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Introdução: Tanto a oligodontia/hipodontia, quanto a presença de hiperdontia na Síndrome de Gardner (SG) têm sido associadas ao desenvolvimento de Câncer Colorretal (CCR), com envolvimento dos genes AXIN2 e APC, respectivamente. Nessas condições, a evolução para CCR ocorre a partir de pólipos intestinais pré-existentes. Objetivos: Verificar a presença de evidência científica da associação de oligodontia/hipodontia e hiperdontia com o CCR, bem como a prevalência de evolução para CCR nesses quadros. Métodos: Revisão integrativa com levantamento de estudos nas bases de dados PubMed, SciELO, LILACS, Scopus e Embase sem restrição de idioma e ano de publicação. Resultados: Foram incluídos 16 artigos - 10 oligodontia/hipodontia e 6 de hiperdontia, todos com análise molecular para os genes. Dos 10 estudos com mutações no gene AXIN2, 3 (30%) evoluíram para CCR. Na SG em 1 estudo (16,6%) houve evolução para CCR. Com relação às anomalias, na SG a hiperdontia foi relatada em 4 estudos e dentes impactados em 6 estudos. Nos 10 estudos para o gene AXIN2 foram relatados oligodontia/hipodontia sendo os dentes mais ausentes o incisivo lateral superior, pré-molares e molares. Conclusão: A prevalência de CCR foi maior em pacientes com oligodontia/hipodontia. Embora ainda não se possa fazer a associação do CCR com a presença dessas anomalias dentárias têm-se na literatura fortes indicadores dessa correlação. Por isso, o papel do cirurgião-dentista é fundamental na identificação de pacientes com hiperdontia e oligodontia/hipodontia para o encaminhamento e realização do rastreamento e tratamento precoce de pólipos adenomatosos.

Introduction The Journal of Morphological Sciences (JMS), the official journal of the Brazilian Society of Anatomy, is an interesting option for researchers to publish in the field of morphology. It includes articles that cover a wide variety of topics, such as gross and microscopic human and animal anatomy, embryology, cell and molecular biology, clinical cases and reviews. We aimed to perform a bibliometric study to analyze the profile of JMS publications from 2000 to 2017, in order to understand in depth the origins of the researches and the subject of the manuscripts published in this journal. Material and Methods This is a descriptive bibliographical review research, with a bibliometric analysis of the scientific production of the JMS between 2000 and 2017. A total of 894 publications were analyzed, and they were distributed in 63 issues and organized into 18 volumes. Three groups of information were considered: a) “identification of the published articles;” b) “origin and authorship of the published articles;” and c) “research subject in the published articles.” Results Most of the published manuscripts were original articles (72.5%) and their subjects were mainly microscopic animal anatomy (27%) and gross human anatomy (26.6%). A total 63.3% of the manuscripts involved at least one Brazilian institution, and 59.4% of those were public institutions. Conclusion This manuscript provides an important contribution to those who are publishing in the JMS, since the authors can find a great deal of information on the quality of the science that is being published in the journal, as well as demographic information on authors and institutions.

Aims to describe a case in which dental changes were observed and investigation proceeded to consider Enamel-Renal-Syndrome (ERS), a rare disorder that associates amelogenesis imperfecta with nephrocalcinosis. Case report an 11-year-old male patient upon intraoral examination revealed generalized gingival hyperplasia, a few teeth were absent clinically and the remaining ones were yellowish-brown in color. The enamel alterations were suggesting of amelogenesis imperfecta. Unerupted teeth with increased pericoronal spaces, suggesting hyperplasic follicles or dentigerous cysts and an enamel with lower thickness and density were observed in the panoramic radiography. The patient was referred for an assay to investigate mucopolysaccharidosis; however, it was negative. A renal ultrasound showed bilateral nephrocalcinosis and laboratory exams, including calcium, phosphate, and creatinine levels were below the average. An incisional gingival biopsy showed numerous round to ovoid basophilic calcifications in the connective tissue. The final diagnosis was ERS. Conclusion Dentists should refer patients with similar clinical presentation for renal ultrasound evaluation in order to rule out the possible diagnosis of ERS.

Adenoid cystic carcinoma (ACC) is a malignant epithelial tumor of the salivary glands that commonly affects the minor salivary glands, and the palate is the most recurrent site. Treatment is challenging because this tumor tends to recur with distant metastases and poor prognosis. A 50-year-old woman complained of numbness in the upper left hemimaxilla, extended to the labial region, nose wing, and nasolabial sulcus, with clinical intra-oral alterations similar to an endodontic fistula tooth 25 palatal mucosa and discrete periapical rarefaction on radiologic evaluation. Endodontic treatment was provided with laser therapy administered for persistent numbness. After 3 months a palate volume increase and small ulcerated region were noted encompassing the region of teeth 24 to 27. The panoramic and tomographic examination was performed showing extensive bone destruction, and the patient underwent a biopsy followed by microscopic and immunohistochemistry evaluation and diagnosis as ACC. The patient was forwarded to a head and neck surgeon.

Pathological parameters have been indicated as tumor prognostic factors in oral carcinoma. Objective: The objective of this study was to investigate the impact of pathological parameters on prognosis of patients affected only by tongue and/or floor of the mouth squamous cell carcinoma (SCC). Methodology: In total, 380 patients treated in the Brazilian National Cancer Institute (INCA) from 1999 to 2006 were included. These patients underwent radical resection followed by neck dissection. The clinical and pathological characteristics were recorded. The Kaplan-Meier method and Cox proportional hazards model were used in survival analysis. Overall survival (OS), cancer-specific survival (CSS) and disease-free interval (DFI) were estimated. Cox residuals were evaluated using the R software version 3.5.2. Worst OS, CSS and DFI were observed in patients with tumors in advanced pathological stages (p

A primary cutaneous CD-30 positive T -cell lymphoproliferative disorders are rare and heterogeneous group of primary skin tumors, which include primary cutaneous anaplastic large cell lymphoma (PCALCL) and lymphomatoid papulois. We report a rare an atypical case of PCALCL with an aggressive and refractory behavior that occurred in the lip vermilion, and that had been initially diagnosed as aggressive herpes. The lesion was recurrent and refractory to the CHOEP chemotherapy protocol followed by radiotherapy, but after hyper CVAD (acronym) + brentuximab with a considerable improvement. After 4 cycles of hyper CVAD plus brentuximab, the patient underwent 100% compatible sibling bone marrow transplantation, with success in the procedure. Primary cutaneous anaplastic large cell lymphoma is a rare disease of difficult diagnosis and may be confused with chronic infectious diseases, postponing treatment.

Renal osteodystrophy is a term that encompasses complications of bone metabolism associated with chronic kidney disease. Progressive loss of renal function raises parathyroid hormone levels (PTH) favor high bone turnover and skeletal manifestations. The clinic condition is a diagnostic and management challenge. A 37-year-old man, chronic renal, was referred by endocrinologist for evaluation of expansive gingiva lesion, present for 3 months, with a histopathological diagnosis suggestive of osteosarcoma. On the extraoral examination, facial asymmetry of rapid evolution and intraoral presented bone swelling of the jaws, hard palate, and dental mobility. Radiography and computed tomography findings reveal alterations in cortical and medullary spaces. Laboratory tests showed high parathormone, phosphorus, and alkaline phosphatase. The histopathological exam was reviewed considered the clinical and biochemical history, excluded malignancy and was compatible with renal osteodystrophy. The patient was referred to the head and neck surgeon and submitted parathyroidectomy and followed up by dentist and medical team.

to describe a case in which dental changes were observed and investigation proceeded to consider Enamel-Renal-Syndrome (ERS), a rare disorder that associates amelogenesis imperfecta with nephrocalcinosis.an 11-year-old male patient upon intraoral examination revealed generalized gingival hyperplasia, a few teeth were absent clinically and the remaining ones were yellowish-brown in color. The enamel alterations were suggesting of amelogenesis imperfecta. Unerupted teeth with increased pericoronal spaces, suggesting hyperplasic follicles or dentigerous cysts and an enamel with lower thickness and density were observed in the panoramic radiography. The patient was referred for an assay to investigate mucopolysaccharidosis; however, it was negative. A renal ultrasound showed bilateral nephrocalcinosis and laboratory exams, including calcium, phosphate, and creatinine levels were below the average. An incisional gingival biopsy showed numerous round to ovoid basophilic calcifications in the connective tissue. The final diagnosis was ERS.Dentists should refer patients with similar clinical presentation for renal ultrasound evaluation in order to rule out the possible diagnosis of ERS.

The aim of this study was to assess gender differences in prognostic factors among patients treated surgically for oral squamous cell carcinoma (OSCC). The medical records of 477 eligible patients (345 males, 132 females) obtained from the Brazilian Cancer Institute were reviewed. Survival was calculated by Kaplan–Meier method. Cox regression models were used to obtain adjusted hazard ratios (aHR) for males and females. Multivariate analysis showed that past tobacco use (aHR 0.2, 95% confidence interval (CI) 0.1–0.7) and regional metastasis (aHR 2.3, 95% CI 1.5–3.5) in males, and regional metastasis (aHR 2.2, 95% CI 1.2–4.3), distant metastasis (aHR 6.7, 95% CI 1.3–32.7), and hard palate tumours (aHR 11.8, 95% CI 3.3–47.7) in females, were associated with a higher risk of death. There were no differences in survival between males and females. Regional metastasis was found to be a negative prognostic factor in OSCC for both genders. Past tobacco use was an independent prognostic factor for worse survival among males, while distant metastasis and hard palate tumours were independent prognostic factors for worse survival among females. Further studies are necessary to corroborate the relationships found in this study.

Eosinophilic ulcer (EU) is a chronic, benign, and self-limiting condition, more common in the tongue of men. This study reports 4 patients diagnosed as having EU. Of the cases studied, 3 involved men and 1 involved a woman, with a mean age of 60 years. All lesions were described as ulcers covered by a membrane of fibrin with raised margins, which were rigid to touch, and a red halo located at lateral tongue. The mean lesion size was 1.45 cm, and the lesions were present for 10 to 60 days. Squamous cell carcinoma was among probable diagnostic hypothesis in all cases. An incisional biopsy was performed, and the histopathologic analysis revealed an EU in all cases with intense inflammatory infiltrate and presence of eosinophils. After the biopsy and removal of the causative agent (if detected), all the lesions regressed. Performing a biopsy is extremely important to differentiate EU from squamous cell carcinoma.

Gardner syndrome (GS) is an autosomal dominant disease characterized by adenomatous intestinal polyps, and if not treated, shows a great chance of becoming malignant. Multiple osteomas in the skull, maxilla, and mandible, along with multiple cutaneous and subcutaneous masses are also characteristics of GS. A 30-year-old woman reported an epidermoid cyst on the frontal bone and a scar on the left shoulder due to osteoma removal. Tooth absence, malocclusion, and osteoma in the palatal region were observed in the intraoral exam. A panoramic radiography analysis revealed permanent teeth retention, supernumerary teeth, and radiopaque areas suggestive of osteomas. Although there were Gardner syndrome manifestations, only after our evaluation, a colonoscopy examination was ordered and intestinal polyps were removed with a diagnosis of tubular adenoma with low grade dysplasia. Today, the patient is under follow-up with a gastroenterologist and oral and maxillofacial surgeons, orthodontists, and stomatology experts with a common goal of function, esthetic, and quality of life improvements.

Osteolipoma is an extremely rare histologic variant of lipoma that contains mature lamellar bone. We report a case of a 27-year-old man with history of a small, rounded lesion in the right cheek that started in childhood, without symptomatology, but with continuous growing. On the examination, facial asymmetry was observed, without lymph node enlargement. A firm nodule of about 2.5 cm was seen involving his right buccal mucosa. A panoramic radiograph showed radiopaque irregular shapes in the region of the first upper and lower right molars. The lesion was excised, and pathologic examination of the specimen showed fragments of trabecular bone between adipocytes. Definitive pathologic diagnosis was osteolipoma. There was no recurrence at 7 months’ follow-up. Although osteolipoma is very rare, it is important to keep it in the differential diagnosis of nodules with a firm consistency in the oral mucosa and when noting a radiopaque image on panoramic exam.

An 81-year-old white female, with no history of smoking or drinking, reported persistent pain and increase in lesion size in the last 3 months. Intraoral exam showed an exophytic nodular and ulcerated lesion in the region of left labial commissure, 1.5 cm in size and of soft consistency; next to the lesion, there was an extensive white plaque. A white plaque with striated appearance was also observed on the right buccal mucosa. An incisional biopsy was performed in the nodular area with the histopathologic diagnosis of squamous cell carcinoma. The patient was referred to the head and neck service for treatment. This carcinoma case may have been preceded by oral lichenoid lesions and proliferative verrucous leukoplakia and these lesions should be detected during clinical exam and followed closely, as they carry a higher risk for malignant transformation and carcinoma arising from them and may show better prognosis if detected early.

White sponge nevus (WSN) is a rare autosomal dominant disorder with variable expressivity and high penetrance. Oral mucosa is the most affected region. Usually, this entity occurs at birth or later in childhood or adolescence. A 42-year-old man complained of white, asymptomatic lesions, which were present for more than 3 years. No family history of similar lesions was reported. Intraoral examination revealed nonremovable, symmetric, diffuse white plaques, all over the buccal mucosa, bilaterally, with a corrugated surface. An incisional biopsy was performed and histopathologic analysis revealed stratified squamous epithelium, with hyperparakeratosis, an extensive area with acanthosis and keratotinocytes with perinuclear eosinophilic condensation. Based on clinical and microscopic aspects, the diagnosis made was WSN. Further material was collected for cytopathologic examination in order to confirm the diagnosis. Treatment is not necessary due to the benign nature of WSN. Correct diagnosis is important to exclude other more serious lesions with similar characteristics.

Background A solitary fibrous tumor (SFT) of the oral cavity is an extremely rare entity. Its diagnosis is complicated because of its diverse morphology and similarity to other mesenchymal diseases. Case Report A rare case of SFT involving floor of the mouth is presented. The tumor was well circumscribed and almost spherical, measuring approximately 3 cm in diameter. Patient was submitted to biopsy and histopathologic examination showed a tumor composed of spindle to epithelioid cells showing pale to eosinophilic cytoplasm, oval or elongated nuclei with inconspicuous nucleoli. Tumor cells showed strong positivity for CD34, vimentin and Bcl-2. SFTs may present as a diagnostic challenge. The patient was followed for 8 years without recurrences. Results SFT is an uncommon disease in maxillofacial region; however it should be considered in the differential diagnosis of spindle cell neoplasms with oral manifestation. Only a few cases have been reported in the floor of mouth. We describe a new case of SFT arising at this location. Key words:Solitary fibrous tumors, mouth floor, oral diagnosis.

Background/Aim: The TP53 gene is a tumor suppressor gene. Its product is a nuclear protein that regulates cell cycle arrest, apoptosis and DNA repair. Anti-p53 clones DO-7 and PAb-240 recognize the amino acid sequences 21-25 and 213-217, respectively. This study aimed to evaluate the expression of these clones and their relationship with clinicopathological features and survival analysis in oral squamous cell carcinomas (OSCC). Methods: Information on 53 primary OSCC was collected at the Brazilian National Cancer Institute. An immunohistochemical method was applied to evaluate p53 expression (DO-7 and PAb-240). Their expression was analyzed quantitatively and correlated with clinicopathological features. Kaplan-Meier survival curves and log rank test were used. Results: Immunopositivity for DO-7 was present in 64% of the cases, while 58% were positive for PAb-240. There was no correlation between immunoexpression of both antibodies and clinicopathological features or survival analysis. DO-7 expression was significantly higher (p = 0.001) than that of PAb-240. Conclusions: There were quantitative differences between the expression of the antibodies studied, which may reflect a different specificity of each one. To confirm immunohistochemical results and estimate the true prognostic role of TP53 in OSCC, it is important to perform mutation analysis.

Objective This study aimed to evaluate apoptosis by assessing cleaved caspase-3 immunoexpression in hyperplastic, potentially malignant disorder (PMD), and malignant tumors in intraoral and lower lip sites. Material and Methods A retrospective study using paraffin blocks with tissues from patients with inflammatory fibrous hyperplasia (IFH), actinic cheilitis, oral leukoplakia, lower lip and intraoral squamous cell carcinoma (SCC) was performed. The tissues were evaluated by immunohistochemical analysis with anti-cleaved caspase-3 antibody. Apoptotic area index was then correlated with lesion type. Results From 120 lesions assessed, 55 (46%) were cleaved caspase-3-positive. The SCC samples (n=40) had the highest apoptotic area indices (n=35; 87.5%). Significant differences were detected between SCCs and PMDs (p=0.0003), as well as SCCs and IFHs (p=0.001), regarding caspase-3 immunopositivity. Carcinomas of the lower lip had lower apoptotic area indices than intraoral cancer (p=0.0015). Conclusions Cleaved caspase-3 immunoexpression showed differences in oral SCCs and PMDs and demonstrated a distinct role of apoptosis in carcinogenesis of intraoral and lower lip cancer. In future, the expression of cleaved caspase-3 with other target molecules in oral cancer may be helpful in delineating the prognosis and treatment of these tumors.

Aim: To compare the clinicopathological profile of oral squamous cell carcinoma (OSCC) in groups with and without recurrence. Methods: Records of all patients who underwent surgery for primary OSCC at a single institution during 1999 were identified. Patient demographics, lesion site, clinical and pathologic stage, pathologic grading, pattern of invasion, lymphocytic infiltrate, perineural invasion, and treatment and survival data were collected. Descriptive statistics were calculated for each variable and survival was calculated using Kaplan-Meier and Cox models. Patients were divided into 2 groups: with (n = 25) and without (n = 28) recurrence. Results: Tongue (p = 0.02) and poorly differentiated (p = 0.04) tumors were associated with recurrence. Kaplan-Meier and Cox models revealed tobacco use and the absence of lymphocytic infiltrate to be associated with the poorest survival in recurrent OSCC. Conclusion: The tumor site, tobacco use, and pathological features were involved in the recurrence of OSCC and should be taken into account for OSCC treatment and follow-up.