Lymphomas affecting the submandibular glands / Linfomas que afectan las glándulas submandibulares

Background: Lymphomas affecting the submandibular glands are very uncommon and few reports are currentlyavailable in the literature. Therefore, the aim of the current study is to describe the clinical and microscopic fea-tures of an original series of lymphomas affecting the submandibular glands.Material and Methods: The pathology files of two institutions were searched for lymphoma cases affecting thesubmandibular glands. The original hematoxylin and eosin, and immunohistochemical slides were revised by apathologist for diagnosis confirmation following the revised 4th edition of the World Health Organization classification of tumours of haematopoietic and lymphoid tissues. Clinical data regarding age, sex, clinical manifestation,treatment, follow-up and status at last appointment were retrieved from the patients’ medical charts.Results: During the period investigated, 16 cases were included in the study. Females predominated (10:6) with amean age of 57.8 years-old. Tumors usually presented as asymptomatic swellings. MALT lymphoma representedthe most common subtype, followed by diffuse large B cell lymphoma and follicular lymphoma. Three patientsdied, one of them affected by plasmablastic lymphoma, one by DLBCL and one by MALT lymphoma.Conclusions: Low-grade B cell lymphomas predominate in the submandibular glands, but DLBCL and other sub-types may also be rarely diagnosed in this salivary gland.(AU)

Leiomiomas e leiomiosarcomas da região oral e maxilofacial: um estudo clinicopatológico e imunoistoquímico de uma série de casos / Leiomyomas and leiomyosarcomas of the oral and maxillofacial region: a clinicopathological and immunohistochemical study of 27 cases

Os tumores de músculo liso são considerados raros na cavidade oral, com a frequência de leiomioma (LM) e leiomiossarcoma (LMS) oral sendo menor que 1% do total de neoplasias nesta região. Os LMs são caracterizados clinicamente como um nódulo de crescimento lento e assintomático, sendo classificados como angioleiomioma (ALM) e LM sólido. Histologicamente, os LM sólidos apresentam-se como uma proliferação de células fusiformes com citoplasma eosinofílico, formando feixes ou fascículos entrelaçados, enquanto que os ALM são compostos por feixes de células musculares lisas bem diferenciadas em torno de vasos sanguíneos de tamanho variável. A remoção cirúrgica conservadora é o tratamento preconizado e as recorrências são extremamente raras. Já o LMS apresenta-se clinicamente como uma lesão tumoral que exibe um crescimento rápido e localmente destrutivo, exibindo histologicamente um crescimento fascicular de células neoplásicas fusiforme, com figuras de mitose, atipia celular e necrose tecidual. O tratamento mais usado é a excisão cirúrgica radical, frequentemente exibindo recidivas locais e metástases a distância. Devido à longa lista de diagnósticos diferenciais microscópicos destas neoplasias, reações de imunoistoquímica costumam ser fundamentais para o correto diagnóstico. Assim, o objetivo deste estudo foi analisar as características clínicas, histopatológicas e imunoistoquímicas de uma série de casos de LM e LMS afetando a região oral e maxilofacial. Todos os casos diagnosticados como LM e LMS no período de 2000 a 2019, foram recuperados dos arquivos de seis serviços de diagnóstico oral. As características clínicas e demográficas foram obtidas a partir dos registros patológicos dos pacientes, enquanto as características microscópicas e imunoistoquímicas foram revisadas,por pelo menos dois autores simultaneamente, ou repetidas sempre que necessário para confirmação do diagnóstico. Os anticorpos utilizados na imunoistoquimica foram: anti-α-SMA, actina músculo-específica(HHF 35), h-caldesmona e Ki67, entretanto, sempre que necessário, utilizamos S100, desmina e CD34. Foram obtidos 22 casos de LM e cinco LMS. No grupo de LM, houve predomínio do sexo masculino, com média de idade de 45,7 anos. O lábio superior foi o local mais acometido, sendo 18 casos classificados como angioleiomiomas e quatro como LM sólido.O tempo de evolução médio foi de 44.5 meses e 91% dos pacientes foram assintomáticos.Já no grupo LMS houve predomínio do sexo feminino, com média de idade de 47,6 anos. A mandíbula foi o local mais afetado.Todos os pacientes foram sintomáticos, apresentando dor, dormência do lábio inferiror e parestesia do nervo alveolar inferior, com um tempo de evolução variando entre 2-4 mêses. Proliferação difusa de células fusiformes, com necrose e figuras mitóticas, foram achados microscópicos frequentes. LM e LMS foram consistentemente positivos para α-SMA, HHF-35 e h-caldesmona. Concluimos nesse estudo que tanto o LM como o LMS orais são neoplasias raras, sendo que a última geralmente se apresenta como doença metastática. A avaliação histológica de rotina pode ser muito sugestiva para o diagnóstico de LMs orais, mas a imunoexpressão de h-caldesmona é fortemente recomendada para confirmação do diagnóstico de LMS.

Smooth muscle tumors are considered rare in the oral cavity, with the frequency of leiomyoma (LM) and leiomyosarcoma (LMS) being less than 1% of the total number of neoplasms diagnosed in this region. LM are clinically characterized as a slow and asymptomatic nodule, being classified as angioleiomyoma and solid LM. Histologically, solid LM appear as a proliferation of spindle cells with eosinophilic cytoplasm forming bundles or interlaced fascicles, while angioleiomyomas are composed of bundles of bland, well-differentiated smooth muscle cells and intervening variably sized blood vessels. Surgical removal is the recommended treatment and recurrences are extremely rare. LMS, on the other hand, presents clinically as a tumoral lesion that exhibits rapid and locally destructive growth, histologically showing a fascicular growth of spindle-shaped neoplastic cells, with figures of mitosis, cell atypia and tissue necrosis. The most used treatment is radical surgical excision, often showing local recurrences and distant metastases. Due to the long list of microscopic differential diagnoses of these two neoplasms, immunohistochemistry reactions are usually fundamental for the correct diagnosis. Thus, the aim of this study was to describe in detail the clinical, histopathological and immunohistochemical characteristics of a series of cases of LM and LMS affecting the oral and maxillofacial region. All cases diagnosed as LM and LMS in the period from 2000 to 2019, were recovered from the files of six oral diagnostic services. Clinical and demographic characteristics were obtained from patients' pathological records, while microscopic and immunohistochemistry characteristics were reviewed, by at least two authors simultaneously, and completed when necessary to confirm the diagnosis. Antibodies used in immunohistochemistry were: anti-α-SMA, muscle-specific actin(HHF-35), h- caldesmon and Ki67, however, whenever necessary, we used S100, desmin and CD34. Twenty-two LM and five LMS were obtained in the study. In the LM group, there was a predominance of males, with a mean age of 45.7 years. The upper lip was the most affected site, with 18 cases classified as angioleiomyoma and four as solid LM. The mean evolution time was 44.5 months and 91% of patients were asymptomatic. In the LMS group, there was a predominance of females, with a mean age of 47.6 years. The jaw was the most affected site. All patients were symptomatic, with pain, lower lip numbness and inferior alveolar nerve paresthesia, with evolution time ranging from 2- 4 months. Diffuse proliferation of spindle cells, with necrosis and mitotic figures, were frequent microscopic findings. LM and LMS were consistently positive for smooth α- SMA, HHF-35 and h-caldesmon. We concluded in this study that both LM and oral LMS are uncommon neoplasms, the latter usually presenting as a metastatic disease. H&E assessment can be very suggestive for oral LM, but immunohistochemical staining for h-caldesmon is strongly recommended to confirm the diagnosis of LMS.

Emprego de marsupialização para tratamento de cisto odontogênico ortoceratinizado extenso em mandíbula: relato de caso / Use of marsupialization for treatment of a large orthokeratinized odontogenic cyst in jaw: case report

O cisto odontogênico ortoceratinizado (COO) é um cisto de desenvolvimento incomum dos maxilares e que foram considerados uma entidade distinta dos ceratocistos odontogênicos (COs), uma vez que apresentam um comportamento menos agressivo. A forma de tratamento recomendado para COO geralmente envolve medidas conservadoras, tendo em vista a sua menor taxa de recorrência em relação ao ceratocisto odontogênico. Histologicamente, os COOs são cistos de desenvolvimento, revestidos por epitélio ortoceratinizado e constituídos por uma camada uniforme de epitélio escamoso estratificado regular, sendo que a camada basal exibe células em paliçadas ou planas, com hipercromatismo nuclear. O objetivo deste estudo é, portanto, relatar um caso clínico de um paciente atendido na Clínica de cirurgia bucomaxilofacial e estomatologia do Hospital Metropolitano Odilon Behrens (HMOB), portador de um COO extenso em mandíbula, tratado por marsupialização e com acompanhamento clínico e radiográfico no período de 07 anos. A literatura relevante sobre as características clínico patológicas do COO foi revisada, e não foi encontrado nenhum estudo que utilizasse como única técnica de tratamento a marsupialização do COO. Logo, o presente trabalho se trata de um estudo inédito em que se obteve sucesso no tratamento.(AU)

The orthokeratinized odontogenic cyst (OOC) is a cyst of unusual development of the jaws and which were considered a distinct entity of odontogenic keratocysts (OKs), since they present a less aggressive behavior. The recommended form of treatment for OOC usually involves conservative measures in view of its lower recurrence rate in relation to odontogenic keratocysts. Histologically, OOCs are developmental cysts coated with orthoceratinized epithelium and consist of a uniform layer of regular stratified squamous epithelium, with the basal layer exhibiting cells in palisades or flat, with nuclear hyperchromatism. The purpose of this study is to report a clinical case of a patient attended at the Bucomaxillofacial and Stomatology Clinic of the Hospital Metropolitano Odilon Behrens (HMOB), with an extensive OOC in the mandible, treated by marsupialization and clinical and radiographic follow-up in the period of 07 years. The relevant literature on the pathological clinical features of OOC has been reviewed. In the literature, no study was found that used OOC marsupialization as the only treatment technique. Therefore, this is an unprecedented study that has been successful in treatment.(AU)