João Paulo Silva Servato

Odontogenic tumours in children and adolescents: a collaborative study of 431 cases

This study describes the oral and maxillofacial pathological characteristics of a series of odontogenic tumours in children and adolescents from three Brazilian reference centres. The records were reviewed for all odontogenic tumours in patients up to 18 years old based on criteria proposed by the World Health Organization (WHO) in 2005. Data concerning sex, age, skin colour and tumour location were collected and plotted. Four hundred and thirty one odontogenic tumours in children and adolescents were found, accounting for 37.5% of the total number of odontogenic tumours diagnosed. Benign tumours were predominant (99.8% of the cases), and odontoma was the most frequent type (41.4%), followed by keratocystic odontogenic tumours (25.5%) and ameloblastoma (14.6%). Odontogenic tumours were rarely detected in early childhood, and their prevalence increased with age. An almost equal distribution was observed with respect to sex and the site of the lesions. This study is the largest reported retrospective analysis describing odontogenic tumours in children and adolescents to date. The authors detected some variation in the relative frequency of odontogenic tumours compared with similar reports. Additional studies should be conducted based on the new WHO classification and predetermined age parameters to enable comparative analysis among different worldwide populations.

Small cell carcinoma of the salivary gland: a systematic literature review and two case reports.

Salivary gland small cell carcinoma is a rare neoplasm, accounting for less than 1% of salivary gland tumours. Little is known about the epidemiologic factors and treatment of this lesion. The authors report two cases and perform a systematic literature search from 1960 to 2011 for articles on salivary gland small cell carcinoma. Once the papers were reviewed, a database was generated to analyse clinical and pathological features, treatments and outcomes, and an attempt was made to identify prognostic factors. Available data were retrieved for 44 cases which fully satisfied the inclusion criteria; the median age was 64.25 years and the male:female ratio was 2.4:1. The parotid gland was the most common site (79.6%). The overall 1-, 2- and 5-year survival rates were 75.3%, 56.4% and 36.6%, respectively. According to the present review, patient age, tumour size, distant metastasis and cytokeratin-20 positive immunostaining were all significant prognostic factors in a univariate analysis. No particular treatment approach appeared to improve survival. This work reinforces knowledge about salivary gland small cell carcinomas epidemiologic features, and identifies new prognostic markers. The optimal management of this lesion remains controversial.

Metastatic tumours to the head and neck: retrospective analysis from a Brazilian tertiary referral centre

Data regarding the development of head and neck metastases are limited, with the majority of the studies focusing on single cases affecting mainly the oral cavity and surrounding tissues. This study describes the incidence of head and neck metastases from primary tumours originating elsewhere. The medical records of selected patients were reviewed, and socio-demographic data as well as information about the localization of the primary tumours and their metastases to this region, treatment modalities employed, follow-up, and outcomes were collected. A total of 24 cases were found. The mean age at diagnosis was 52.1±17.6 years and the male to female ratio was 1:1.4. Most primary tumours were located in the lung and breast. The overall 1-, 2-, and 5-year survival rates were 63.0%, 39.1%, and 19.6%, respectively. Radiotherapy for the metastatic focus significantly prolonged the survival time of the patients (P=0.048). Cervical lymph node metastases are a common event in patients with primary tumours growing elsewhere.

Ameloblastic carcinoma: a Brazilian collaborative study of 17 cases

Ameloblastic carcinoma (AMECA) is an odontogenic malignancy that combines the histological features of ameloblastoma and cytological atypia. Because of its rarity, it poses difficulties in diagnosis. The aim of this study was to investigate the socio‐demographic data, histopathology, immunohistochemical features, treatment and outcomes of 17 cases.

Involvement of oral tissues by AL amyloidosis: a literature review and report of eight new cases.

ObjectiveAmyloidosis is a term used to describe a group of diseases in which there is an extracellular deposition of amorphous fibrillar proteins known as amyloid. The aim of this study was to present clinicopathological data from eight oral amyloidosis-affected patients and a deep review of the literature about the disease.Materials and methodsA retrospective study was conducted based on the records of oral amyloidosis-affected patients diagnosed in our institution between 1978 and 2012. The clinicopathological features and immunohistochemical (IHC) staining with anti-kappa and anti-lambda light chain antibodies were carried out and analyzed.ResultsEight patients were diagnosed with the disease; the tongue and women in their sixth decade of life were mostly affected. All lesions demonstrated apple-green birefringence and immunoreactivity for kappa-light chain, and four cases also showed lambda positivity. According to our series, four cases were diagnosed with localized amyloidosis and four with systemic amyloidosis. Prognosis for the systemic ones was gloomy, but good for the localized ones, which was characterized by a slow pattern of deposition without evolution to systemic involvement.ConclusionsThis study reinforces our knowledge about predilections, outcomes, and the importance of making a correct and quick diagnosis of oral amyloidosis and shows the necessity of more studies detailing oral amyloidosis predilection on a global scale. The importance and utility of IHC in the typing of the biochemical nature of amyloid deposits are becoming increasingly necessary for proper management of the patient. Correct classification of the type of amyloid is important for treatment consequences.Clinical relevanceThis article highlights the clinicopathological data of patients with amyloidosis affecting oral tissues and compare these new findings with other worldwide descriptions. Because of its rarity, such data are often unfamiliar to most clinicians and pathologists.

Adenoid ameloblastoma: clinicopathologic description of five cases and systematic review of the current knowledge

OBJECTIVE To describe the clinicopathologic and immunohistochemical features of five cases of adenoid ameloblastoma. STUDY DESIGN Clinicopathologic data were gathered from medical records and compared with those compiled from a systematic review. Slides were also immunohistochemically stained for Ki-67, p16, p53, and cytokeratins (7, 8, 14, 18, and 19). RESULTS There were 3 males (60%) and 2 (40%) females. The mean age was 44 ± 10 years. Of the five adenoid ameloblastomas, 4 (80%) occurred in the posterior maxilla. Patients typically complained of asymptomatic swelling. All patients received surgical resection as primary therapy; 1 (20%) patient also received adjuvant radiotherapy. Recurrence was diagnosed in all patients. Immunohistochemically, the tumors stained focally positive for CK7, 8, 14, and 18 and diffusely positive for CK-19, p16, and p53. The mean Ki-67-positive cells were 72.4 ± 24.9 positive cells per high-power field (range 53-111). CONCLUSIONS To our knowledge, this is the largest series of adenoid ameloblastoma reported in the literature. Our data suggest that this entity demonstrates aggressive behavior characterized by a high likelihood of recurrence.

Trypanosoma cruzi P21: a potential novel target for chagasic cardiomyopathy therapy.

Chagas disease, which is caused by the parasite Trypanosoma cruzi, is an important cause of cardiomyopathy in Latin America. It is estimated that 10%–30% of all infected individuals will acquire chronic chagasic cardiomyopathy (CCC). The etiology of CCC is multifactorial and involves parasite genotype, host genetic polymorphisms, immune response, signaling pathways and autoimmune progression. Herein we verified the impact of the recombinant form of P21 (rP21), a secreted T. cruzi protein involved in host cell invasion, on progression of inflammatory process in a polyester sponge-induced inflammation model. Results indicated that rP21 can recruit immune cells induce myeloperoxidase and IL-4 production and decrease blood vessels formation compared to controls in vitro and in vivo. In conclusion, T. cruzi P21 may be a potential target for the development of P21 antagonist compounds to treat chagasic cardiomyopathy.

Giant complex odontoma of the anterior mandible: report of case with long follow up

This paper describes an exceptional case of an enormous complex odontoma affecting the mandibular symphysis of a 9-year-old boy. Because of its dimensions, the lesion produced cortical bone expansion, dental displacement and impactation, which are clinical signs very seldom described for odontomas. The lesion was surgically excised in a conservative way using an intraoral approach with local anesthesia. After 7 years of follow up, all teeth had erupted and the mandibular bone healed totally. Because of its radiographic mixed radiolucent and radiopaque appearance and its expansive growth, it is imperative to make the differential diagnosis of giant complex odontoma for other more aggressive mixed odontogenic tumors, such as ameloblastic fibro-odontoma, odotoameloblastoma and cystic calcified odontogenic tumor. Conservative approach appears to be indicated in the treatment of such lesions.

Ameloblastic Fibrosarcoma: A Case Report and Literature Review

Here is described a case of ameloblastic fibrosarcoma (AFS) affecting the posterior mandible of a woman who was treated surgically and recovered without signs of recurrence or metastasis after 12 years of follow-up. Tumor sections were immunostained for cell cycle, epithelial and mesenchymal markers. Immunohistochemical analysis evidenced high Ki-67 positivity in stromal cells (mean of 20.9 cells/High power field). Epithelial cells displayed strong positivity for p53, p63 and cytokeratin 19. In addition to the case report, a systematic review of current knowledge is presented on the AFSs clinical-demographic features and prognostic factors. Based on the review, 88/99 cases were diagnosed as AFS, 9/99 as ameloblastic fibro-odontosarcoma and 2/99 as ameloblastic fibrodentinosarcoma. All these lesions displayed very similar clinical-demographic and prognostic features. Moreover, the review provided evidence that first treatment, regional metastasis, distant metastasis and local recurrence were significant prognostic values for malignant odontogenic mesenchymal lesions. Based on the findings, segregation among ameloblastic fibrosarcoma, ameloblastic fibrodentinosarcoma and ameloblastic fibro-odontosarcoma seems illogical, considering all these lesions have similar predilections and outcomes.

Primary amelanotic mucosal melanoma of the oronasal region: report of two new cases and literature review

Primary amelanotic mucosal melanoma is a rare entity with challenging histopathological features. Because these tumors are thought to be biologically more aggressive, they have a poorer prognosis than that of pigmented melanomas. In this work, we present a literature review about the clinical, histopathological, and immunohistochemical features of primary amelanotic mucosal melanoma of the oronasal region and report two new cases. Amelanotic mucosal melanoma commonly affects men in the seventh decade of life and tend to have a poor prognosis, as seen by the high incidence of metastasis, recurrences, and, ultimately, death. There is a similar pattern in the clinic-pathological predilections (such as age, gender, primary site, and metastatic potential) of amelanotic mucosal melanoma when comparing with data reported for pigmented lesions. This work reinforces knowledge about amelanotic mucosal melanomas and epidemiologic predilections. The optimal management of this lesion remains controversial.