Double Lip Surgical Correction in Ascher’s Syndrome: Diagnosis and Treatment of a Rare Condition

Ascher’s syndrome is a rare disease first described in 1920 by Ascher, an ophthalmologist from Prague. The disease is characterized by a double upper lip, blepharochalasis, and nontoxic thyroid enlargement, though the thyroid enlargement may be evident in only 10% to 50% of patients. These deformities, though not posing functional problems, may cause severe psychological distress to the affected person because of the disfiguring effect on smiling. Prompt recognition of this syndrome can prevent unnecessary tests and diagnostic delays, and allow for quicker scheduling of appropriate surgical treatment

Tenascina e fibronectina em adenoma pleomórfico de glândula salivar

OBJECTIVES: To analyze the expression and distribution pattern of extracellular matrix components in pleomorphic adenomas of the major and minor salivary glands and to compare the morphological findings of these tumors with the immunohistochemical expression, considering the different types of stroma predominating in each case. METHODS AND RESULTS: The expression of tenascin (TN) and fibronectin (FN) was analyzed in 23 cases of pleomorphic adenomas, 11 major and 12 minor salivary gland tumors, by the streptavidin-biotin method using anti-tenascin and anti-fibronectin antibodies. In addition, the immunohistochemical results were correlated with the morphological findings of the lesions. All cases analyzed were immunoreactive for the antibodies used. Fibronectin showed strong labeling in fibrous and chondroid stroma, while labeling was weak in hyaline and myxoid stroma. Tenascin expression was more intense in fibrous and chondroid stroma and moderate in hyaline and myxoid stroma. CONCLUSIONS: No difference in the expression of these proteins was observed between major and minor salivary gland tumors.OBJETIVO: Analisar a expressão e o padrão de distribuição de componentes da matriz extracelular em adenomas pleomórficos de glândula salivar maior e menor e comparar os achados morfológicos destes tumores com a expressão imuno-histoquímica considerando os diferentes tipos de estromas presentes em cada caso. MÉTODOS E RESULTADOS: A expressão da tenascina (TN) e fibronectina (FN) foi analisada em 23 casos de adenomas pleomórficos, sendo 11 tumores em glândula salivar maior e 12 em glândula salivar menor, utilizando-se o método da estreptoavidina-biotina para os anticorpos anti-tenascina e anti-fibronectina. Os resultados imuno-histoquimicos foram correlacionados com os achados morfológicos das lesões. Todos os casos foram imunorreativos para a fibronectina mostrando forte expressão nos estromas fibrosos e condróides, embora fraca marcação tenha sido evidenciada nos estromas hialinos e mixóides. A expressão da tenascina foi mais intensa nos estromas condróides e fibrosos, sendo moderada nos estromas hialino e mixóide. CONCLUSÕES: Não houve diferença na expressão destas proteínas entre os tumores de glândula salivar maior e menor

Odontogenic tumors: analysis of 127 cases

De uma série de 5.289 casos de lesões orais e dos maxilares diagnosticadas no Laboratório de Patologia Oral da Faculdade de Odontologia da Universidade Federal do Rio Grande do Norte no período de 30 anos (1970-1999), foram analisados 127 casos de tumores odontogênicos confirmados histologicamente. A lesão mais freqüente foi o odontoma (50,40%) seguida pelo ameloblastoma (30,70%). A prevalência de tumores odontogênicos foi maior nas mulheres e o pico de incidência ocorreu na segunda e terceira décadas de vida. A localização anatômica mais comum foi a mandíbula e não foram encontrados casos de tumores malignos.One hundred and twenty-seven cases of histologically confirmed odontogenic tumors were retrieved from a total of 5,289 oral and maxillary lesions diagnosed at the Division of Oral Pathology, Federal University of Rio Grande do Norte, during a period of 30 years (l970-l999). The most common histological diagnosis was odontoma (50.40%), followed by ameloblastoma (30.70%). The prevalence of odontogenic tumors was greater in females and the peak incidence occurred in the second and third decades of life. The main anatomical location was the mandible, and no malignant tumors were found

Immunohistochemical expression of vascular endothelial growth factor and matrix metalloproteinase-9 in radicular and residual radicular cysts

OBJECTIVE: This study assessed and compared the immunoexpression of vascular endothelial growth factor (VEGF) and matrix metalloproteinase-9 (MMP-9) in radicular cysts (RCs) and residual radicular cysts (RRCs), relating them to the angiogenic index and the intensity of the inflammatory infiltrate. MATERIAL AND METHODS: Twenty RCs and 10 RRCs were evaluated by immunohistochemistry using anti-VEGF and anti-MMP-9 antibodies. The angiogenic index was determined by microvessel count (MVC) using anti-von Willebrand factor antibody. RESULTS: The expression of both VEGF and MMP-9 was higher in RCs than in RRCs. RCs and RRCs presented strong epithelial expression of VEGF, irrespective of the intensity of the inflammatory infiltrate. Lesions with strong expression of MMP-9 showed significantly higher number of immunopositive cells for VEGF (

Immunohistochemical analysis of bone resorption regulators (RANKL and OPG), angiogenic index, and myofibroblasts in syndrome and non-syndrome odontogenic keratocysts

Objective The aim of this study was to immunohistochemically analyse bone resorption regulators (receptor activator of nuclear factor kappa B ligand [RANKL] and osteoprotegerin [OPG]), angiogenic index, and myofibroblasts in Gorlin syndrome-related odontogenic keratocysts (SOKCs) and non-syndrome odontogenic keratocysts (NSOKCs). Study design Twenty-two SOKCs, 22 primary NSOKCs, and eight recurrent NSOKCs were evaluated by immunohistochemistry using anti-RANKL and anti-OPG antibodies. The angiogenic index was determined by microvessel count (MVC) using anti-CD34 antibody. Anti-α-smooth muscle actin (α-SMA) antibody was used for the identification of myofibroblasts. Results Analysis of the expression of RANKL and OPG in the epithelial lining and fibrous capsule did not reveal significant differences between groups (P > 0.05). In the epithelial lining, the RANKL/OPG ratio was RANKL 0.05). In the fibrous capsule, the ratio was RANKL = OPG in most primary (81.8%) and recurrent NSOKCs (75.0%) and in most SOKCs (45.5%) (P > 0.05). No significant differences in the angiogenic index or number of myofibroblasts were observed between primary NSOKCs, recurrent NSOKCs, and SOKCs (P > 0.05). Conclusions The present results suggest that differences in the biological behaviour of SOKCs and NSOKCs may not be related to the expression of RANKL and OPG, to the RANKL/OPG ratio, to the angiogenic index, or to the number of myofibroblasts in these lesions

Clinicopathological analysis of odontogenic tumors over 22 years period : experience of a single center in northeastern Brazil

Odontogenic tumors (OTs) are uncommon neoplastic lesions of the maxilla and mandible, which present difficult diagnosis and therapeutics. This paper aims to determine the frequency and distribution of OTs, over a period of 22 years, at a public university in Northeastern Brazil. We reviewed all cases of OTs from oral pathology laboratory of Federal University of Rio Grande do Norte (UFRN), from 1996 to 2017. The tumors were classified according to the latest (2017) World Health Organization Classification of Tumors. Data on age, gender, anatomic site, symptomatology, radiographic findings and tumor size were analyzed. In the analyzed period, 247 cases of OTs were diagnosed. Epithelial tumors were more common with 127 cases (51.8%). The most common tumors were ameloblastoma (n = 112 / 45.4%), odontoma (n = 89 / 36.1%) and odontogenic myxoma (n = 17 / 6.9%). Malignant odontogenic tumors were extremely rare in the studied population with only 2 cases (0.8%) of diagnosed carcinomas. These tumors were diagnosed in a wide age range, from 5 to 81 years, being more common in the second and third decades of life. In general, the mandible was the most affected anatomic site (n = 162/66%) and the mandible:maxilla ratio was of 2:1. Ameloblastoma was the tumor with the highest number of symptomatic cases (n = 26) and with the highest mean size (cm) with 4.5cm. Odontogenic tumors were rare in the sample studied (2.2%), with ameloblastoma and odontoma being the most common tumors. Continuous studies that show the characteristics of these lesions are fundamental, especially after modifications in the international classification

Bone morphogenetic protein-2/4 and bone morphogenetic protein receptor type IA expression in metastatic and nonmetastatic oral squamous cell carcinoma

Purpose The study aimed to analyze the expression of bone morphogenetic protein-2/4 (BMP-2/4) and its receptor BMPR-IA (BMP receptor type IA) in metastatic and nonmetastatic oral squamous cell carcinoma (OSCC) and its implications for disease prognosis. Materials and methods The experimental group included 16 cases of OSCC without metastasis and 7 cases of OSCC with metastasis. The presence or absence of nodal metastasis was used as a parameter for the evaluation of disease prognosis. Ten cases of oral fibroepithelial hyperplasia were selected as the control group. The expression of BMP-2/4 and BMPR-IA was analyzed by immunohistochemistry. Results In the experimental group with metastasis, strong expression of BMP-2/4 was observed in most cases (71.4%), whereas BMPR-IA exhibited weak expression (85.7%). In the experimental group without metastasis, there was strong expression of BMP-2/4 (62.5%) and BMPR-IA (100%). A significant association was observed between the prognosis of OSCC and the intensity of BMP-2/4 staining (P = .002). Weak immunoreactivity to BMP-2/4 and BMPR-IA was observed in all control specimens. Conclusions The results suggest that strong expression of BMP-2/4, associated with low expression of BMPR-IA, observed in metastatic OSCC has a prognostic value, with the loss of responsiveness to BMPs through the loss of expression of their receptors being indicative of the development of metastasis

Nonodontogenic cysts of the oral and maxillofacial region - demographic profile in a Brazilian population over a 40-year period

The aim of the study was to determine the distribution of histologically diagnosed nonodontogenic cysts (nOCs) over a 40-year period in a Brazilian population. Biopsy records from patients with nOC from the files of the Oral Pathology Service during the period of 1970–2009 were evaluated. Among 10,311 oral biopsies, 58 met the criteria of nOCs. The most frequent nOCs were nasopalatine duct cysts (32.8%), followed by epidermoid cysts (20.7%) and oral lymphoepithelial cysts (17.2%). Nasopalatine duct cysts showed predominance among females (68.4%). Epidermoid cysts were most commonly found in the floor of the mouth (36.4%), tongue (27.3%), and buccal mucosa (27.3%). Oral lymphoepithelial cysts exhibited female prevalence (80.0%) and were commonly located in the tongue (44.4%). The frequency of nOCs found in the population studied here is slightly different from those reported in other case series. Nasopalatine duct cysts, epidermoid cysts, and oral lymphoepithelial cysts were the most common nOCs found, accounting for 70.7% of all nOCs