Oral Juvenile Xanthogranuloma: A Clinicopathological, Immunohistochemical and BRAF V600E Study of Five New Cases, with Literature Review.

Juvenile xanthogranuloma (JXG) is the most common form of non-Langerhans cell histiocytosis and oral mucosal involvement is exceedingly rare. Histiocytic disorders harbor activating mutations in MAPK pathway, including the report of BRAF V600E in JXG of extracutaneous site. However, no information is available for oral JXG. Herein, the clinicopathological and immunohistochemical features of five new oral JXG were evaluated in conjunction with literature review. Also, we assessed the BRAF V600E in oral samples. Five oral JXG were retrieved from pathology archives. Morphological and immunohistochemical analyses were performed. The BRAF V600E status was determined with TaqMan allele-specific qPCR. The series comprised of three female and two male patients, most of them adults, with a median age of 39 years (range 13-68 years). Clinically, the lesions appeared as asymptomatic solitary nodules, measuring until 2.5 cm, with more incident to the buccal mucosa. Morphologically, most of the cases presented classical histological features of JXG, with histiocytic cells consistent with the non-Langerhans cell immunophenotype. BRAF V600E was not detected in the cases tested. This is the first and largest published series of oral JXG affecting adults and a Brazilian population. The molecular pathogenesis of oral JXG remains unknown. Clinicians and pathologists must recognize JXG to avoid misdiagnoses with oral benign or malignant lesions.

Acquired oral syphilis: A multicenter study of 339 patients from South America.

OBJECTIVE: To report the clinicopathologic features of acquired oral syphilis cases in South American countries. MATERIALS AND METHODS: Clinical data were retrospectively collected from the records of 18 oral diagnostic services in Argentina, Brazil, Chile, Colombia, Venezuela, Uruguay, and Peru. Serologies of nontreponemal and treponemal tests were used for diagnosis. RESULTS: The series comprised 339 cases of acquired oral syphilis. Secondary syphilis ranked as the most common stage (86.7%). Lesions were more frequent among males (58.0%) and young adults with a mean age of 33.3 years. Individuals aged 20-29 years were most affected (35.3%). The most commonly involved sites were the tongue (31.6%), lip/labial commissure (25.1%), and hard/soft palate (20.4%). Clinically, acquired oral syphilis usually presented as mucous patches (28.4%), papules (25.7%), and ulcers (18.1%). Skin manifestations occurred in 67.7% of individuals, while lymphadenopathy and fever were observed in 61.3% and 11.6% of all subjects, respectively. Most patients were treated with the benzathine penicillin G antibiotic. CONCLUSION: This report validates the spread of acquired oral syphilis infection among young adults in South America. Our directives include accessible diagnostic tools for proper disease screening, surveillance, and counselling of affected individuals, especially in low- and middle-income countries.

Mature T/NK-Cell lymphomas of the oral and maxillofacial region: A multi-institutional collaborative study.

BACKGROUND: The diagnosis of oral and maxillofacial mature T/NK-cell neoplasms is challenging because of their rarity, morphological heterogeneity and complex immunophenotype with scarce available data describing their clinical and microscopic aspects. Therefore, in this study, we investigated a series of mature T/NK-cell neoplasms affecting this anatomical region and provided an updated literature review. METHODS: Cases diagnosed as mature T/NK-cell lymphomas affecting the oral and maxillofacial region were retrospectively retrieved from six pathology files and their diagnoses were confirmed using haematoxylin and eosin-stained slides, immunohistochemical reactions and in situ hybridization for Epstein-Barr virus (EBV) detection. Patients' clinical data were collected from their pathology forms. RESULTS: A total of 22 cases were included in this study. Eleven (50%) consisted of extranodal NK/T-cell lymphomas, nasal type; eight (36.4%) were peripheral T-cell lymphomas, NOS; two (9.1%) were adult T-cell leukaemia/lymphomas, and one (4.5%) was an ALK-positive anaplastic large cell lymphoma. Overall, males predominated, with a mean age of 55.7 years. The palate was the most affected site (50%), and tumours usually presented as destructive and painful ulcers. EBV was present in all cases of extranodal NK/T-cell lymphoma nasal type but was absent in the other subtypes. CONCLUSION: Among mature T/NK-cell lymphomas of the oral and maxillofacial region, extranodal NK/T-cell lymphoma, nasal type and peripheral T-cell lymphoma, NOS predominated. Older men were the most affected patients, and this heterogeneous group of neoplasms has a very aggressive clinical behaviour.

Primary melanoma of the oral cavity: A multi-institutional retrospective analysis in Brazil / Melanoma primario de la cavidad bucal: Un análisis retrospectivo multiinstitucional en Brasil

Background: Melanoma is an aggressive malignant tumor, rarely observed in the oral cavity. The aim of this studywas to describe the clinicopathologic features of a series of oral melanomas.Material and Methods: A retrospective descriptive study was performed. A total of 15,482 biopsy records from twooral and maxillofacial pathology services in Brazil were analyzed. All cases of oral melanomas were reviewed,and clinical, demographic, histopathological data, treatment, and follow-up status were collected. In addition, im-munohistochemistry stains (pan-cytokeratin AE1/AE3, vimentin, α-SMA, CD45, S-100 protein, HMB-45, MelanA, and Ki-67) were performed Results: The series comprised of 5 males (71.4%) and 2 females (28.6%), with a mean age of 58.0 ± 9.2 years (range:45-69 years) and a 2.5:1 male-to-female ratio. The gingiva (n = 3, 42.8%) and hard palate (n = 2, 28.6%) were the mostcommon affected sites, presenting clinically as ulcerated swellings with a brown to black color. Cervical lymph nodemetastasis was detected in three patients during the first examination. Microscopically, 6 cases (85.7%) were mela-notic, and one (14.3%) was amelanotic. Most cases (n = 4, 57.1%) presented a predominance of epithelioid cells. S-100and HMB-45 were positive in all cases (n = 7, 100.0%). In contrast, only 4 cases (57.1%) were positive for Melan-A.The proliferative index with Ki-67 was high, with labeling index ranging from 70.0% to more than 90% of positivecells. Five patients died from complications of the tumors after a mean follow-up period of 7.8 months.Conclusions: Melanoma is an aggressive malignant tumor that rarely occurs in the oral cavity. It occurs mainly inadult and elderly patients and often is diagnosed in advanced stages. The current findings were similar to previousstudies and reflected the characteristics of the services from where lesions were retrieved.(AU)

Avaliação clínica e histopatológica da eficácia do tratamento utilizando terapia fotodinâmica associada ao laser de baixa potência em pacientes portadores de queilite actínica - Um acompanhamento de 6 meses / Clinical and Histopathological Evaluation of Photodynamic Therapy Associated with The Low-Level Laser Therapy on Patients with Actinic Cheilitis - A Six-Month Follow-Up Trial

O objetivo do presente estudo foi avaliar a eficácia clínica e histopatológica da terapia fotodinâmica (TFD) associada à terapia a laser de baixa potência no tratamento de pacientes com queilite actínica (QA) após seis meses de acompanhamento. Material e Métodos: pacientes com QA ativa foram submetidos a duas sessões de TFD associadas à laserterapia de baixa potência, utilizando o 5-aminolevulinato de metila como droga fotossensibilizadora. Após seis meses de acompanhamento, uma nova biópsia incisional foi realizada para avaliar a resposta ao tratamento. Resultados: excelentes taxas de eficácia foram observadas na melhora dos sintomas neste estudo, no entanto, a análise histopatológica não foi tão promissora e apenas a melhora do grau de displasia só pode ser observada em 20% da amostra. Conclusão: de acordo com os resultados do presente estudo, pode-se concluir que a terapia fotodinâmica associada à laserterapia de baixa potência parece ser uma opção a ser considerada no tratamento da Queilite Actínica. No entanto, os pacientes devem ser acompanhados de perto clinicamente e, eventualmente, histopatologicamente

Objective: the aim of the present study was to evaluate the clinical and histopathological efficacy of photodynamic therapy (PDT) associated with the low-level laser therapy (LLLT) in the treatment of patients with Actinic Cheilitis (AC) after a six-month follow-up. Material and Methods: patients with active AC undergone through two sessions of PDT, associated with the low-level laser therapy using the Methyl 5-aminolevulinate as a photosensybilizer drug. After a six-month follow-up, a new incisional biopsy was performed in order to evaluate the response to treatment. Results: excellent efficacy rates were observed in the improvement of symptoms in this study, however, histopathological analysis was not so promising and the improvement of dysplasia degree could only be seen in 20% of the sample. Conclusion: according to the results of the present study, we concluded that the photodynamic therapy associated with low-level laser therapy is an option to be considered when treating Actinic Cheilits. However, patients should be clinically followed-up in a severing agenda and eventually, histopathologically