Oral manifestations of Hydroa vacciniforme-like lymphoproliferative disorder: a clinicopathological study of a Peruvian population.

BACKGROUND: Hydroa vacciniforme-like lymphoproliferative disorder (HVLPD) is a chronic Epstein-Barr virus (EBV)-positive lymphoproliferative disease which may either present as an indolent condition or progress to a systemic T-cell lymphoma. METHODS: All HVLPD diagnosed over a 10-year period were retrieved, and clinical data regarding sex, age, oral and systemic manifestations, and clinical follow-up were obtained. Immunohistochemistry was done in order to characterize the lymphoid cells, and in situ hybridization was used to demonstrate the presence of EBV. RESULTS: Eleven cases were included, with a male predominance and a mean age of 25.1 years. Buccal mucosa and the lips were the most affected oral sites, appearing as painful ulcers. All patients exhibited facial oedema, usually affecting the lips, nose and periorbital region. The clinical course was gradual but progressive, with four patients having fever and 3 showing lymphadenopathies. All cases showed a moderate to severe lymphocytic infiltrate with angiotropism, angiocentricity and epidermotropism. Two cases affecting the lip skin exhibited a periappendageal lymphocytic infiltrate. Few large pleomorphic cells were found, surrounded by smaller and medium-sized lymphoid cells, as well as reactive plasma cells, macrophages, neutrophils and eosinophils. All lesions exhibited a cytotoxic T-cell (CD8+) phenotype with a variable proliferative index. All cases were associated with EBV, and all patients died due to complications of the disease. CONCLUSIONS: HVLPD is a rare disease that may show oral involvement with a cytotoxic T-cell phenotype, and is strongly associated with EBV. As shown in this series, HVLPD may show aggressive clinical behaviour.

Intraoral EBV-positive sporadic Burkitt lymphoma in an elderly patient with bilateral presentation

Sporadic Burkitt lymphoma (SBL) is a variant of Burkitt lymphoma that occurs worldwide, affecting mainly children and young adults. Association with Epstein-Barr virus (EBV) can be identified in approximately 20-30% of cases. Herein we described a case of a 63-year-old male presenting intraoral bilateral mandibular swelling, subjacent to fixed dental prosthesis, with one month of duration. Incisional biopsies were performed, and after two days, the patient was hospitalized due to malaise and breathing difficulty, and died after a week when an abdominal tumor was detected. The mandibular biopsies revealed a diffuse proliferation of medium-sized monomorphic atypical lymphoid cells exhibiting numerous mitoses and areas of "starry-sky" pattern. The tumor showed immunohistochemical positivity for CD20, CD10, Bcl-6, and Ki-67 (≈ 100%); it was negative for CD3, Bcl-2, Vs38c, and MUM-1. Positivity for EBV was found by in situ hybridization. The final diagnosis was intraoral SBL positive for EBV. Clinical, morphological and molecular criteria are necessary for the correct diagnosis of aggressive B-cell neoplasms positive for EBV in elderly patients.

Extranodal NK/T-Cell Lymphoma, Nasal Type in Guatemala: An 86-Case Series Emphasizing Clinical Presentation and Microscopic Characteristics.

Extranodal NK/T-cell lymphoma, nasal type (ENKTCL-NT) is a lymphoid malignancy that mainly affects the nasopharynx and is associated with the Epstein-Barr virus (EBV). Increased incidence is seen in some Latin American and Asian countries. In this study, we describe a case series of 86 Guatemalan patients with ENKTCL-NT from a single diagnostic head and neck center. We emphasize the distinctive clinical, microscopic, and immunohistochemical (IHC) features, as well as EBV positivity by in situ hybridization (ISH). Most of the patients (90.6%) were of Mayan descent and low socioeconomic status (SES). Males were more often affected than females, comprising 68.3% of cases. Patient age ranged from 8 to 71, with a mean of 34.7 years. All cases arose in the upper aerodigestive tract and mainly presented as a rapidly progressive, necrotizing midfacial process affecting the nasal, nasopharyngeal, sinonasal, palatal, and oropharyngeal structures. Microscopically, ENKTCL-NT showed a diffuse polymorphic and atypical lymphoid infiltrate. Angiocentric and angiodestructive growth patterns were present with associated necrosis. Peripheral hyaline necrosis of blood vessels was a histologic hallmark. The ISH and IHC profiles included positivity of EBV, LCA, CD3, CD45RO, CD30 (focal in 39.2%), granzyme-B, TIA-1, perforin (in 82.3%), and CD56 (in 83.7%). CD20 was negative, and the Ki-67 index ranged from 70 to 90%. In Guatemala, this lymphoma is strongly associated with people of low SES and indigenous ethnicity. When affected, the palatal mucosa provides the best site to obtain a representative biopsy. Since ENKTCL-NT is highly aggressive, it is extremely important to recognize the spectrum of clinical presentations and microscopic features in order to avoid misdiagnosis and treatment delay.

Frequency of monoclonal B-cell lymphocytosis in relatives of patients with chronic lymphocytic leukemia.

INTRODUCTION: Monoclonal B-cell lymphocytosis is a symptom free condition characterized by the circulation of small clonal population of B lymphocytes in peripheral blood (less than 5x10(9)/L) expressing an immunophenotype similar to chronic lymphocytic leukemia. Different studies based on big hospital series have manifested a higher risk in subjects with monoclonal B-cell lymphocytosis to progress to a chronic lymphocytic leukemia. The behavior of this hematologic entity is unknown therefore its frequency in sporadic chronic lymphocytic leukemia patient relatives was determined. METHODS: Transversal descriptive study, 8 color flow cytometry was performed using two of the tubes of the Euro Flow recommended panel, with modifications, for the diagnose of chronic lymphoproliferative disorders of B lymphocytes; besides, a fluorescence in situ hybridization was performed. univariate and bivariate analyses of the information were performed. RESULTS: Monoclonal B-cell lymphocytosis frequency found in 51 analyzed relatives was 2%, it was a female participant, 59 years old, with a total leukocyte count of 7.7x10(9)/L and a B lymphocyte count of 0.124x10(9)/L; from these, 0.04x10(9)/L were clonal cells with restrictions of the kappa light chain. Rearrangements of the IGH gene (14q32) were found. CONCLUSION: Monoclonal B-cell lymphocytosis was detected in one relative of a patient with sporadic chronic lymphocytic leukemia in a frequency similar to the one reported in general population.

INTRODUCCIÓN: La linfocitosis monoclonal de células B es una condición asintomática que se caracteriza por la circulación de pequeñas poblaciones clonales de linfocitos B en sangre periférica (menos de 5x109/L) que expresan un inmunofenotipo similar al de la leucemia linfoide cónica. Diferentes estudios basados en grandes series hospitalarias, han puesto de manifiesto un riesgo más elevado de los sujetos con linfocitosis monoclonal de células B de progresar a una leucemia linfoide crónica. En Colombia se desconoce el comportamiento de esta entidad hematológica, por tal razón se determinó su frecuencia en familiares de pacientes con leucemia linfoide crónica esporádica. MÉTODOS: Estudio descriptivo transversal, se realizó citometría de flujo de 8 colores utilizando dos de los tubos del panel recomendado por Euro Flow para el diagnóstico de enfermedades linfoproliferativas crónicas de linfocitos B con modificaciones, además se hizo hibridación fluorescente in situ. Se realizó análisis univariado y bivariado. RESULTADOS: La frecuencia de linfocitosis monoclonal de células B encontrada en los 51 familiares analizados fue del 2%, se trató de un participante del sexo femenino y 59 años de edad, con un recuento total de leucocitos de 7,7x109/L y un recuento de linfocitos B de 0,124x109/L; de estos 0,04x109/L eran células clonales con restricción de la cadena ligera kappa. Se encontraron reordenamientos del gen IGH (14q32). CONCLUSIÓN: Se detectó linfocitosis monoclonal de células B en un familiar de paciente con leucemia linfoide cónica esporádica en una frecuencia similar a la informada en la población general.

Frequency of monoclonal B-cell lymphocytosis in relatives of patients with chronic lymphocytic leukemia / Frecuencia de linfocitosis monoclonal de células B en familiares de pacientes con leucemia linfoide crónica

Introduction: monoclonal B-cell lymphocytosis is a symptom free condition characterized by the circulation of small clonal population of B lymphocytes in peripheral blood (less than 5x10(9)/L) expressing an immunophenotype similar to chronic lymphocytic leukemia. Different studies based on big hospital series have manifested a higher risk in subjects with monoclonal B-cell lymphocytosis to progress to a chronic lymphocytic leukemia. The behavior of this hematologic entity is unknown therefore its frequency in sporadic chronic lymphocytic leukemia patient relatives was determined. Methods: transversal descriptive study, 8 color flow cytometry was performed using two of the tubes of the Euro Flow recommended panel, with modifications, for the diagnose of chronic lymphoproliferative disorders of B lymphocytes; besides, a fluorescence in situ hybridization was performed. univariate and bivariate analyses of the information were performed. Results: monoclonal B-cell lymphocytosis frequency found in 51 analyzed relatives was 2%, it was a female participant, 59 years old, with a total leukocyte count of 7.7x109/L and a B lymphocyte count of 0.124x10(9)/L; from these, 0.04x10(9)/L were clonal cells with restrictions of the kappa light chain. Rearrangements of the IGH gene (14q32) were found. Conclusion: monoclonal B-cell lymphocytosis was detected in one relative of a patient with sporadic chronic lymphocytic leukemia in a frequency similar to the one reported in general population.

Introducción: La linfocitosis monoclonal de células B es una condición asintomática que se caracteriza por la circulación de pequeñas poblaciones clonales de linfocitos B en sangre periférica (menos de 5x10(9)/L) que expresan un inmunofenotipo similar al de la leucemia linfoide cónica. Diferentes estudios basados en grandes series hospitalarias, han puesto de manifiesto un riesgo más elevado de los sujetos con linfocitosis monoclonal de células B de progresar a una leucemia linfoide crónica. En Colombia se desconoce el comportamiento de esta entidad hematológica, por tal razón se determinó su frecuencia en familiares de pacientes con leucemia linfoide crónica esporádica. Métodos: Estudio descriptivo transversal, se realizó citometría de flujo de 8 colores utilizando dos de los tubos del panel recomendado por Euro Flow para el diagnóstico de enfermedades linfoproliferativas crónicas de linfocitos B con modificaciones, además se hizo hibridación fluorescente in situ. Se realizó análisis univariado y bivariado. Resultados: La frecuencia de linfocitosis monoclonal de células B encontrada en los 51 familiares analizados fue del 2%, se trató de un participante del sexo femenino y 59 años de edad, con un recuento total de leucocitos de 7,7x10(9)/L y un recuento de linfocitos B de 0,124x109/L; de estos 0,04x10(9)/L eran células clonales con restricción de la cadena ligera kappa. Se encontraron reordenamientos del gen IGH (14q32). Conclusión: Se detectó linfocitosis monoclonal de células B en un familiar de paciente con leucemia linfoide cónica esporádica en una frecuencia similar a la informada en la población general.

Actualización del reporte de biopsiapor aspiración con aguja fina de mama / Report of breast fine needle aspiration biopsy: an update

Las recomendaciones para la biopsia por aspiración con aguja fina de mama se desarrollaron y aprobaron en 1997 por el Instituto Nacional de Cáncer en Bethesda, Estados Unidos y fueron adaptadas a nuestro país en 2007, sin embargo, en los últimos años no se han realizado cambios formales en estas indicaciones. El objetivo de este módulo es presentar la actualización del reporte de biopsia por aspiración con aguja fina de mama, usando el sistema de reporte Bethesda, realizado por consenso con un grupo de patólogos, clínicos, radiólogos, cirujanos de mama y otros profesionales de la salud de Colombia y otros países, y con base en la experiencia realizando biopsia por aspiración con aguja fina de mama del Hospital Pablo Tobón Uribe y de Dinámica IPS.

Recommendations for breast fine needle aspiration biopsy were developed and approved in 1997 by The National Cancer Institute of Bethesda, United States, , and were adapted to our country on 2007, however, in last years these indications have not changed in a formal manner. The purpose of this review was to provide an update of the report for breast fine needle aspiration biopsy using the Bethesda system. This guide was made by consensus with pathologists, clinicians, radiologists, breast surgeons and other health professionals of Colombia and other countries. The update was basis on the experience of Hospital Pablo Tobon Uribe and Dinamica IPS in performing breast fine needle aspiration biopsy.

Linfangiectasia intestinal asociada a hemihipertrofia: Reporte de caso / Intestinal Lymphangiectasia associated with Hemihypertrophy: A case report

La linfangiectasia intestinal primaria es una enfermedad caracterizada por dilatación de los vasos linfáticos del intestino, manifestándose como una enteropatía perdedora de proteínas. Presentamos un paciente con diarrea crónica, prolapso rectal recurrente y hemihipertrofia de miembro superior izquierdo, asociado a linfopenia e hipoalbuminemia. Por endoscopia digestiva superior y biopsia se diagnostica linfangiectasia intestinal primaria y se inicia tratamiento nutricional exitosamente.

Primary intestinal lymphangiectasia is a disease characterized by dilated intestinal lymph vessels which manifests as a protein losing enteropathy. We present a patient with chronic diarrhea, recurrent rectal prolapse and left upper limb hemihypertrophy associated with lymphopenia and hypoalbuminemia. Primary intestinal lymphangiectasia was diagnosed with upper endoscopy and biopsy. Nutritional treatment was successfully begun.

Desorden linfoproliferativo T sistémico de la infancia asociado al virus Epstein-Barr: reporte de un caso / Systemic Epstein-Barr virus-positive T celllymphoproliferative disease of childhood: a case report

Resumen: el desorden linfoproliferativo T sistémico de la infancia asociado al virus Epstein-Barres una entidad clínica de descripción reciente, potencialmente mortal en niños y en adultos jóvenes.Se caracteriza por una proliferación clonal de linfocitos T con un fenotipo citotóxico activadoinfectados por el virus. Es más frecuente en países de Asia y México, y se desconoce su incidenciaen el medio. Se reporta un caso de esta enfermedad, con progresión indolente y desenlace fatal.

Abstract: systemic Epstein-Barr virus-positive T cell Iymphoproliferative disease of childhood is arecently described, potentially lethal clinical entity in children and young adults, characterized bya clonal proliferation of EBV-infected T-cells with an activated cytotoxic phenotype. It is typicallyprevalent in Asian countries and Mexico, and its incidence is unknown in our country. This reportdescribes a case of systemic Epstein-Barr virus positive.

Expression of hMLH1 and hMSH2 proteins in pleomorphic adenoma of minor salivary glands: relationship with clinical and histologic findings.

OBJECTIVE: The aim was to examine the relationship between clinicopathological features and immunoexpression of hMLH1 and hMSH2 proteins in pleomorphic adenoma (PA) of minor salivary glands. STUDY DESIGN: Paraffin-embedded samples of typically benign PA lesions (n = 35) were prepared for histologic and immunohistochemical assessment. Based on the clinicopathologic features, the samples were categorized into low- and high-risk subtypes for their estimated potential for malignant transformation. RESULTS: Immunohistochemical analysis revealed strong correlations regarding the expression estimation and staining-intensity distribution (SID) scores between the two proteins. Although there was no relationship between marker immunoexpression and SID scores regarding clinical parameters, statistically significant variations for these parameters were evident regarding some histologic criteria or for risk stratification subtypes. CONCLUSION: The findings of this study could suggest the relationship of a DNA mismatch repair deficiency with high-risk subtypes of PA and the implication of its role in the origin and progression of these subtypes.

Melanoma maligno de la mucosa oral: reporte de un caso / Malignant melanoma of the oral mucosa: report of a case

Los melanomas de la cavidad oral son tumores raros que corresponden al 0,5 por ciento de todas las malignidades orales. Los sitios de presentación más frecuentes son el paladar y la encía. Generalmente al momento del diagnóstico suelen estar en estadios avanzados. Describimos un caso de un hombre de 70 años que consultó por una lesión nodular pigmentada en el paladar duro, con otras máculas pigmentadas alrededor, y un nódulo cervical de 2 cm. El estudio histológico mostró una extensa infiltración tumoral por células fusiformes y epiteliodes con moderada pleomorfia celular y pigmento melánico en su citoplasma, la inmunohistoquímica reveló positividad para S-100 y HMB-45. Se realizó citología por aspiración del nódulo cervical con aguja fina, visualizándose células con citoplasma amplio con presencia de pigmento melánico y núcleos pleomórficos. El diagnóstico diferencial de esta lesión incluye tumores vasculares benignos y malignos, tumores y quistes de glándulas salivales además de lesiones pigmentarias benignas de las mucosas.

Oral melanomas are rare tumors corresponding to 0.5% of all the oral malignancies. The hard palate and gingiva are more frequently affected. At the moment of diagnosis they are generally in an advanced stage. We describe a case of a 70 years old male with nodular and pigmented lesion in hard palate and other macular pigmented lesions, besides a 2cm cervical nodule. The histo-pathological study showed an extensive infiltration by moderately pleomorphic spindle and epitheliod tumoral cells with melanin pigment in the cytoplasm. Immune-histo-chemical staining was positive to S-100 and HMB-45. Cytology by aspiration of the cervical node was carried out with a fine needle, showing cells with wide cytoplasm and presence of melanin pigment and pleomorphic nuclei. The differential diagnosis includes benign and malign vascular tumors, tumors and cysts of salivary glands, and benign pigmented lesions of the mucosa.

Leiomiosarcoma de mediastino anterior. Aportación de un caso y revisión de la literatura / Mediastinal leiomyosarcoma: a study of one case and review of the literature

Los leiomiosarcomas del mediastino son tumores raros, que generalmente derivan de la pared de la tráquea, esófago y grandes vasos, aunque existen algunos casos de origen incierto. Describimos un caso de un varón de 68 años, con clínica de síndrome de compresión de vena cava superior. La radiología y tomografía computerizada de tórax revela una masa localizada en el mediastino anterior. El estudio patológico muestra una infiltración tumoral de células fusiformes con moderada pleomorfismo, que reaccionan positivamente a la inmunotinción con actina y desmina. El diagnóstico diferencial incluye todos los sarcomas y tumores con patrón fusocelular que se presentan con esta localización. El tratamiento que se ha empleado en todos los casos revisados de la literatura ha sido la resección quirúrgica, y en pocos casos se ha usado terapia adyuvante de quimio y radioterapia (AU)