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.

Anaplastic Large Cell Lymphoma with Oral Manifestation: A Series of Four Cases and Literature Review.

The aim of this study was to describe the clinicopathological and immunohistochemical features of four cases of anaplastic large cell lymphoma (ALCL) diagnosed through oral manifestations. Clinical data were collected from charts of a single oral pathology laboratory over a 5-year period (2014-2019) and all cases were evaluated by conventional hematoxylin and eosin staining and an extended immunohistochemical panel comprising CD45, CD20, CD3, CD4, CD7, CD30, CD99, CD138, cytokeratin AE1/AE3, EMA, ALK, MUM-1 and Ki-67. The study included 3 male (75%) and 1 female (25%) patients, with a median age of 44 years. The most common intraoral affected site was the alveolar ridge (50%). Clinically, all cases were characterized as an ulcerated bleeding mass. Microscopically, proliferation of anaplastic large lymphoid cells with medium to large-sized, abundant amphophilic to eosinophilic cytoplasm and eccentric nuclei were observed. All cases were positive for CD30, while two cases strongly express ALK. Two patients died of the disease. Careful correlation of clinical, morphological and immunohistochemical data are necessary to establish the diagnosis of oral manifestation of ALCL since its microscopical features may mimic other malignant tumors. Clinicians and pathologists should consider ALCL in the differential diagnosis when evaluating oral ulcerated swellings exhibiting large lymphoid cells in patients with lymphadenopathy.

EBV-positive mucocutaneous ulcers: a presentation of two cases and a brief literature review

A úlcera mucocutânea associada ao Vírus Epstein Barr é uma doença linfoproliferativa indolente de linfócitos B que foi descrita em 2010 por Stefan D. Dojcinov e cols. Pode ocorrer em associação com etiologias imunossupressoras, incluindo deficiência imune primária, infecção pelo vírus da Imunodeficiência Humana (HIV), pacientes pós transplantes e em uso de metotrexato ou antagonistas TNF-alfa. Os pacientes apresentam quadro clínico de úlcera de surgimento insidioso, com localização cutânea ou de mucosas, preferencialmente de orofaringe. Os achados histopatológicos da lesão revelam áreas ulceradas com infiltrado polimórfico de linfócitos, plasmócitos, histiócitos e eosinófilos em meio a células grandes, pleomórficas que lembram células de Hodgkin e de Reed-Sternberg. O epitélio adjacente geralmente mostra núcleos atípicos e hiperplasia pseudoepiteliomatosa. As células grandes pleomórficas mostram positividade para CD20, CD30, Oct-2, PAX5 e EBV. Estas células podem ser também positivas para MUM1, focalmente positivas para Bcl-6 e não costumam expressar CD10. Apesar da presença de células muito atípicas, o curso clínico é indolente, sem progressão para doença disseminada, sendo essencial diferenciá-la de doenças linfoproliferativas malignas. Descrevemos dois casos de pacientes com diagnóstico de úlcera mucocutânea associada ao vírus Epstein Barr (EBV) para adicionar aos poucos casos já descritos na literatura.

Hanseníase virchowiana em fase reacional com predomínio das manifestaçöes ósteo-musculo-articulares e com o achado do bacilo na biópsia de sinóvia / Virchowian hansenianis in reactional phase with predominance of muscular and articular manifestations and presence od bacillus in synovia's biopsy

Apresentaçäo de um enfermo com hanseníase virchowiana, cujo início dos sintomas datava de seis anos, internado por várias ocasiöes sem que o diagnóstico fosse feito. Três meses antes da última internaçäo houve manifestaçöes gerais e músculo-articulares importantes, acompanhadas de tendinite e sinovite de monta. Na admissäo, o exame clínico evidenciava os sinais clássicos da hanseníase lepromatosa e o bacilo foi demonstrado na linfa pesquisada e na biópsia de sinóvia que se encontrava bastante espessada. Säo enfatizados os fatos referentes a necessidade de uma maior conscientizaçäo sobre esta notável endemia nacional e sobre os aspectos polimórficos da lepra em fase reaciconal, a qual se reveste de outras expressöes clínicas decorrentes de fenômenos imunológicos e que se torna enganadora ao imitar outras patologías, principalmente as colagenoses

Hipereosinofilia na paracoccidioidomicose: relato de um caso / Hypereosinophilia in the paracoccidioidomycosis: case report

É relatado um caso de paracoccidioidomicose disseminada (tipo juvenil) em cujo transcurso foi observado número excepcionalmente elevado de eosinófilos no sangue. Casos incomuns como esse foram revistos

Mucormicose (zigomicose) rino cerebral: apresentaçäo de dois casos com sobrevida / Mucomycosis (zygomycosis) rhinocerebral: report of two cases with prognosis

Säo relatados dois casos de mucormicose rinocerebral em jovens diabéticos. Num paciente, a micose teve curso arrastado e apesar do envolvimento sino-órbito-cerebral, a sintomatologia refletiu apenas o comprometimento órbito-ocular. No outro paciente, a doença teve curso agudo nasal e palatina, seios paranasais, tecidos periorbitários, olho e nervos cranianos, porém sem acometimento cerebral. Houve sobrevida de ambos os pacientes, porém, em apenas um deles o diagnóstico foi completo pelo isolamento do agente