Abstract The biological behavior of lesions is highly dependent on the imbalance between their proliferative and apoptotic capacity. This study evaluated a correlation between the proliferative and apoptotic rates of different oral vascular anomalies (VAs) by analyzing the immunoexpression of proliferation (Ki-67) and apoptosis (Bcl-2 and Bax) markers in endothelial cells of 20 cases of GLUT-1 positive infantile hemangiomas (IHs), 20 cases of pyogenic granulomas (PGs) and 20 cases of vascular malformations (VMs). Immunoexpression analysis of Ki-67, Bcl-2 and Bax revealed a lower median percentage of positive cells in VMs cases compared to IHs and PGs cases (P <0.001). The Wilcoxon signed‐rank test showed significantly higher percentages of immunostaining for Bax than for Bcl‐2 in IHs (P = 0.048). In the group of PGs, a positive correlation was observed between the immunoexpressions of Ki-67 and Bax (r = 0.476; P = 0.034). Although oral IHs, PGs and VMs present similar clinical and histopathological features, each of these lesions has its etiopathogenic particularities. The results of this study suggest that different biological behaviors of VAs may be related to differences in the proliferative and apoptotic profiles of their endothelial cells.

Resumo O comportamento biológico das lesões é altamente dependente do desequilíbrio entre sua capacidade proliferativa e apoptótica. Neste estudo, avaliou-se se existe correlação entre as taxas proliferativas e apoptóticas de diferentes anomalias vasculares orais (AVs) analisando a imunoexpressão de marcadores de proliferação (Ki-67) e apoptose (Bcl-2 e Bax) em células endoteliais de 20 casos de hemangiomas infantis GLUT-1 positivos (HIs), 20 casos de granulomas piogênicos (GPs) e 20 casos de malformações vasculares (MVs). A análise de imunoexpressão de Ki-67, Bcl-2 e Bax revelou uma porcentagem mediana menor de células positivas nos casos de MVs em comparação aos casos de HIs e GPs (P <0,001). O teste de Wilcoxon mostrou porcentagens significativamente maiores de imunocoloração para Bax do que para Bcl-2 em HIs (P = 0,048). No grupo dos GPs, foi observada correlação positiva entre as imunoexpressões de Ki-67 e Bax (r = 0,476; P = 0,034). Embora HIs, GPs e MVs orais apresentem características clínicas e histopatológicas muito semelhantes, cada uma dessas lesões tem suas particularidades etiopatogênicas. Os resultados deste estudo sugerem que diferentes comportamentos biológicos dos AVs podem estar relacionados a diferenças nos perfis proliferativo e apoptótico de suas células endoteliais.

Abstract Oral mucocele (OM) is the most common lesion of minor salivary glands. The present study aimed to report the clinical and demographic features of a large series of OMs and identify possible predictive variables associated with the recurrence rate of these lesions. A retrospective descriptive cross-sectional study was performed. A total of 43,754 biopsy records from four pathology services in Brazil were analyzed. All cases of OMs were reviewed, and clinical and demographic data were collected. The study comprised 1,002 females (56.2%) and 782 males (43.8%), with a mean age of 19.8±16.4 years (range: 01-87 years) and a 1.3:1 female-to-male ratio. The lower lip (n=1,160; 67.4%), and floor of the mouth (n=172; 10.0%), were the most common affected sites, presenting clinically as nodules (n=978; 79.4%) of smooth surface (n=428; 77.5%) and normal color (n=768, 46.7%). Excisional biopsy was the treatment in most cases (n=1,392; 78.0%). Recurrent OMs represented 6.2% of all diagnosed cases (n=117). OMs recurred more commonly in younger patients (aged<20 years) (p<0.0001), in lesions larger than 2 cm in diameter (p<0.0001), and in those located in the ventral tongue (p=0.0351). Also, recurrence rates were higher significantly in cases treated with laser surgery than in those with conventional surgery (p=0.0005). Patients with OMs should be carefully informed of its possible recurrence, especially when found on the ventral tongue of young patients.

Resumo A mucocele oral (MO) é a lesão mais comum das glândulas salivares menores. O presente estudo teve como objetivo relatar as características clínicas e demográficas de uma grande série de MOs e identificar possíveis variáveis preditivas associadas à taxa de recorrência dessas lesões. Foi realizado um estudo transversal descritivo retrospectivo. Foram analisados 43.754 registros de biópsias de quatro serviços de patologia no Brasil. Todos os casos diagnosticados como MOs foram revisados e dados clínicos e demográficos foram coletados. Participaram do estudo 1.002 mulheres (56,2%) e 782 homens (43,8%), com média de idade de 19,8 ± 16,4 anos (variação: 01-87 anos) e proporção de mulheres para homens de 1,3:1. O lábio inferior (n=1.160; 67,4%) e assoalho da boca (n=172; 10,0%), foram os locais mais acometidos, apresentando-se clinicamente como nódulos (n=978; 79,4%) de superfície lisa (n =428; 77,5%) e coloração normal (n=768, 46,7%). A biópsia excisional foi o tratamento na maioria dos casos (n=1.392; 78,0%). As MOs recorrentes representaram 6,2% de todos os casos diagnosticados (n = 117). As recorrências recorreram mais comumente em pacientes mais jovens (idade < 20 anos) (p < 0,0001), em lesões maiores que 2 cm de diâmetro (p < 0,0001) e naquelas localizadas na superfície ventral da língua (p = 0,0351). Além disso, as taxas de recorrência foram significativamente maiores nos casos tratados com cirurgia a laser do que aqueles com cirurgia convencional utilizando bisturi (p = 0,0005). Pacientes com mucoceles devem ser informados sobre uma possível recorrência, principalmente quando encontrados no lábio ou assoalho bucal de pacientes jovens.

ABSTRACT Objective: To compare salivary and serum biochemical levels in patients with chronic renal failure undergoing hemodialysis. Material and Methods: The sample was composed of 57 patients treated in Hemodialysis Reference Centers, from a state of Northeastern Brazilian, with age ≥21 years old with at least 3 months of hemodialysis treatment time. Serum data were obtained from records. Unstimulated and stimulated saliva were collected. Flow rate (mL/min) was measured. Spectrophotometry was performed for the measurement of salivary levels of calcium (570 nm), urea (340 nm), and creatinine (510 nm). Statistical analysis used Mann Whitney and Kruskal-Wallis tests (p<0.05). Results: Unstimulated and stimulated salivary flow rates were 0.43 mL/min and 1.69 mL/min, respectively. There was significant difference (p<0.001) of levels of calcium (5.41 mg/dL and 9.70 mg/dL), urea (118.03 mg/dL and 183.22 mg/dL) and creatinine (0.59 mg/dL and 9.20 mg/dL) between saliva and serum, respectively. Concerning the time of hemodialysis, salivary and serum calcium not exhibited significant association; however, serum urea (p=0.012) and serum creatinine (p=0.025) showed significant association to the time of hemodialysis. Conclusion: Salivary biochemical levels of urea, creatinine and calcium can indicate the presence of a possible chronic renal failure and the saliva demonstrated to be a potential auxiliary biofluid for clinical monitoring renal alterations.

Abstract The aim of this study was to investigate the epidemiological and clinical characteristics of chronic inflammatory periapical diseases in different regions of Brazil and to compare with data from the literature. A multicenter study was carried out in four Brazilian referral centers in oral diagnosis. Histopathological records were reviewed, and all cases diagnosed microscopically as periapical granuloma, radicular cyst, and periapical abscess were included. Demographic and clinical data were collected. Descriptive statistics and Pearson’s chi-square test were performed. A total of 10,381 cases of chronic inflammatory periapical diseases were found (13.8% of 74,931 archived specimens) over a period of 65 years. Radicular cysts were the most common lesion (59.9%). Women (56.1%) with a mean age of 37.01 years old (range 13 to 100 ± 14.42) and people of white skin color (59.2%) were the most affected individuals by chronic inflammatory periapical diseases. The lesions were generally asymptomatic (28.1%), located in the maxilla (60.1%), and posterior region (49.8%). The radicular cysts were larger when compared to periapical granulomas (p < 0.001). The disagreement between the clinical and histopathological diagnoses was higher when the final diagnosis was a periapical granuloma (p < 0.001). Chronic inflammatory periapical diseases continue to be common lesions affecting mainly adults. This should be a consequence of the burden of untreated caries in permanent teeth. Women are more affected and radicular cyst was the most common lesion.

Abstract Chloride intracellular channel-4 (CLIC4) is regulated by p53 and tumor necrosis factor-α (TNF-α), it is linked to the increase of transforming growth factor-β (TGF-β), and myofibroblastic differentiation in skin carcinogenesis. This study analyzed the immunoexpression of CLIC4, p53, TGF-β, TNF-α, and α-SMA in 50 actinic cheilitis (AC) and 50 lower lip squamous cell carcinoma (LLSCC). AC and LLSCC immunoexpression were categorized as score 1 (<5% positive cells), 2 (5-50%) or 3 (>50%). For CLIC4, nuclear and cytoplasmic immunostaining of epithelial cells was considered individually. For morphologic analysis, the World Health Organization criteria were used to epithelial dysplasia grade of ACs, and Bryne grading of malignancy system was applied for LLSCC. Higher nuclear CLIC4 (CLIC4n) and TGF-β were observed in ACs with low-risk of transformation, while cytoplasmic CLIC4 (CLIC4c), p53 and TNF-α were higher in the high-risk cases (p<0.05). In LLSCCs, CLIC4c was higher in cases with lymph node metastasis, advanced clinical stages, and histological high-grade malignancy. p53 expression was higher in high-grade LLSCCs, whereas TGF-β decreased as the clinical stage and morphological grade progressed (p<0.05). ACs showed an increased expression of CLIC4n and TGF-β, while CLIC4c and α-SMA were higher in LLSCCs (p<0.0001). Both lesions showed negative correlation between CLIC4n and CLIC4c, while in LLSCCs, negative correlation was also verified between CLIC4c and p53, as well as CLIC4c and TGF-β (p<0.05). Change of CLIC4 from the nucleus to cytoplasm and alterations in p53, TGF-β, TNF-α, and α-SMA expression are involved in lip carcinogenesis.

Resumo O canal intracelular de cloreto 4 (CLIC4) é regulado pela p53 e fator de necrose tumoral α (TNF-α) e está relacionado ao aumento do fator de crescimento transformador β (TGF-β) e na diferenciação miofibroblástica na carcinogênese cutânea. Este estudo analisou a imunoexpressão de CLIC4, p53, TGF-β, TNF-α e α-SMA em 50 queilites actínicas (QA) e 50 carcinomas de células escamosas de lábio inferior (CCELI). A imunoexpressão da QA e CCELI foram categorizadas em escore 1 (<5% de células positivas), 2 (5-50%) ou 3 (>50%). Para CLIC4, a imunomarcação nuclear e citoplasmática das células epiteliais foi considerada separadamente. Para análise morfológica, foram utilizados os critérios da Organização Mundial da Saúde para a gradação das displasias epiteliais nas QAs, e o sistema de gradação de malignidade de Bryne foi utilizado para os casos de CCELIs. Alta imunoexpressão de CLIC4 nuclear (CLIC4n) e TGF-β foi observada em QA de baixo risco de transformação, enquanto CLIC4 citoplasmática (CLIC4c), p53 e TNF-α foram elevadas nos casos de alto risco (p<0.05). No CCELI, a imunoexpressão de CLIC4c foi maior em caos com metástase linfonodal, estágio clínico avançado e alto grau histológico de malignidade. A expressão de p53 foi elevada em CCELI de alto grau, enquanto o TGF-β diminuiu à medida que o estádio clínico e o grau morfológico progrediram (p<0.05). QAs exibiram uma elevada expressão de CLIC4n e TGF-β, enquanto o CLIC4c e α-SMA foram elevados em CCELIs (p<0.0001). Ambas as lesões mostraram correlação negativa entre CLIC4n e CLIC4c, enquanto nos CCELIs, também se verificou correlação negativa entre CLIC4c e p53, assim como entre CLIC4c e TGF-β (p<0.05). Alteração do CLIC4 do núcleo para o citoplasma e alterações na expressão de p53, TGF-β, TNF-α, e α-SMA estão envolvidas na carcinogênese labial.

Abstract Regulatory T (Treg) cells can suppress antitumor immune response, but little is known about possible age-related differences in the number of these cells in the microenvironment of oral tongue squamous cell carcinoma (OTSCC). The aim of this study was to determine the number of FoxP3+ Treg cells in the microenvironment of OTSCC in young (≤ 45 years) and older (≥ 60 years) patients, and to correlate the findings with clinicopathological parameters (sex, tumor size/extent, regional lymph node metastasis, clinical staging, and histopathological grade of malignancy). Forty-eight OTSCCs (24 diagnosed in young patients and 24 diagnosed in older patients) were selected. Lymphocytes exhibiting nuclear immunopositivity for FoxP3 were quantified at the tumor invasive front and the results were analyzed statistically using the non-parametric Mann-Whitney test. FoxP3+ lymphocytes were observed in all cases assessed. The number of FoxP3+ lymphocytes in OTSCC tended to be higher in older patients (p = 0.055). Analysis of OTSCC in males and in early clinical stages revealed a higher number of Treg cells in older patients than in young ones (p < 0.05). In older patients, the number of Treg cells tended to be higher in smaller tumors (p = 0.079). Tumors with intense inflammatory infiltrate exhibited a larger number of Treg cells, both in young (p = 0.099) and older patients (p = 0.005). The results suggest a greater participation of Treg cells in immunoinflammatory responses in the microenvironment of OTSCC in older patients, particularly in males and in early stages.

Abstract Objective: To perform an in vitro analysis of antibacterial and antifungal potential of an alcoholic extract from the leaves of Guapira Graciliflora Mart. against oral microorganisms and determine its chemical composition. Material and Methods: A hydroalcoholic extract of the leaves form G. graciliflora was obtained through maceration, vacuum concentration and freeze-drying. Antibacterial and antifungal activities were evaluated against Streptococcus mutans, Streptococcus salivarius, Streptococcus oralis, Streptococcus parasanguinis, Streptococcus mitis and strains of Candida albicans using broth microdilution method. Phytochemical analysis determined the total phenolic compounds, protein concentration and total of sugars present in the extract. Results: G. Graciliflora demonstrated antifungal activity against the LM 11 and LM 410 clinical isolates of C. albicans (MIC 0.5 mg/mL and 2 mg/mL, respectively). The other microorganisms tested were resistant to the extract. The phytochemical analysis revealed 3% proteins, 13% total sugars and 17% phenolic compounds. Conclusion: G. Graciliflora has antifungal activity against clinical strains of C. albicans and exhibits proteins, sugars and phenolic compounds in its chemical composition.

ABSTRACT Spindle cell squamous cell carcinoma of the tongue is a rare variant of squamous cell carcinoma. This paper reports the case of a spindle cell squamous cell carcinoma of the tongue, in a 64-year-old male patient, and presents a review of the etiopathogenesis, clinicopathological and immunohistochemical features and treatment of the malignancy. The patient presented for evaluation of a painful swelling on his tongue. Extraoral examination revealed palpable submandibular and superior cervical lymph nodes. Based on the presumptive diagnoses of squamous cell carcinoma or malignant salivary gland neoplasm, an incisional biopsy was performed. Histopathological analysis showed a proliferation of atypical spindle cells, exhibiting extensive pleomorphism. Tumor cells were positive for vimentin, P53 and alpha-smooth muscle actin, focally positive for epithelial membrane antigen and P63, and negative for pan-cytokeratin (AE1/AE3), CK7, CD138, CD34, CD56, and S-100. The positivity index for Ki-67 was approximately 40%. The diagnosis of spindle cell squamous cell carcinoma was established and the patient was referred to a head and neck surgery service. In the oral cavity, spindle cell squamous cell carcinoma of the tongue is an aggressive variant of squamous cell carcinoma, which usually presents as an exophytic mass located on the tongue of elderly males. Due to its distinct histopathological characteristics, immunohistochemistry is a valuable and helpful tool to establish the diagnosis of spindle cell squamous cell carcinoma of the tongue.

RESUMO O carcinoma de células escamosas de células fusiformes da língua, é uma variante rara do carcinoma de células escamosas. Este trabalho relata o caso de um paciente do sexo masculino de 64 anos de idade com carcinoma de células escamosas de células fusiformes da língua. Foi feita uma revisão dos aspectos relacionados a etiopatogenia, características clinicopatológicas e imuno-histoquímicas, além do tratamento dessa neoplasia. O paciente procurou atendimento para avaliação de tumefação dolorosa na língua. O exame extraoral revelou linfonodos cervicais submandibulares e cervicais superiores palpáveis. Foi realizada biópsia incisional com base nas hipóteses diagnósticas de carcinoma de células escamosas ou neoplasia maligna de glândula salivar. A análise histopatológica demonstrou proliferação de células fusiformes atípicas, exibindo extenso pleomorfismo. As células tumorais foram positivas para vimentina, p53 e alfa-actina de músculo liso, focalmente positivas para antígeno de membrana epitelial e P63, e negativas para pancitoqueratina (AE1/AE3), CK7, CD138, CD34, CD56 e S-100. O índice de positividade para o Ki-67 foi de aproximadamente 40%. Foi feito o diagnóstico de carcinoma de células escamosas, de células fusiformes, e o paciente foi encaminhado para um serviço de cirurgia de cabeça e pescoço. Na cavidade oral, o carcinoma de células escamosas de células fusiformes da língua, é uma variante agressiva do carcinoma de células escamosas, que geralmente se apresenta como massa exofítica localizada na língua de homens idosos. Devido às suas distintas características histopatológicas, a imuno-histoquímica é uma ferramenta valiosa e útil para estabelecer o diagnóstico de carcinoma de células escamosas de células fusiformes da língua.

Abstract Objective: To evaluate self-medication for toothache and its associated factors in children and adolescents. Material and Methods: A cross-sectional study was carried out with 252 children/adolescents aged 6-16 years. A questionnaire was applied with questions related to demographic and socioeconomic characteristics; experience of, and self-medication for, toothache; as well as aspects related to the condition. Descriptive and inferential statistical analyses (Pearson's Chi-square test and Fisher's exact test) were performed, with a 5% significance level. Results: The prevalence of toothache was 41.7%. In 96 cases analyzed, there was prevalence of 69.8% of self-medication for toothache. There were no statistically significant associations between self-medication for toothache and variables related to the children/adolescents (gender and age), their parents or guardians (age and schooling), socioeconomic characteristics (family income and number of people in the household) and aspects related to toothache (fever, crying and school absenteeism) (p>0.05). The most commonly used drug was paracetamol (60.7%), whose choice was based to its previous use by the study population for conditions not related to toothache (47.8%). Conclusion: There was a high prevalence of self-medication for toothache in the study population. No independent variable was associated with self-medication practice.

Abstract The aim of this study was to evaluate the immunoexpression of human leukocyte antigen-DR (HLA-DR) in actinic cheilitis (AC) and lower lip squamous cell carcinoma (LLSCC), and to correlate the findings with clinical (tumor size/extent, regional lymph node metastasis, and clinical stage) and histopathological (grade of epithelial dysplasia and inflammatory infiltrate for AC and histopathological grade of malignancy for LLSCC) parameters. Twenty-four AC and 48 LLSCC cases (24 with regional nodal metastasis and 24 without regional nodal metastasis) were selected. The scores of immunopositive cells for HLA-DR in the epithelial component of the lesions were assessed and the results were analyzed statistically using the nonparametric Mann-Whitney test. Epithelial expression of HLA-DR was observed in only five (20.8%) cases of AC (two low-grade and three high-grade lesions), with a very low median score of immunopositivity. By contrast, expression of HLA-DR was found in most LLSCC (97.9%), with a relatively high median score of positive cells. The score of HLA-DR-positive cells tended to be higher in tumors with regional lymph node metastasis, tumors in advanced clinical stages, and low-grade tumors, but the difference was not statistically significant (p > 0.05). In addition, there was a tendency towards higher expression of HLA-DR in highly/moderately keratinized tumors, and tumors with little/moderate nuclear pleomorphism (p > 0.05). The results suggest a potential role of HLA-DR in lip carcinogenesis, particularly in the development and progression of LLSCC. The expression of this protein can be related to the degree of cell differentiation in these tumors.

Abstract Introduction Actinic cheilitis is a inflammatory condition affecting mainly the lower lip and it is caused by chronic and excessive exposure of the lips to the ultraviolet radiation in sunlight. Objective Identifying clinical and histopathologic characteristics in 40 cases histopathologically diagnosed as actinic cheilitis. In addition, to investigate possible associations between these aspects. Method Defined as an observational, transversal, retrospective and descriptive study, it registered data regarding age, gender, occupation, symptomatology, records of sun exposure, frequency of sunblock use, tabagism, skin color, clinical aspect and histopathological classification. The data was submitted to the chi square test of Pearson (p<0.05). Result There was a predominance of male gender, leucodermia, and ages ranging between 50 and 60 years. The most common occupation was farming. From our sample, 85% had history of chronic sun exposure, in which 50% reported the use of some type of sunblock and only 25% were smokers. The main clinical condition was non-ulcerated leukoplakia and in the histopathological study, the hyperkeratosis were more common. A correlation between the degree of tissue alteration verified in the histopathological diagnosis and the studied clinical variables was not established (p=0.112). Conclusion The clinical aspect of the wound can conceal tissue alterations in different stages, emphasizing the importance of a premature diagnosis.

Resumo Introdução A queilite actínica e uma condição de natureza inflamatória que acomete o lábio inferior, e é causada pela exposição prolongada e crônica dos lábios à radiação ultravioleta proveniente dos raios solares. Objetivo Identificar as características clínicas e histopatológicas em uma série de 40 casos diagnosticados histopatologicamente como queilite actínica. Além disso, investigar possíveis associações entre estes aspectos. Método Caracterizado como um estudo observacional, transversal, retrospectivo e descritivo. Foram registrados dados a respeito da idade, gênero, ocupação, sintomatologia, histórico de exposição ao sol, uso de proteção solar, tabagismo, cor da pele, aspecto clínico e classificação histopatológica. Os dados foram submetidos ao teste de Qui-Quadrado de Pearson (p<0,05). Resultado Houve uma prevalência do gênero masculino, leucodermas, com faixa etária entre 50 e 60 anos e a ocupação mais presente foi a de agricultor. Da amostra, 85% apresentou histórico de exposição crônica ao sol, onde 50% relatou uso de algum tipo de proteção solar e apenas 25% era tabagista. A principal apresentação clínica foi leucoplasia não ulcerada, e no estudo histopatológico as hiperceratoses foram as mais presentes. Não foi possível correlacionar o grau de alteração tecidual verificada no diagnóstico histopatológico com as variáveis clínicas estudadas (p=0,112). Conclusão Não foi possível correlacionar o grau de alteração tecidual verificada no diagnóstico histopatológico com os aspectos clínicos observados. O aspecto clínico da lesão pode mascarar alterações teciduais em diversos estágios, o que enaltece a importância do diagnóstico precoce.

Abstract The immunoexpression of p16, p53, and Bax in oral tongue squamous cell carcinoma (OTSCC) in young and elderly patients is assessed based on clinical and morphological parameters. The sample consists of 60 OTSCC cases: 30 in young (age ≤ 45 years) and 30 in elderly (age ≥ 60 years) patients. Clinical (tumor size, regional node metastasis, distant metastasis, and clinical stage) and morphological (histological grade of malignancy) parameters were evaluated. Immunohistochemical quantitative analysis was performed using anti-p16, anti-p53, and anti-Bax antibodies. None of the evaluated proteins exhibited statistically significant differences between young and elderly patients (p>0.05). There was a significant association of p16 immunoexpression with clinical parameters in elderly patients. There were no associations of p53 and Bax with any of the clinico-morphological parameters. Correlations between p16 and Bax and between p53 and Bax immunoexpression were observed in young patients (r = 0.363; p = 0.048) and in elderly patients (r = 0.433; p = 0.017), respectively. In conclusion, the assessed proteins could not be used to determine differences in the biological behavior of OTSCC between young and elderly patients. Therefore, all proteins activated the pro-apoptotic pathway of OTSCC in both groups.

ABSTRACT Melanotic neuroectodermal tumor of infancy is a rare and fast-growing neoplasm. In this study, we describe the case of a 6-month-old female patient, who presented swelling in the anterior maxilla. Tomographic reconstruction showed an unilocular hypodense and expansive area associated with the upper right central primary incisor. The presumptive diagnoses were dentigerous cyst, adenomatoid odontogenic tumor, melanotic neuroectodermal tumor of infancy and rhabdomyosarcoma, and an incisional biopsy was performed. Microscopically, the lesion revealed a biphasic cell population, consisting of small, ovoid, neuroblastic-like cells and epithelioid cells containing melanin. Immunohistochemically, the melanocyte-like component was strongly and diffusely positive for HMB-45 and Melan-A, but weakly positive for S100. Based on these findings, definitive diagnosis of melanotic neuroectodermal tumor of infancy was established. Then, enucleation of the lesion was performed by careful curettage. After 2 year follow-up, no clinical or radiographical evidence of recurrence was verified. The present case highlights the importance of early diagnosis and therapeutic intervention at the appropriate time to achieve a favorable outcome for the patient.

RESUMO O tumor neuroectodérmico melanocítico da infância é uma neoplasia rara e de crescimento rápido. Neste estudo, relata-se o caso de uma paciente do sexo feminino de 6 meses de idade, que apresentou tumefação na região anterior de maxila. A reconstrução tomográfica revelou área unilocular hipodensa e expansiva associada ao incisivo central superior direito decíduo. Realizou-se biópsia incisional, considerando as hipóteses diagnósticas de cisto dentígero, tumor odontogênico adenomatoide, tumor neuroectodérmico melanocítico da infância e rabdomiossarcoma. Microscopicamente, a lesão revelou população celular bifásica, consistindo de células pequenas, ovoides, de aparência neuroblástica, e de células epitelioides, contendo melanina. A análise imuno-histoquímica demonstrou que o componente celular contendo melanina era positivo de forma intensa e difusa para HMB-45 e Melan-A, mas levemente positivo para S100. Com base nestes achados, foi estabelecido o diagnóstico definitivo de tumor neuroectodérmico melanocítico da infância. Em seguida, foi realizada a enucleação da lesão com curetagem cuidadosa. Após 2 anos de acompanhamento, não foram verificadas evidências clínicas ou radiográficas de recorrência. O presente caso destaca a importância do diagnóstico precoce e da intervenção terapêutica no momento apropriado, a fim de alcançar um desfecho favorável para o paciente.

ABSTRACT Orthokeratinized odontogenic cysts are developmental odontogenic cysts, presenting with low frequency, low rate of recurrence and their ethiopathogenesis is unknown. Radiographically, they show a radiolucent area in the mandibular posterior region. The aim of this report is to describe an unusual case of orthokeratinized odontogenic cysts, radiographically exhibiting radiopaque areas with an aspect of calcifications in the lesion. Based on this, the clinical hypothesis of ameloblastic fibro-odontoma was suggested. After incisional biopsy and microscopic analysis, the conclusive diagnosis was orthokeratinized odontogenic cysts. The radiopaque foci were observed to be associated with a foreign body reaction. The patient was submitted to surgery under local anesthesia, with intraoral access for complete excision of the lesion and to re-establish esthetics. After follow-up of 24 months there were no signs of recurrence. Knowledge of this type of reaction is important because of the risk that the lesion may mimic a potentially more aggressive lesion, affecting the choice of treatment.

RESUMO Os cistos odontogênicos ortoceratinizados são cistos de desenvolvimento de origem odontogênica, de baixa frequência, etiopatogenia desconhecida e baixa taxa de recorrência. Radiograficamente, apresenta-se como uma área radiolúcida em região posterior de mandíbula. O objetivo deste artigo é descrever um caso não usual de cistos odontogênicos ortoceratinizados, que exibia, radiograficamente, focos radiopacos semelhantes à calcificações, no interior da lesão, sugerindo diagnóstico clínico de fibro-odontoma ameloblástico. Após a biópsia incisional e análise microscópica, o diagnóstico conclusivo foi de cistos odontogênicos ortoceratinizados. Observou-se que os focos radiopacos estavam associados, microscopicamente, a presença de uma reação de células gigantes multinucleadas por corpo estranho. O paciente foi tratado cirurgicamente, sob anestesia local, com acesso intrabucal para remoção total da lesão, com o restabelecimento da estética e sem sinais de recidiva após 24 meses. A importância do conhecimento deste tipo de reação dar-se pelo risco de mimetizar uma lesão potencialmente mais agressiva, direcionando a escolha do tratamento da lesão.

Abstract Teratomas are tumors composed by tissues derived from the three germ cell layers, and they are relatively uncommon in head and neck. The term epignathus has been applied to teratomas from the oropharynx. This paper reports the case of a giant epignathus teratoma discovered at birth, which was successfully managed and followed up for 7 years. A newborn boy presented a polypoid tumor mass exteriorizing through the mouth over a length of 9 cm, with some surface areas resembling skin and others exhibiting hair. Computed tomography showed that the mass arose deep from the left hemiface. Alpha-fetoprotein (AFP) levels were high (316,000 ng/mL). Surgery was performed and microscopic analysis confirmed the diagnosis of mature teratoma. Because of residual tumor and high AFP levels, the patient was submitted to chemotherapy, resulting in complete regression of the lesion and normalization of AFP levels. Surgical repair of a cleft palate was performed at 5 years of age. At 7 years of age, the patient was in good general health and showed no clinical signs of recurrence. Although epignathus is a rare condition, it should be diagnosed in the fetus as early as possible. Prenatal care provides unquestionable benefits, providing the early diagnosis of anomalies that can jeopardize the life of the fetus and contributing to the indication of cases that require treatment before birth.

Resumo Teratomas são tumores constituídos por tecidos derivados das três camadas de células germinativas e são relativamente incomuns em cabeça e pescoço. O termo epignathus tem sido utilizado para designar teratomas que se originam na orofaringe. Este artigo relata o caso de um teratoma epignathus gigante descoberto ao nascimento, o qual foi tratado com sucesso e proservado por 7 anos. Um menino recém-nascido apresentou uma massa tumoral polipoide que se exteriorizava através da boca por uma extensão de 9 cm, com regiões da superfície semelhantes à pele e outras exibindo pelos. Exame de tomografia computadorizada revelou que a massa se originava profundamente na hemiface esquerda. Os níveis de alfa-fetoproteína (AFP) se apresentavam elevados (316.000 ng/mL). Foi realizada cirurgia e a análise microscópica confirmou o diagnóstico de teratoma maduro. Por apresentar lesão residual e altos níveis de AFP, o paciente foi submetido à quimioterapia, resultando em regressão completa da lesão e normalização dos níveis de AFP. Correção cirúrgica de uma fenda palatina foi realizada aos 5 anos de idade. Aos 7 anos de idade, o paciente apresentava um bom estado de saúde geral, sem sinais clínicos de recorrência da lesão. Embora o epignathus seja uma condição rara, seu diagnóstico no feto deve ser realizado o mais precocemente possível. O cuidado pré-natal proporciona benefícios inquestionáveis, permitindo o diagnóstico precoce de anomalias que podem comprometer a vida do feto e contribuindo para a indicação de casos que requerem tratamento antes do nascimento.