CEDC) and ifosfamide, carboplatin, and etoposide (ICE) regimens. Results: The most common primary tumor site was the abdomen (85%), and the most common metastatic sites were the lymph nodes (65%), followed by the bones (60%), liver (55%), skin (45%), and bone marrow (25%). At the end of induction therapy, 14 patients (70%) achieved complete response, with 1 achieving very good partial response, 4 achieving partial response, and 1 showing mixed response. Nine patients (45%) received local radiotherapy. At a median follow-up of 47 months (range, 17-91 months), none of these patients experienced relapse, progression, or secondary malignancy, or died. Three years after chemotherapy completion, none of the patients had experienced grade ${\geq}3$ late adverse effects. Conclusion: Patients younger than 18 months with stage 4 MYCN nonamplified neuroblastoma showed excellent outcomes, without significant late adverse effects, when treated with alternating cycles of CEDC and ICE, followed by surgery and differentiation therapy.