Cherubism: a case report of a three-generation inheritance and literature review.

Cherubism is a rare, non-neoplastic pathologic entity first described by Jones in 1933. It affects mostly younger individuals and is usually inherited. Cherubism presents as a painless jaw enlargement that affects both the maxilla and the mandible and is characterized by replacement of the osseous tissue by fibrous connective tissue. Radiologically, the lesions appear as multiple, multilocular radiolucent spaces with distinct borders divided by bony septations, which often dramatically alter the shape and size of the jaw structures. These often result in marked facial abnormalities. Cervical lymphadenopathy is not uncommon in those affected. The disease is self-limited and can reverse itself with time. Approximately 250 cases have been reported in published studies, mostly affecting males. The purpose of our report was to present a case of cherubism diagnosed in a young girl and the long-term (3 generations) follow-up evaluation of her mother and grandmother, both of whom had been diagnosed with the same pathologic entity.

Neck dissection in relation with disease-free, disease-specific, and overall survival of patients with squamous cell cancer of the oral cavity.

PURPOSE: The aims of this study were to assess the accuracy of the presurgical TNM staging of patients with oral squamous cell carcinoma who underwent neck dissection, to explore the relation between the site of the primary tumor and the histopathologically determined neck metastasis, and to perform survival analysis in relation to the histopathologic neck status. PATIENTS AND METHODS: A retrospective chart review with prospective follow-up of oral squamous cell carcinoma patients who underwent neck dissection was performed. Presurgical clinical neck status (cN) and postsurgical histopathologic neck status (pN) were recorded. Sensitivity, specificity, and likelihood ratios were calculated. Kaplan-Meier survival analysis was performed. RESULTS: The patients clinically staged as cN+ have more than 2-fold odds of having a pN+ neck. Those staged as cN0 have 4 times less odds of a pN+ neck. The mean follow-up was 45.8 months. The median overall survival was 27 months (41 mo in the patients with pN0 and 19.5 mo in the patients with pN+). For the patients with pN0, the 1-year overall survival probability was 95%, falling to 90% at 2 years and 81.8% at 5 years. In the patients with pN+, the 1-year overall survival probability was 72.2%, falling to 44.4% at 2 years and 26.7% at 5 years. Disease-specific and disease-free survival exhibited similar trends. CONCLUSIONS: No means of presurgical assessment either clinical or imaging was sufficiently sensitive and specific enough to predict the metastatic status of the neck. An approximately 50% decrease in the 5-year overall survival rate may be expected when regional metastasis is confirmed.

Coronary angiography through the interosseous artery: expanding the limits of the wrist approach.

A 59-year-old diabetic man with a history of numerous coronary angiographies (CAs) and peripheral artery disease underwent CA due to a non-ST elevation myocardial infarction. Femoral, radial, and ulnar arteries were unpalpable.

Immunohistochemical Expression of Epithelial Cell Adhesion Molecule (EpCAM) in Salivary Gland Cancer: Correlation with the Biological Behavior.

Salivary gland neoplasms comprise a diverse group of tumors with different biological behaviors and clinical outcomes. Understanding the underlying molecular alterations associated with these malignancies is critical for accurate diagnosis, prognosis, and treatment strategies. Among the many biomarkers under investigation, epithelial cell adhesion molecule (EpCAM) has emerged as a promising candidate in salivary gland cancer research. This article aims to provide a comprehensive overview of the differential expression of EpCAM in salivary gland cancer and its potential correlation with the biological behavior of these tumors. The clinical characteristics of 65 patients with salivary gland malignancy of different histopathological subtypes were included. We report the differential expression of EpCAM and the relationship between the clinical and histopathologic features of these tumors. Regarding the evaluation of the effect of EpCAM expression on survival, in our study, we showed that tumors with high EpCAM expression had reduced disease-free survival (DFS) and overall survival (OS) (p < 0.001) compared to patients with cancers with low EpCAM expression. In addition, the concurrent presence of perineural invasion and positive EpCAM expression appeared to be associated with shorter disease-free survival and overall survival. In conclusion, our study confirmed the prognostic value of detecting perineural invasion and EpCAM expression.

The Value of Three-Dimensional Modeling on Repositioning and Soft Tissue Filling in Microsurgical Reconstruction of Secondary Mandibular Defects: A Retrospective Study.

Purpose Secondary mandibular defect reconstruction is a challenging operation. It is performed rather rarely, as in most of the cases a primary reconstruction is preferable. Restoration of function and symmetry, in secondary reconstruction of mandibular defects, requires accurate repositioning of segments and appropriate soft tissue filling. The purpose of this study is to estimate the value of three-dimensional (3D) digital modeling to meet the above requirements, in secondary microsurgical reconstruction of mandibular defects. Methods Five cases of mandibular defects, with various degrees of asymmetry and dysfunction, were restored secondarily, with fibula flaps, after virtual reconstruction on a 3D model derived from their computed tomography, with the utilization of CAD-CAM software. Software reproduced symmetrical 3D models by mirroring the healthy side. Occlusion was restored by fine adjustments on 3D models and finally a reconstruction plate was pre-bent on them, prior to its sterilization for use in surgery. Three out of the five cases received an osteo-cutaneous flap, while in the other two patients, an osseous flap was used. Lower face symmetry, mouth opening, and main patient's complaints were evaluated pre- and postoperatively to assess the value of the 3D modeling. Results All flaps survived. Hematoma occurred in two patients and was resolved after evacuation. In two patients, a fistula observed, was attributed to screw loosening, and treated after a surgical debridement and screw replacement. Intraoral exposure in the posterior part of the fibula flap was recorded in one patient, possibly from wound dehiscence due to tension of the intraoral tissue, and successfully covered with an ipsilateral nasolabial flap. The resulting symmetry and function were satisfactory in all the patients. Conclusion Secondary mandibular defects are often related with cosmetic disfigurement, misalignment of mandibular segments, and mandibular malfunction. For the correction of mandibular continuity, symmetry, and restoration of function, preoperative 3D modeling may be an important tool, according to our results.

Quality of Life in Patients With Tongue Cancer After Surgical Treatment: A 12-Month Prospective Study.

Aim of the study The project assessed the quality of life in post-operative patients with tongue cancer at three-month intervals in the first year after the operation. Material and methods A longitudinal prospective cohort study was conducted in the oral and maxillofacial department of a large public general hospital in northern Greece. Fifty-six patients out of a total of 156 with oral cancer were chosen for the study, who completed distinct quality-of-life surveys (EORTC QLQ-C30, and QLQ Head & Neck Module FACT-G). Results Tumor size correlated negatively with various EORTC QLQ-C30 scores, such as role functioning (p = 0.004) and cognitive functioning (p = 0.007), in the third evaluation. Tumor size correlated positively with subscale problems such as social eating (p = 0.001) and weight loss (p = 0.004) in the QLQ Head & Neck Module. The role functioning subscale (p = 0.003), the pain subscale (p = 0.001), and the speech issues QLQ Head & Neck module subscale (p = 0.003) adversely correlated with cancer stage. Patients who received flap reconstruction significantly differed from those who did not, on the EORTC QLQ-C30 cognitive functioning (U = 139.0, p = 0.006), dyspnea (U = 391.5, p = 0.006), and diarrhea (U = 425.0, p = 0.007) subscales during the third evaluation. Differences were also found in the QLQ-H&N35 subscale of sticky saliva (U = 391.0, p = 0.006). Patients with flap reconstruction did not significantly differ from those with immediate closure after one year. Differences concerned the EORTC QLQ-C30 subscales of cognitive functioning, dyspnea, and diarrhea, and the QLQ Head & Neck Module subscale of sticky saliva on the third assessment. No statistically significant correlations were observed between tumor size and cancer stage in the fourth assessment, but the grade of cancer positively correlated with the EORTC QLQ-C30 subscale of constipation (p = 0.000). Conclusions Our study suggests that quality of life is impaired in patients with tongue cancer who have undergone surgical interventions, particularly within the first month post-operation. However, quality of life is fully restored one year after the surgical excision. Future studies should explore early interventions to help healthcare providers better treat this unique group of patients.

Quality of Life in Oral Cancer Patients in Greek Clinical Practice: A Cohort Study.

Cancer of the oral cavity is one of the most common cancers all over the world. Oral cancer and its treatment impacts on patients' Quality of Life (QOL). The purpose of the present study was to assess oral cancer patients' QOL after the completion of surgical therapy, and to investigate factors affecting it. This was a prospective cohort study, conducted at the Department of Oral & Maxillofacial Surgery, of a large general public hospital in Northern Greece. The sample consisted of 135 consecutive eligible cancer patients. Three distinct questionnaires were used. The first one included questions regarding the participants' demographic characteristics and relevant clinical information. The second comprised the European Organization for Research and Treatment core module (EORTC QLQ-C30) and its head and neck module EORTC QLQ-H&N35. The third was the Functional Assessment of Cancer Therapy-General (FACT-G) assessment of quality of life. We also included the physician-completed Karnofsky scale to assess the functional status of the participants. We found that location of the tumor affects QOL and specifically social contact (H = 17.89, p = 0.001), on the first assessment, and nutritional supplements (H = 22.49, p = 0.000), on the fourth assessment. QOL in patients deteriorates immediately after treatment but significantly improves over time. Health care professionals should take into account these results and arrange care plans in order to find ways to increase patients' QOL.

Branchial cyst carcinoma revisited: stem cells, dormancy and malignant transformation.

PURPOSE: Branchial cleft cysts are among the most common causes for a congenital neck mass. Branchial cleft cyst carcinoma (BCCC) is a type of cancer that arises from cells within these cysts. Despite the distinct criteria that have been reported for its diagnosis, BCCC remains a controversial entity. CLINICAL REPORT: We report a case of type I, first BCCC, on a 71-year-old white man. The diagnosis was based on the proposed criteria following lesion history and location, surgical excision, histology, and panendoscopy. DISCUSSION: We argue for the first time the hypothesis that congenital branchial cysts and BCCC tumors may result from progenitor cell rests of the embryological branchial development. After a period of dormancy, these cells could eventually awake and proliferate, thus giving rise to branchial cleft cysts. With the acquirement of mutations due to genomic instability, some clones of these cells could transform to malignant stem cells, thus clinically manifesting as BCCC. CONCLUSIONS: The wide recognition of stem cells and their role in carcinogenesis provides a new context for the etiopathogenesis of controversial and rare entities such as the BCCC.

Is there an association with Wilson's disease and multiple tongue dysplasia lesions and in situ carcinoma? A case-report based literature review.

Wilson's Disease (WD) is a rare autosomal recessive hereditary disease with copper accumulation in the body, particularly the liver, brain and cornea. WD is widely treated with chelation agents who enable the copper excretion. Since high concentrations of copper are toxic, WD is associated with hepatocellular carcinoma and cholangiocarcinoma, with low incidence of other types of cancer. We present a case of a 33 year old man who was treated in the Oral and Maxillofacial Surgery Department of the Aristotle University of Thessaloniki with multiple dysplasia lesions and an in situ carcinoma of the tongue, which is to our knowledge the first case report of oral lesions to a patient with WD. Literature is reviewed on copper levels on patients with head and neck cancer, and on chelation agents and their effect on cancer cells.

Clinical, histological and demographic predictors for recurrence and second primary tumours of head and neck basal cell carcinoma. A 1062 patient-cohort study from a tertiary cancer referral hospital.

Basal cell carcinoma (BCC) accounts for nearly 25% of all cancers in the human body and for almost 75% of skin malignancies; approximately 85% of basal cell carcinomas develop in the head and neck region. Limited demographic, clinical and histological predictors for second primary and/or recurrent BCC have been identified to date. Our objective was to identify predictors of recurrence and second primary tumour development of BCC in the head and neck region. We included 1062 patients with a histologically confirmed diagnosis of BCC. Multivariate and Cox regression analysis were used to access demographic, clinical and histological predictors. Study follow up included 4,302 patient-years, each patient was followed-up for an average 4.0 +/- 1.8 years (range 1-12). Overall recurrence rate was 4%. High-risk histology type was associated with an increased risk for recurrence (odds ratio (OR) = 3.47, 95%CI: 1.07-11.25). We calculated a 4-fold increased risk for recurrence with positive excision margins (OR = 4.31, 95%CI: 1.82-10.22), a 21% increased risk for recurrence (OR = 1.21, 95%CI: 1.06-1.37) and a 25% increased risk for second primary BCC development (OR = 1.25, 95%CI: 1.17-1.34) per year of follow-up. The median time free of second primary tumour was 7 years, while the median time free of recurrence was 12 years. The strongest predictors for recurrence are positive excision margins and high-risk histology type, indicating the need for additional patient care in such cases.

Craniofacial surgical management of a patient with systematic juvenile idiopathic arthritis and Crohn's disease.

Juvenile idiopathic arthritis (JIA) is the most common chronic inflammatory disease in early age. It affects one or more joints, lasts more than 3 weeks, and appears in patients younger than 16 years. Juvenile idiopathic arthritis is classified according to the International League of Associations for Rheumatology consensus depending on the number of affected joints in the beginning of the disease. When JIA affects the temporomandibular joint, the development of the mandible is constrained. Patients show a tendency toward retrognathism and a vertical facial development pattern. The purpose of this study was to present a rare case of a young teenager who experienced JIA and Crohn's disease at the same time. The patient was referred to the hospital for aesthetic and functional problems, mainly convex facial profile and obstructive sleep apnea caused by the craniofacial abnormality. The patient was treated by sagittal split mandibular advancement osteotomy and advancement genioplasty. The mechanisms of obstructive sleep apnea development and the surgical treatment through osteotomies are commentated on. The surgical outcome is functionally and aesthetically favorable and solid 2 years after the operation. Surgical management of the craniofacial region can be a problem-solving treatment modality for patients with juvenile arthritis.

Lipoma of Parotid Gland: Report of Two Cases.

INTRODUCTION: Lipoma is a benign mesenchymal tumor originating from adipose tissue cells. In the head and neck, this tumor is not common. The occurrence of lipomas in the parotid gland is extremely rare, ranging between 0.6 and 4%. CASE REPORTS: In this article, we present two clinical cases of lipomas of the superficial lobe of the parotid gland. DISCUSSION: Clinical diagnosis of parotid gland lipomas is difficult, while usually, they are asymptomatic and presented as a painless swelling of the parotid gland. Tomographic imaging modalities are very accurate in preoperative diagnosis in contrast to FNA or FNAB which is not helpful in the case of lipoma. This pathological entity should be considered in the differential diagnosis of parotid gland's mass lesions. The usual indication for surgical intervention is cosmetic appearance. Meticulous surgical excision should be performed to avoid disturbing adverse events.

Synchronous Ganglioneuroma and Schwannoma Mistaken for Carotid Body Tumor.

Ganglioneuromas are a very rare benign neural tumor, commonly derived from the ganglia of the sympathetic system, and are composed of mature Schwann cells, ganglion cells, and nerve fibres. They may arise anywhere from the base of the skull to the pelvis along the paravertebral sympathetic plexus. We report a rare case of synchronous ganglioneuroma and schwannoma, mistaken for carotid body tumor. The coexistence of these two entities in head and neck region is very rare.

Primary mucosal melanoma of the oral cavity: current therapy and future directions.

BACKGROUND: Primary mucosal melanoma of the oral cavity is a highly aggressive malignancy of melanocytic origin. The aim of this study is to report a case series of oral mucosal melanomas (OMMs) and provide a review of the literature with regard to treatment guidelines for and prognosis of this pathologic entity. STUDY DESIGN: We report three cases of OMMs treated in our institutions and the results of a literature review, in which the words "oral" and "mucosal melanoma" were used as the main keywords. RESULTS: Surgical resection of the primary tumor with wide resection margins appears to be the recommended primary treatment modality with the aim to achieve tumor-free margins. Elective neck dissection and adjuvant radiotherapy have been advocated for locoregional control. Chemotherapy has not been shown to improve survival and is mainly used for palliative purposes. Immunotherapy and biochemotherapy seem to significantly improve survival and could open new therapeutic horizons. CONCLUSIONS: The prognosis of OMMs remains poor despite treatment. Early diagnosis and aggressive surgical resection followed by adjuvant therapies could be the key to improving survival. Multicenter randomized clinical trials, which may be difficult to conduct because of the rarity to the lesion, would aid in the development of new strategies.

Ewing's Sarcoma of Mandible: An Impressive Case of Spontaneous Mandible Regeneration.

Ewing's sarcoma (ES) is an uncommon aggressive bone malignancy that mainly affects children and adolescents. Mandible involvement is quite rare and usually represents metastasis from another skeletal site. Combined therapy including wide surgical resection and preoperative and postoperative chemotherapy has been demonstrated as the mainstay of therapeutic approach. As improved therapeutic modalities have significantly increased survival over the last decades, functional and esthetic reconstruction of the postmandibulectomy defect is usually necessary for patient's total rehabilitation. We report a case of ES of the mandible with special consideration to the postresection spontaneous structural and functional regeneration of the mandible. HOW TO CITE THIS ARTICLE: Chatzistefanou I, Kabesi S, Paraskevopoulos K, Koliouskas D, Antoniades K. Ewing's Sarcoma of Mandible: An Impressive Case of Spontaneous Mandible Regeneration. Int J Clin Pediatr Dent 2016;9(3):273-277.

Juvenile angiofibroma: report of a case with intraoral presentation.

We report a case of juvenile angiofibroma that presented as an ulcerative exophytic mass on the posterior hard palate of a 14-year-old male patient. The clinical features, diagnosis, and management of an extremely rare intraoral presentation of this benign lesion are reviewed.

Orthodontic and periodontal considerations in managing teeth exhibiting significant delay in eruption.

AIM: Significant delay in tooth eruption may be observed in many orthodontic patients. This delay may lead to complications, such as devitalization, ankylosis, external root resorption, injury to the adjacent teeth, and mucogingival problems. These complications may result in prolonged treatment time, esthetic deformities, damage to the periodontal tissues, and tooth loss. This report investigates the special considerations needed in treating such cases. METHODS: Combined orthodontic and surgical treatment can be used in managing teeth exhibiting significant delay in eruption. RESULTS: A high rate of success can be achieved in terms of esthetics, occlusion, and periodontal health. CONCLUSION: Nontraumatic surgery, adequate control of gingival inflammation, and use of minimal orthodontic forces may ensure a higher percentage of success and prevention of problems associated with teeth exhibiting significant delay in eruption.