Multiple oral sinus tract and trismus leading to a surprising diagnosis of osteopetrosis: Case report.

AIMS: We report a rare case of late diagnosis of malignant osteopetrosis in a 36-year-old male patient due to multiple intraoral sinus tracts and trismus. CASE REPORT: The patient reported a history of facial scars that could not be attributed to the older external fistulas that were present and various complicated dental extractions since infancy. In addition, the patient had not been previously diagnosed with any other significant diseases other than blindness since infancy. Computed tomography revealed a marble-like sclerotic pattern of all cranial bones, a thickened parietal bone, and a narrowing of the encephalic space and the optic canal. Further laboratory and imaging studies revealed complete sclerosed bone of the chest and pelvis, anemia, reticulocitosis, extramedular hematopoiesis, altered dehydrogenasis lactate, and acid phosphatasis. An interdisciplinary treatment was initiated with medical and dental care monitoring. The patient is still receiving attention after 4 years of follow-up. CONCLUSIONS: The outcome of this case represents the daily challenges faced by interdisciplinary care providers and reveals pearls and pitfalls that can serve as a reference for professional practice in such cases.

Lymphomas Affecting the Sublingual Glands: A Clinicopathological Study.

BACKGROUND: Lymphomas affecting the sublingual glands are extremely rare and very few case reports are currently available. Therefore, the aim of the current study is to describe the clinicopathological features of a series of lymphomas involving the sublingual glands. METHODS: Cases diagnosed in four pathology services were assessed and the formalin-fixed paraffin-embedded tissue blocks were retrieved for diagnosis confirmation. Clinical data were obtained from patients' medical files. RESULTS: We obtained seven cases of lymphomas in the sublingual glands, representing two follicular lymphomas, two diffuse large B cell lymphomas not otherwise specified (DLBCL NOS), two extranodal marginal zone B cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphomas) and one mantle cell lymphoma (MCL). In all cases the tumor cells infiltrated the glandular parenchyma, although in two of them the neoplastic cells were located more superficially and permeated the glandular acini and ducts. Clinically, the tumors presented as asymptomatic nodules and two patients (affected by DLBCL NOS and MCL) died, while the other five patients remained alive at last follow-up. CONCLUSION: Lymphomas affecting the sublingual glands are usually of the mature B cell lineage, often represent low-grade subtypes and may clinically resemble other more common lesions in the floor of the mouth like salivary gland tumors.

Mantle cell lymphoma involving the oral and maxillofacial region: a study of 20 cases.

OBJECTIVE: To investigate the clinicopathologic features of mantle cell lymphoma (MCL) involving the oral and maxillofacial region. METHODS: The MCL cases were retrieved from the pathosis database of 6 pathology laboratories. Original hematoxylin and eosin slides and immunohistochemical reactions were reviewed for confirmation of the initial diagnosis. Clinical data of the cases were obtained from the patients' pathosis and/or medical charts. RESULTS: Twenty cases were included in the study, showing a male predominance and a mean age of 66 years. The oral cavity (12 cases) and the oropharynx (5 cases) were the most commonly involved subsites. Most cases presented as asymptomatic swellings, with 2 cases showing bilateral involvement of the palate. The classic histologic variant predominated (12/20 cases). All cases expressed CD20 with nuclear cyclin D1 positivity. SOX11 was seen in 9/13 cases, CD5 in 6/16 cases, Bcl2 in 16/19 cases, CD10 in 2/20 cases, and Bcl6 in 4/16 cases. Ki67 showed a mean proliferation index of 40.6%. The Epstein-Barr virus (EBV) was negative in all cases investigated. Follow-up data was available for 7 patients, with 5 currently alive and 2 deceased. CONCLUSION: Mantle cell lymphoma, albeit rare, may manifest in the oral and maxillofacial region. Its histologic heterogeneity demands a high degree of diagnostic skill from pathologists.

Rhabdomyosarcoma with TFCP2 Rearrangement or Typical Co-expression of AE1/AE3 and ALK: Report of Three New Cases in the Head and Neck Region and Literature Review.

BACKGROUND: Rhabdomyosarcoma (RMS) harboring EWSR1/FUS-TFCP2 fusions has been recently described as a distinct form of RMS with an aggressive course and predilection for the craniofacial bones, especially the jaws. METHODS: We report three new cases of this rare entity, two from Brazil and one from Guatemala, with detailed clinicopathologic, immunohistochemical, and molecular descriptions. Additionally, we explored the English-language literature searching RMS with TFCP2 rearrangement or typical immunophenotype with co-expression of AE1/AE3 and ALK in the head and neck region. RESULTS: Case 1 is a 58-year-old male with a 3-month history of painful swelling in the anterior maxilla. Case 2 is a 22-year-old male presenting with right facial swelling and proptosis. Case 3 is a 43-year-old female with a rapidly growing tumor located in the zygomatic region. Imaging examinations revealed highly destructive intraosseous masses in the first two cases, and a soft tissue tumor with bone invasion in case 3. Microscopically, all cases showed a hybrid spindle and epithelioid phenotype of tumor cells which expressed desmin, myogenin and/or Myo-D1, AE1/AE3, and ALK. FISH confirmed molecular alterations related to TFCP2 rearrangement in Cases 1-2. In case 3, there was no available material for molecular analysis. The patients were subsequently referred to oncologic treatment. Additionally, we summarized the clinicopathologic, immunohistochemical, and molecular features of 27 cases of this rare RMS variant in the head and neck region reported in the English-language literature. CONCLUSION: RMS with TFCP2 rearrangement is a rare and aggressive tumor with a particular predilection for craniofacial bones, especially the jaws. Knowing its clinicopathologic and immunohistochemical profile can avoid misdiagnosis.

Florid cemento-osseous dysplasia-related osteonecrosis: A series of cases.

OBJECTIVE: The aim of this study was to assess the clinicopathological features of florid cemento-osseous dysplasia (FCOD)-related osteonecrosis highlighting their histopathological aspects and bone structure. METHODS: Twenty-two FCOD-related osteonecrosis cases were evaluated retrospectively. Osteonecrosis, osteomyelitis, bacterial colonization, bone resorption, reactive bone, osteon-like structure, lamellar bone, and basophilic lines were analyzed. Specific staining and fluorescence and polarized light microscopy analyses were also performed. RESULTS: The mandible was more affected by FCOD-related osteonecrosis. There was a predominance of African-Brazilian women in the fifth and seventh decades of life. Osteomyelitis was present in 82 % of cases whereas bone resorption and bacterial colonization were present in 100 % of FCOD-related osteonecrosis cases. Thick basophilic lines were seen in all cases (100 %). Actinomycosis and osteoclasts were not often. CONCLUSIONS: This study showed female adult preference, mandibular location, and some findings such as osteomyelitis, bone resorption, and bacterial colonization were histopathological features more frequent in FCOD-related osteonecrosis. In the absence of a close clinical and radiographic correlation, the morphology of the necrotized bone similar to cementum could help to recognize FCOD.

Molecular profile of head and neck rhabdomyosarcomas: A systematic review and meta-analysis.

OBJECTIVE: This systematic review aimed to identify the molecular alterations of head and neck rhabdomyosarcomas (HNRMS) and their prognostic values. STUDY DESIGN: An electronic search was performed using PubMed, Embase, Scopus, and Web of Science with a designed search strategy. Inclusion criteria comprised cases of primary HNRMS with an established histopathological diagnosis and molecular analysis. Forty-nine studies were included and were appraised for methodological quality using the Joanna Briggs Institute Critical Appraisal tools. Five studies were selected for meta-analysis. RESULTS: HNRMS predominantly affects pediatric patients (44.4%), and the parameningeal region (57.7%) is the most common location. The alveolar variant (43.2%) predominates over the embryonal and spindle cell/sclerosing types, followed by the epithelioid and pleomorphic variants. PAX-FOXO1 fusion was observed in 103 cases of alveolar RMS (79.8%). MYOD1 mutation was found in 39 cases of sclerosing/spindle cell RMS (53.4%). FUS/EWSR1-TFCP2 gene fusions were identified in 21 cases of RMS with epithelioid and spindle cell morphologies (95.5%). The 5-year overall survival rate of patients was 61.3%, and MYOD1 mutation correlated with significantly higher mortality. CONCLUSION: The genotypic profile of histologic variants of HNRMS is widely variable, and MYOD1 mutation could be a potential prognostic factor, but more studies are required to establish this.

Aplicación Android para la adquisición inalámbrica y visualización de señales biomédicas / Android application for wireless acquisition and visualization of biomedical signals / Aplicativo Android para aquisição sem fio e visualização de sinais biomédicos

RESUMEN Introducción: Los avances tecnológicos experimentados por los teléfonos y tabletas con sistema operativo Android han permitido el desarrollo de innumerables aplicaciones en el área de la medicina. Hasta nuestro conocimiento, en nuestro sistema de salud, no se reporta el uso de un dispositivo portátil que permita al especialista, monitorear a distancia, de forma inalámbrica las señales biomédicas asociadas a un paciente. Objetivo: Desarrollar una aplicación Android (herramienta) que permita adaptarse a múltiples sistemas de monitorización inalámbrica con el fin de capturar, visualizar y almacenar señales biomédicas. Método: Se muestra la arquitectura general del sistema de comunicación inalámbrico que integra a la herramienta y se propone el diseño software de la herramienta y el diagrama de interacción de las cinco actividades que la componen: "Menú", "Pacientes", "Configuración", "Escáner", "Graficar". Resultados: Se mostraron las diferentes pantallas y funcionalidades de la aplicación, para dos dispositivos médicos cubanos (y modos): Sistema de Medición Biomédica para la Exploración Vestibular (Recepción) y Sistema de Monitoreo Electrocardiográfico Inalámbrico para dispositivos Android (opera en modo Transmisión/Recepción). Conclusiones: La aplicación proporciona una interfaz sencilla e intuitiva, lo que facilita la interacción con el usuario. Su evaluación cualitativa mediante pruebas pilotos mostró excelentes resultados en ambos casos.

ABSTRACT Introduction: Technological advances experienced by phones and tablets with Android operating system have enabled the development of countless applications in the field of medicine. As far as we know, there is not reported in our health system the use of a portable device that allows the specialist to wirelessly monitor remotely the biomedical signals associated with the patient. Objective: Development of an Android application (as a tool) that can be adapted to multiple wireless monitoring systems in order to capture, visualize and store biomedical signals. Method: The general architecture of the wireless communication system that integrates the tool is shown and the software design of the tool and the interaction diagram of the five activities that compose it are proposed: "Menu", "Patients", "Configuration", "Scanner", "Graph". Results: Different screens and functionalities of the application were shown, compatibles for two Cuban medical devices (and modes): Biomedical Measurement System for Vestibular Exploration (Reception) and the Wireless Electrocardiographic Monitoring System for Android devices (operates in Transmission/Reception mode). Conclusions: The application provides a simple and intuitive interface, which facilitates interaction with the user. Its qualitative evaluation through rapid tests showed excellent results in both cases.

RESUMO Introdução: Os avanços tecnológicos vivenciados pelos telefones e tablets com sistema operacional Android permitiram o desenvolvimento de inúmeras aplicações na área da Medicina. Até onde sabemos, em nosso sistema de saúde, não foi relatado o uso de um dispositivo portátil que permita ao especialista monitorar remotamente, sem fio, os sinais biomédicos associados a um paciente. Objetivo: Desenvolver um aplicativo Android (ferramenta) que possa ser adaptado a vários sistemas de monitoramento sem fio para capturar, exibir e armazenar sinais biomédicos. Método: Apresenta-se a arquitetura geral do sistema de comunicação sem fio que integra a ferramenta e propõe-se o desenho do software da ferramenta e o diagrama de interação das cinco atividades que a compõem: "Menu", "Pacientes", "Configuração", "Scanner", "Gráfico". Resultados: Foram mostradas as diferentes telas e funcionalidades do aplicativo para dois dispositivos médicos cubanos (e modos): Sistema de Medição Biomédica para Exame Vestibular (Recepção) e Sistema de Monitoramento Eletrocardiográfico Sem Fio para dispositivos Android (opera no modo Transmissão/Transmissão). Conclusões: O aplicativo oferece uma interface simples e intuitiva, o que facilita a interação com o usuário. Sua avaliação qualitativa por meio de testes pilotos apresentou excelentes resultados em ambos os casos.

Corrección del efecto de desplazamiento angular horizontal de la cabeza en pruebas de estimulación visual en MATLAB / Correction of horizontal and angular head displacement effects by MATLAB visual stimulation tests / Correção do efeito do deslocamento angular horizontal da cabeça em testes de estimulação visual no MATLAB

RESUMEN Introducción: Las irregularidades en los movimientos oculares constituyen un indicador importante para diagnosticar determinadas enfermedades neurodegenerativas. La electrooculografía es la técnica más difundida para medir dichos movimientos oculares. Durante la realización de una prueba visual, el paciente puede realizar movimientos de cabeza indeseados que añaden perturbaciones a la señal electrooculográfica, modificando su morfología y, por tanto, alterando determinados parámetros diagnósticos. Objetivo: Desarrollar un método para corregir el efecto del desplazamiento angular horizontal de la cabeza en la señal electrooculográfica. Método: Se detalla un modelo matemático utilizado en la Universidad de Oriente desde marzo de 2021 a diciembre de 2021, para la implementación de la corrección en dos tipos de señales electrooculográficas artificiales con diferentes movimientos de cabeza horizontales. Resultados: Se evalúo cualitativamente el comportamiento del método utilizado a través de su implementación en señales generadas artificialmente en MATLAB. Finalmente se caracterizaron los efectos de la corrección en los parámetros diagnósticos de la señal electrooculográfica. Conclusiones: El método implementado demostró su validez para casos específicos, en el que se logra para dos tipos de señales eliminar los errores introducidos por el desplazamiento de la cabeza. La corrección mejora el error introducido en la amplitud de la señal electrooculográfica sin corregir y mantiene inalterables, a falta de un análisis más profundo, los demás parámetros diagnósticos.

ABSTRACT Introduction: Eye movement disorders are an important indicator for the diagnosis of certain neurodegenerative diseases. Electrooculography is the most widespread technique for measuring such eye movements. During the performance of the eye test, patients may forge unwanted head movements that add disturbances to the electrooculographic signal, modifying its morphological characteristic and, therefore, changing certain diagnostic parameters. Objective: To develop a method for the correction of the effect of the horizontal and angular head displacement by the electrooculographic signal. Method: It is detailed the use of a mathematical model for the correction of two types of artificial electrooculographic signals with different horizontal head movements at the Universidad de Oriente, from March 2021 to December 2021. Results: The behavior of the method used was evaluated qualitatively through its implementation in the signals generated artificially in MATLAB. Finally, the correction effects on the diagnostic parameters of the electrooculographic signal were characterized. Conclusions: The implemented method proved its validity for specific cases, in which it is possible to eliminate the errors caused by head displacement in two types of signals. The correction minimizes the error introduced in the uncorrected electrooculographic signal amplitude and keeps unchanged the other diagnostic parameters in absence of further analyses.

RESUMO Introdução: As irregularidades nos movimentos oculares são um importante indicador para diagnosticar certas doenças neurodegenerativas. A eletrooculografia é a técnica mais difundida para medir esses movimentos oculares. Durante um teste visual, o paciente pode realizar movimentos involuntários da cabeça que adicionam distúrbios ao sinal eletrooculográfico, modificando sua morfologia e, portanto, alterando alguns parâmetros diagnósticos. Objetivo: Desenvolver um método para corrigir o efeito do deslocamento angular horizontal da cabeça no sinal eletrooculográfico. Método: Um modelo matemático usado na Universidade de Oriente de março de 2021 a dezembro de 2021 é detalhado para a implementação da correção em dois tipos de sinais eletrooculográficos artificiais com diferentes movimentos horizontais da cabeça. Resultados: O comportamento do método utilizado foi avaliado qualitativamente através de sua implementação em sinais gerados artificialmente no MATLAB. Por fim, foram caracterizados os efeitos da correção sobre os parâmetros diagnósticos do sinal eletrooculográfico. Conclusões: O método implementado demonstrou sua validade para casos específicos, nos quais é possível eliminar os erros introduzidos pelo deslocamento da cabeça para dois tipos de sinais. A correção melhora o erro introduzido na amplitude do sinal eletrooculográfico não corrigido e mantém os demais parâmetros diagnósticos inalterados, na ausência de uma análise mais profunda.

Clinicoradiological spectrum of primary aneurysmal bone cysts of the maxillofacial region: A series of 31 cases.

OBJECTIVES: The aim of the current study was to analyse the demographic, clinical and radiological features of primary aneurysmal bone cysts (ABCs) involving the maxillofacial region. METHODS: Histologically confirmed cases affecting the maxillofacial region were retrospectively reviewed over a 21-year period (2000-2021). Cases were collected from the archives of five Oral Pathology laboratories from three countries: South Africa, Guatemala and Brazil. The information was analysed, with emphasis on the clinical and radiological spectrum. RESULTS: Following the inclusion criteria, a total of 31 cases of primary ABCs were included in the study. A nearly equal male-to-female distribution was seen, with ABCs occurring in males at an earlier age compared to females. Localised swelling was the main clinical presentation. ABCs had a mandibular predominance, particularly in the posterior regions. All ABCs presented as blow-out expansile well-demarcated radiolucent lesions with the majority having a multilocular appearance. Cortical expansion was seen in 91% of cases with loss of cortical integrity being common (78%). CONCLUSION: Primary ABCs involving the maxillofacial region are extremely rare with the majority of current published literature consisting of isolated case reports. The current study is the first large series detailing the radiological features.

Use of Gamma Radiation for the Genetic Improvement of Underutilized Plant Varieties.

Agricultural biodiversity includes many species that have biological variants (natives, ecotypes, races, morphotypes). Their use is restricted to local areas because they do not fulfill the commercial requirements; however, it is well documented that these species are a source of metabolites, proteins, enzymes, and genes. Rescuing and harnessing them through traditional genetic breeding is time-consuming and expensive. Inducing mutagenesis may be a short-time option for its genetic improvement. A review of outstanding research was carried out, in order to become familiar with gene breeding using gamma radiation and its relevance to obtain outstanding agronomic characteristics for underutilized species. An approach was made to the global panorama of the application of gamma radiation in different conventional crop species and in vitro cultivated species, in order to obtain secondary metabolites, as well as molecular tools used for mutation screening. The varied effects of gamma radiation are essentially the result of the individual responses and phenotypic plasticity of each organism. However, even implicit chance can be reduced with specific genetic breeding, environmental adaptation, or conservation objectives.

Oral and Maxillofacial Neuroendocrine Carcinoma: A Systematic Review.

The aim of the present study was to integrate the available data published in the literature on oral and maxillofacial neuroendocrine carcinomas concerning the demographic, clinical and histopathological features of this condition. An electronic search with no publication date restriction was undertaken in April 2021 in four databases. Eligibility criteria included reports published in English having enough data to confirm a definite diagnosis, always showing a neuroendocrine marker. Cases originating in the oropharynx, including base of the tongue and tonsils, were excluded. Outcomes were evaluated by the Kaplan-Meier method along with Cox regression. Twenty-five articles (29 cases) from nine different countries were detected. Mean patient age was 56.3 (± 17.5) years, with a slight male predilection. Symptomatology was present in 72.2% of informed cases. Regarding clinical presentation, a non-ulcerated nodule located in the gingiva with a mean size of 3.4 (± 2.0) cm was most frequently reported. Concomitant metastasis was identified in seven individuals. Histopathologically, most neoplasms were of the small cell type, and immunohistochemistry for both epithelial and neuroendocrine differentiation was used in 65.5% cases. Radical surgery was the treatment of choice in almost all cases, with or without adjuvant therapy. Mean follow-up was 20.5 (± 21.2) months, and only four patients developed recurrences. Eleven (44.0%) individuals died due to the disease. Ulcerated lesions were a prognostic factor. This study provides knowledge that can assist surgeons, oncologists, and oral and maxillofacial pathologists with the diagnosis and management of neuroendocrine carcinomas. Our findings demonstrated that the long-term prognosis of this lesion continues to be poor.

Oncocytic Lipoadenoma: Report of 3 Rare Cases Involving the Parotid Gland, Including a Synchronous Presentation With Paraganglioma of the Right Carotid Bifurcation and Literature Review.

Oncocytic lipoadenoma (OL) is a rare salivary gland tumor characterized by the presence of oncocytic cells and mature adipose tissue. To date, only 30 cases of OL have been reported in the English-language literature. We present 3 additional OL cases involving the parotid, including a synchronous presentation with paraganglioma of the right carotid bifurcation. Microscopically, both the OLs were composed of a mixed population of oncocytes and adipocytes in varying proportions surrounded by a thin, connective tissue fibrous capsule. Oncocytes were positive for pan-cytokeratins (CKs) AE1/AE3, epithelial membrane antigen, CK5, CK7, CK14, CK18, and CK19. Calponin, p63, alpha-smooth muscle actin, and carcinoembryonic antigen were negative. Vimentin and S-100 protein were positive only in adipose cells. Despite distinctive morphologic features, OL is often misdiagnosed, given its rarity. We hope to contribute to surgeons' and pathologists' awareness and knowledge regarding the existence of this tumor and provide adequate management through conservative surgical excision.

Human papillomavirus co-infection and survival in oral and oropharyngeal squamous cell carcinoma: A study in 235 Brazilian patients.

OBJECTIVES: While unknown for oropharyngeal squamous cell carcinoma (OPSCC) and oral squamous cell carcinoma (OSCC), some studies assessing cervical carcinoma have shown that human papillomavirus (HPV) co-infection can be associated with its prognosis. METHODS: Through in situ hybridization (HPV and Epstein-Barr virus [EBV] probes) and immunohistochemistry (p16INK4a, cyclin D1, p53, and Ki-67 antibodies), 126 OPSCC and 109 OSCC samples were assessed. RESULTS: All patients were EBV-negative. OPSCC (25%) showed a significant association with HPV compared to OSCC (11%). Almost all HPV-associated cases were p16INK4a-positive. Regarding OPSCC and OSCC, 23 and 7 cases were positive for high-risk HPV (HRHPV) only, 6 and 3 cases for low-risk HPV (LRHPV) only, and 3 and 2 cases for HRHPV/LRHPV, respectively. HPV-associated carcinomas showed a significantly higher proliferative index than HPV-unassociated carcinomas. Both carcinomas showed a similar overall survival rate, which was not affected by the HPV status. However, when comparing HPV-associated subgroups, patients with HRHPV/LRHPV-associated carcinomas showed worse survival. CONCLUSION: LRHPV-associated and HRHPV/LRHPV-associated cases can also be detected when assessing OSCC and OPSCC. Further studies, especially in populations with a high prevalence of HPV-associated OPSCC, are necessary to understand the clinicopathological behavior of these neoplasm subgroups.

Focal lymphocytic sialadenitis and ectopic germinal centers in oral reactive lesions and primary Sjögren's syndrome: a comparative study.

Focal lymphocytic sialadenitis (FLS), an important diagnostic criterion for Sjögren's syndrome (SS) diagnosis, can also be observed when assessing minor salivary gland (mSG) biopsies from healthy asymptomatic individuals (non-SS patients). Fifty cases of primary SS (pSS group) and 31 cases of oral reactive lesions (non-SS non-sicca group) containing also typical FLS features, were assessed by morphological and immunohistochemical (CD10, CD23 and Bcl-6) analysis, aiming at the detection of GCs. All pSS cases showed FLS with focus score (FS) ≥ 1. In the non-SS non-sicca group, 12, 10 and 9 cases showed FLS with FS ≥ 1, FLS with FS < 1 and FLS associated with chronic sclerosing sialadenitis with FS < 1, respectively. The morphological analysis revealed similar frequency of GCs in pSS (20%) and non-SS non-sicca group (19%). The area (p = 0.052) and largest diameter (p = 0.245) of GCs were higher in pSS than non-SS non-sicca group. The FS and number of foci were significantly higher in pSS than non-SS non-sicca group with FS < 1. Immunohistochemistry confirmed all morphological findings (GCs showing CD23 and Bcl-6 positivity, with variable CD10 expression) and additionally in 3 and 1 cases of the pSS and non-SS non-sicca group, respectively. Moreover, another 6 and 2 cases of the pSS and non-SS non-sicca group with FS ≥ 1, respectively, showed positivity only for CD23. FLS can also be observed when assessing oral reactive lesions, which showed similar frequency of GCs with those found in pSS patients. Further studies, including functional analysis of lymphocytic populations and GCs in FLS, are encouraged.

Plasmacytoma and plasma cell myeloma affecting the jaws: A multi-institutional collaborative study.

BACKGROUND: Plasma cell neoplasms are characterized by the proliferation of a single clone of plasma cells with production of a monoclonal immunoglobulin. They can manifest as a single lesion (plasmacytoma) or as multiple lesions (multiple myeloma). METHODS: Paraffin-embedded tissue blocks of patients microscopically diagnosed with plasma cell neoplasms in the jaws were retrieved from five pathology files. Data including clinical, radiographic, microscopic and immunohistochemical findings, treatment employed and follow-up status were retrieved from the pathology reports. RESULTS: Fifty-two cases were retrieved (mean age: 59.4 years) without sex predilection. The mandible was the most affected site (67.3%), usually associated with pain and/or paresthesia (53.8%). Lesions in other bones besides the jaws were reported for 24 patients (46.2%). Radiographically, tumours usually presented as poorly defined osteolytic lesions with unilocular or multilocular images, while microscopy revealed diffuse proliferation of neoplastic plasma cells with nuclear displacement and abundant eosinophilic cytoplasm. Two cases were classified as anaplastic, and amyloid deposits were found in two other cases. Immunohistochemistry was positive for plasma cell markers and negative for CD20 and CD3, and monoclonality for kappa light chain predominated. The overall survival rate after 5 years of follow-up was 26.6%. CONCLUSION: Plasma cell neoplasms are aggressive tumours with a poor prognosis and involvement of the jaws may be the first complaint of the patient. Thus, oral pathologists, head and neck surgeons and dentists should be aware of their clinical, radiographic and microscopic manifestations.

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.

Burkitt lymphoma of the head and neck: An international collaborative study.

BACKGROUND: Burkitt lymphoma (BL) is an aggressive B-cell lymphoma with three variants (endemic, sporadic, and immunodeficiency-associated), presenting with specific epidemiological and clinical features. Burkitt lymphoma affects the head and neck region (BLHN) in approximately 10% of cases. The aim of this study was to undertake a comparative analysis of the clinicopathologic and immunohistochemical (IHC) features of BLHN diagnosed in patients from Africa, Guatemala, and Brazil. METHODS: Cases diagnosed as BLHN were collected from the files of six oral pathology laboratory services (Brazil, South Africa, and Guatemala) and one Brazilian pediatric oncology hospital from 1986 to 2020. Clinicopathological and IHC data, and Epstein-Barr virus (EBV) status by in situ hybridization data for each case were reviewed and described. RESULTS: Of the 52 cases, BLHN was predominant in pediatric patients [43 (82.69%)] and males [43 (82.69%)], with a mean age of 11.26 ± 9.68 years (range, 1-39 years). Neck and cervical lymph nodes [14 (26.92%)], and involvement of both maxilla and mandible [8 (15.38%)], were the most common anatomical sites. Clinically, tumor/swelling [40 (31.25%)], cervical lymphadenopathy [14 (10.94%)], pain [12 (9.38%)], and bone destruction [12 (9.38%)] were frequent findings. All cases showed typical morphological characteristics of BL. IHC profiles included positivity for CD20 [52 (100%)], CD10 [38 (79.17%)], Bcl6 [29 (87.88%)], and c-Myc protein [18 (81.82%)]. EBV was positive in 18 cases (62.07%). The Ki-67 index ranged from 90 to 100%. CONCLUSION: The clinicopathological and EBV profile of BLHN in South African, Guatemalan, and Brazilian patients is similar.

Proliferative Verrucous Leukoplakia: An Expert Consensus Guideline for Standardized Assessment and Reporting.

The many diverse terms used to describe the wide spectrum of changes seen in proliferative verrucous leukoplakia (PVL) have resulted in disparate clinical management. The objective of this study was to produce an expert consensus guideline for standardized assessment and reporting by pathologists diagnosing PVL related lesions. 299 biopsies from 84 PVL patients from six institutions were selected from patients who had multifocal oral leukoplakic lesions identified over several years (a minimum follow-up period of 36 months). The lesions demonstrated the spectrum of histologic features described in PVL, and in some cases, patients developed oral cavity squamous cell carcinoma (SCC). An expert working group of oral and maxillofacial and head and neck pathologists reviewed microscopic features in a rigorous fashion, in combination with review of clinical photographs when available. The working group then selected 43 single slide biopsy cases for whole slide digital imaging (WSI) review by members of the consensus conference. The digital images were then reviewed in two surveys separated by a washout period of at least 90 days. Five non-PVL histologic mimics were included as controls. Cases were re-evaluated during a consensus conference with 19 members reporting on the cases. The best inter-observer diagnostic agreement relative to PVL lesions were classified as "corrugated ortho(para)hyperkeratotic lesion, not reactive" and "SCC" (chi-square p = 0.015). There was less than moderate agreement (kappa < 0.60) for lesions in the "Bulky hyperkeratotic epithelial proliferation, not reactive" category. There was ≥ moderate agreement (> 0.41 kappa) for 35 of 48 cases. This expert consensus guideline has been developed with support and endorsement from the leadership of the American Academy of Oral and Maxillofacial Pathology and the North American Society of Head and Neck Pathologists to recommend the use of standardized histopathologic criteria and descriptive terminology to indicate three categories of lesions within PVL: (1) "corrugated ortho(para)hyperkeratotic lesion, not reactive;" (2) "bulky hyperkeratotic epithelial proliferation, not reactive;" and (3) "suspicious for," or "squamous cell carcinoma." Classification of PVL lesions based on a combination of clinical findings and these histologic descriptive categories is encouraged in order to standardize reporting, aid in future research and potentially guide clinical management.

Extranodal NK/T cell lymphoma, nasal type: An updated overview.

Extranodal NK/T-cell lymphoma, nasal type (ENKTCL-NT) is an aggressive malignancy associated with Epstein-Barr virus infection, with a geographic and racial predilection for some Asian and Latin American countries. ENKTCL-NT manifests as a necrotic process affecting nasal or upper aerodigestive structures and, rarely, extranasal sites such as skin, and the gastrointestinal tract. ENKTCL-NT was characterized by its poor prognosis irrespective of clinical stage and therapy. However, during the last two decades, advances in its clinicopathologic, genetic and molecular characterization have been achieved, as have changes in the chemotherapy regimens that, in combination with radiotherapy, are significantly improving the survival of these patients, especially in initial stages. For these reasons, we present an overview of the historical background of ENKTCL-NT along with an updated review of its potential etiological factors, clinicopathologic and molecular features, as well as its prognostic models, current treatment protocols, and future directions on potential promising therapeutic approaches.