Comportamiento del linfoma no Hodgkin en la edad pediátrica / Behavior of non-Hodgkin lymphoma at pediatric ages

INTRODUCCIÓN: los linfomas no Hodgkin son neoplasias hematológicas frecuentes en la infancia. OBJETIVO: describir las diferentes formas de presentación de esta enfermedad en la edad pediátrica, y determinar el promedio de años vividos después de concluido el tratamiento. MÉTODOS: se realizó un estudio descriptivo retrospectivo en 79 pacientes con diagnóstico de linfomas no Hodgkin atendidos en el servicio de Oncocirugía del Hospital Pediátrico Docente "William Soler", de marzo de 1995 a marzo de 2014. RESULTADOS: 48 pacientes fueron del sexo masculino y 31 del femenino. El grupo de edad de mayor afectación fue el de 10 a 14 años. El 54,4 % de los pacientes tuvieron linfomas de localización abdominal y el 29,1 % fueron de localización mediastinal. Otros sitios afectados fueron la región cervical, la amígdala palatina y renal primaria en el 10,1, 3,8 y 2,6 % de los pacientes respectivamente. La variante histológica más frecuente fue el linfoma no Hodgkin inmunofenotipo B (75,9 %), seguido del linfoma no Hodgkin inmunofenotipo tipo T en el 21,5 % de los casos. El dolor abdominal y la masa tumoral palpable fue la manifestación clínica principal en el 64,5 %. El promedio de años vividos en el linfoma no Hodgkin de localización renal, cervical y amígdala palatina fue ligeramente superior (5,7 ± 0,3, 5,5 ± 1,8 y 5,2 ± 0,7 respectivamente). CONCLUSIONES: el linfoma no Hodgkin inmunofenotipo B de localización abdominal es el más frecuente. El dolor y el tumor abdominal son las manifestaciones clínicas principales, y los pacientes con linfomas no Hodgkin de la región cervical y amígdala palatina tienen mayor promedio de vida después de concluido el tratamiento.

INTRODUCTION: non-Hodgkin lymphomas are frequent hematological neoplasias in infancy. OBJECTIVE: to describe the different forms of presentation of this disease at the pediatric age and to determine the average years lived after the treatment. METHODS: a retrospective and descriptive study of 79 patients was conducted; they had been diagnosed as non-Hodgkin lymphoma cases and attended to at the oncologic surgery service of "William Soler" pediatric teaching hospital from March 1995 to March 2014. RESULTS: forty eight patients were males and 31 were females. The most affected age group was the 10-14 years-old one. In the group, 54.4 % of patients had lymphomas located in the abdominal region and 29.1 % in the mediastinal location. Other affected areas were cervical region, palatal tonsil and primary renal region in 10.1 %, 3.8 % and 2.6 % of patients, respectively. The most common histological variant was immunophenotype B non-Hodgkin lymphoma (75.9 %) followed by immunophenotype T non-Hodgkin lymphoma (21.5 %). Abdominal pain and palpable tumor mass was the main clinical manifestation in 64.5 % of cases. The average life years in non-Hodgkin lymphoma located in the renal region, cervical region and palatal tonsil was slightly higher (5.7±0.3, 5.5±1.8 and 5.2±0.7, respectively). CONCLUSIONS: the immunophenotype B non-Hodgkin lymphoma of abdominal location is the most common. Abdominal pain and tumors are the main clinical manifestations and the patients with non-Hodgkin lymphomas in the cervical region and the palatal tonsil show higher average life years after treatment.

Linfoma T/NK nasal fenotipo T citotóxico / Nasal NK/T cell lymphoma of cytotoxic phenotype

El linfoma nasal de células T/natural killer fue definido y caracterizado, en el año 2001, por la Organización Mundial de la Salud (OMS). Se localiza preferentemente en las fosas nasales u senos maxilares, mostrando un curso clínico agresivo, definido por destrucción de los tejidos circundantes. Se ha observado una frecuente asociación con el virus de Epstein-Barr, de difícil tratamiento. Se presenta un caso con buena respuesta terapéutica a la radioterapia.

Nasal T cell/natural killer lymphoma was defined and described, in 2001, by the World Health Organization (WHO). It typically appears in the nasal cavity and maxillary sinuses, showing an aggressive clinical course, characterized by destruction of surrounding tissue. A frequent association with Epstein-Barr virus has been reported, which hampers the treatment. We present a case with good therapeutic response to radiotherapy.

Lymphoma of frontotemporal region with massive bone destruction and intracranial and intraorbital extension.

Primary non-Hodgkin's lymphoma with unilateral proptosis and diffuse involvement of the cra-nial vault and brain parenchyma is extremely rare. A 50-year-old woman developed a progressively increasing proptosis of her right eye, associated with a subcutaneous mass over the right frontotemporal region over the last 5 months. CT scan showed a high-density contrast-enhancing lesion with wide involvement of the cranium and intracranial and intraorbital extension. We performed a wedge biopsy for further analysis. Histological examination revealed that the tumor was non-Hodgkin's lymphoma. There was no evidence of systemic involvement. The patient received radiotherapy and was doing well at 18 months' follow-up. Primary malignant lymphoma involving the orbit and cranial vault is a rare malignancy, and treatment remains to be defined.

Linfoma não-Hodgkin de células B: relato de caso / Non-Hodgkin B-cells lymphoma: a case report

O linfoma não-Hodgkin é a segunda neoplasia maligna mais prevalente na região da cabeça e do pescoço. A etiologia dos linfomas é desconhecida, porém distúrbios da função imune podem conferir risco aumentado para essa desordem. Apesar de as lesões orais serem freqüentemente uma manifestação da doença amplamente disseminada, às vezes, o linfoma origina-se nos tecidos bucais, sem ter se espalhado para outros locais. Paciente P.J.Q., 54 anos, masculino, compareceu ao ambulatório de cirurgia bucomaxilofacial do hospital São Félix, queixando-se de aumento de volume na região zigomática e na paranasal esquerda, após trauma severo por martelo, há um mês. Foi realizado punção aspiratória sem ter sido detectado qualquer sinal de infecção ou líquido cístico, no exame radiográfico não foi detectado fratura óssea. Foi colhido material para análise histopatológica, tendo como diagnóstico: linfoma não-Hodgkin de células B. Um ano após o diagnóstico, finalizado o tratamento quimioterápico, o paciente retornou para revisão quando foram realizadas extrações dentárias, para que pudesse ser submetido à radioterapia sem complicações. A proposta deste trabalho é apresentar um caso clínico de linfoma não-Hodgkin de células B, enfatizando a sua importância clínica e a necessidade do diagnóstico precoce na tentativa de melhorar a qualidade de vida dos pacientes.

Non-Hodgkin lymphoma is the second most prevalent malignant cancer of the head and neck region. The etiology of the lymphomas is unknown, but disorders of the immune function may result in an increased risk for this disease. Despite the fact that oral lesions are frequently a manifestation of a systemic pathology, the lymphoma sometimes arises in the oral tissues, without spreading to other organs. Patient P.J.Q, a fifty-four-year-old, male, presented at the clinic of oral and maxillofacial surgery of São Felix hospital, complaining of a swelling located in the left zygomatic paranasal region resulting from severe trauma caused by a hammer a month previously. Aspiratory punction was performed, resulting in no signs of infection or cystic fluid, and a radiograph revealed no bone fracture. Material from the lesion was collected sent for a histopathology examination, which led to a diagnosis of non-Hodgkin B-cell lymphoma. One year after the diagnosis, having completed a course of chemotherapy, the patient returned for follow-up, at which time some teeth were extracted so that the patient could undergo radiotherapy without complications. The aim of this paper is to present a case report of non-Hodgkin B-cell lymphoma, emphasizing the clinical importance of the disease and the need for an early diagnosis with a view to achieving a better life quality for patients.

Salvage abdominal irradiation for refractory non-Hodgkin's lymphoma.

BACKGROUND: Abdominal irradiation, as a part of treatment, is often ignored in the management of refractory non-Hodgkin's lymphoma (NHL). ObjecTIVE: To evaluate the efficacy and the toxicity of this approach after failure of chemotherapy. MATERIALS AND METHODS: 27 patients with intraabdominal lymphoma underwent salvage irradiation between 1982 and 2001. All patients were treated with a Cobalt-60 machine. The total dose administered to the abdomen was 18-20 Gy at the rate of 1.5-1.8 Gy per daily fraction, followed by a boost to gross disease up to 20 Gy. All patients had previously been heavily pretreated with chemotherapy. Fourteen patients, nine with follicular and five with diffuse lymphomas, had primary refractory tumors that had never achieved remission. Thirteen patients, six with follicular and seven with aggressive tumors, had refractory relapsed tumors after achieving one or more complete remissions. RESULTS: The response rate was 77%. The median follow-up was 53 months. The 5-year and 10-year survival rates were 25 and 17%, respectively. The in-field and out-of-field recurrence rates were 22 and 33%, respectively. Survival rates were significantly better for patients with refractory relapse compared to those with primary refractory lymphoma (P < 0.01). There was no significant difference in terms of response, recurrence, or survival rates between follicular and aggressive types. Out-of-field recurrence occurred more frequently in initial stage III and IV disease. Toxic deaths occurred in three patients (11%). CONCLUSION: Salvage radiotherapy for refractory abdominal NHL is a feasible alternative for both follicular and diffuse subtypes and may provide significant palliation and prolongation of survival. It is less effective in patients with primary refractory NHL than in those with refractory relapsed NHL.

Resultados a largo plazo del tratamiento con quimioterapia y radioterapia en linfoma no Hodgking localizado, de grados intermedio y alto / Long term results for intermediate and high grade localized non Hodgkin lymphoma, treated with chemotherapy and radiotherapy

Background: Treatment of intermediate and high grade non-Hodgkin lymphoma (NHL) includes chemotherapy with or without radiotherapy, depending on the clinical stage. The standard treatment for advanced NHL is 8 cycles of combined chemotherapy, cyclophosphamide, adriamicin, vincristine and prednisone (CHOP). Patients presenting with localized disease are treated with fewer chemotherapy cycles and involved field radiotherapy, with good results. Aim: To evaluate the treatment results including overall survival (OS) and event-free survival (EFS) in localized aggressive NHL patients treated at the Pontificia Universidad Católica de Chile, Clinical Hospital. Patients and Methods: Retrospective analysis of all patients with Ann Arbor stages I and II referred to the hematology and radiotherapy clinic between 1998 and 2003. OS and EFS analysis was made according to the Kaplan and Meier method. Log-rank and Cox methods were used for univariate and multivariate analyses, respectively. Chemotherapy and radiotherapy toxicities were scored according to World Health Organization (WHO) and Radiation Therapy Oncology Group (RTOG) scales, respectively. Results: 39 patients (20 men), aged between 20 to 85 years, were the source for this study. The average follow-up was 51 months (range 6-115). The 5 years OS and EFS were 72,4 percent and 63,3 percent, respectively. On univariate analysis, age over 60 was the only variable that affected negatively OS and EFS. Acute toxicity caused by chemotherapy and radiotherapy was uncommon. Conclusions: Age over 60 was the only independent variable associated with poor prognosis. The number of chemotherapy cycles and the drug combination did not influence the results. These results support the usefullness of a shortened chemotherapy regimen plus involved field radiotherapy.

Experiencia de 10 años en linfomas oculares y de anexos oculares / Ten year experience with ocular lymphomas and ocular adnexae lymphomas

Antecedentes. Los linfomas oculares (LO) y de los anexos oculares (LAO) tienen una incidencia de 7- 8% de todos los tumores extraganglionares. Objetivo. Describir las características clínicas, morfológicas e inmunofenotípicas de los LO y LAO atendidos en el Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán. Material y métodos. Se revisaron los expedientes de los pacientes con diagnóstico de LO y LAO de julio de 1994 a julio del 2005. Se analizaron los siguientes datos: presentación clínica, tratamiento, respuesta al tratamiento, supervivencia global, y supervivencia libre de enfermedad (SVLE). Resultados. Se analizaron 10 pacientes, 8 mujeres y 2 hombres. La media de edad fue de 50 años. La remisión completa (RC) se presentó en 8 de 10 pacientes y en los 6 pacientes con linfoma de tejido linfoide asociado a mucosas (MALT). Se encontraron dos con enfermedad refractaria, los cuales estaban en estadio IV. Conclusiones. En este estudio los LO y LAO correspondieron a 0.02% de todos los linfomas no Hodgkin (LNH) estudiados (498 casos). Los linfomas tipo MALT se presentan a edad más avanzada, se encontraron en estadios más tempranos y en todos hubo RC, con una SVLE promedio de 868 días y una media de supervivencia de 442 días.

BACKGROUND: Ocular lymphomas and ocular adnexae lymphomas (OL and OAL) constitute 7-8% of all extranodal lymphomas. OBJECTIVE: Describe the clinical, morphologic and immunophenotypic characteristics of OAL seen in our hospital. MATERIAL AND METHODS: Retrospective analysis of patient records with OL and OAL between July 1994 and July 2005. The following data was analyzed: Clinical presentation, therapy, treatment response, overall survival and disease free survival. RESULTS: Ten patients with OL and OAL were identified. Of these, 8 were women and 2 men. Median age was 50. Eight of 10 patients achieved complete remission, 6 of the 6 presenting MALT Lymphoma. Two patients with stage IV had refractory disease. CONCLUSIONS: In our series 0.02% of lymphomas were OL and OAL of a total 498 LNH. MALT lymphomas appear at a more advanced age, sixty percent of the cases were MALT lymphomas and were diagnosed during their early stages. Patients were followed during 21 months, global survival was 100%, free illness survival had a mean of 868 days and a survival median of 442 days.

Utilidad de la deoxiglucosa marcada con flúor-18 (FDG) en el manejo de los linfomas / Role of 18Fluorine-deoxyglucose (FDG) positron emission tomography in the management of lymphomas

Positron emission tomography (PET) with fluorine-18 labeled deoxyglucose (FDG) is useful in the management of lymphomas. In this review, some general concepts of this metabolic test are defined. It has an excellent diagnostic yield in Hodgkin disease as well as in most non Hodgkin lymphomas. Staging, restaging residual mass evaluation and the control of therapy are the main indications for FDG-PET. Images with FDG have a high diagnostic and prognostic value, that is superior to anatomical images and conventional staging techniques. They are also helpful for the assessment of tumor activity in abnormal lymph nodes or large masses that have been treated and reduce their size slowly or show an incomplete resolution. Currently, the resolution of dedicated PET equipments is 6 mm and bigger lesions can easily be detected. The main differences and advantages of FDG versus gallium-67 in lymphoma are also discussed, as well as the initial local experience with the technique in lymphoma patients.

Consenso Colombiano sobre el tratamiento del linfoma folicular no Hodgkin / Colombian consensus document for the treatment of follicular non Hodgkin lymphoma

El Consenso Colombiano de Hematología Oncológica (CCHO) es un proyecto apoyado por el Instituto Nacional de Cancerología, E.S.E y por la Sociedad Colombiana de Hematología y Oncología Clínica. Su propósito es mejorar los resultados de las intervenciones realizadas en los pacientes con cáncer, ayudando a los profesionales en salud a suministrar la mejor evidencia disponible; a fin de optimizar las decisiones clínicas y promover el uso racional de los recursos.La actividad del CCHO permite desarrollar pautas para la práctica siguiendo la metodología del grupo nominal, y los informes resultantes representan la síntesis de las recomendaciones extraídas de la información recolectada por medio de búsquedas sistemáticas de la literatura médica. La aprobación de las recomendaciones por parte de los miembros del CCHO no significa necesariamente que deba ser adoptada como política; depende del lector.Se revisaron las bases Medline 1966-2005,Cochrane Library tissue 2,2005,Embase 1974-2005,Biosis 1992-2005, Lilacs 1989-2005 y otras bases de datos relevantes.Esta guía ha sido revisada y aprobada por todos los miembros del Consenso,que incluyó hematólogos, oncólogos, epidemiólogos, hematopatólogos, un especialista en políticas de salud y un miembro de la comunidad. Tres hematólogos internacionales, de manera independiente, hicieron la revisión externa del documento de resumen. El documento final del consenso requirió un proceso formal de estandarización. Será obligatoria la revisión periódica y continua de la literatura científica y, cuando se considere apropiado, se integrara la nueva información relevante al consenso original.Población: El ámbito del consenso son los pacientes adultos con diagnóstico de linfoma folicular no Hodgkin (LFNH).

La Medicina Nuclear más allá de la imágenes / The Nuclear Medicine beyond the images

La Medicina Nuclear clínica se apoya tradicionalmente en tres grandes pilares básicos, estos son las imágenes, la terapia con radionucleidos y las técnicas "in vitro". En los últimos años, tanto con el desarrollo de las imágenes moleculares como con el surgimiento de nuevas aplicaciones terapéuticas con radionucleidos, se nos abren insospechadas oportunidades para que nuestra especialidad ocupe un valioso sitial en las nuevas aplicaciones oncológicas. En este artículo se revisa nuestra experiencia en dos novedosas áreas en las cuales hemos tenido la oportunidad de desarrollarlas en nuestro centro. Estas son la terapia con 90Y-DOTATOC en tumores con sobre-expresión de receptores de somatostatina[1] (carcinoides, neuroendocrinos y otros) y el uso de 90Y-Ibritumomab-Tiuxetan en Linfomas No Hodgkin de células B con presencia de antígeno CD20+[2].

Low Grade MALT Lymphoma of the Stomach: Treatment Outcome with Radiotherapy Alone

In order to evaluate the role of radiation therapy in the management of low-grade mucosa-associated lymphoid tissue lymphoma of the stomach (MLS), in patients with no evidence of Helicobacter pylori (H. pylori) or who had not responded to H. pylori eradication treatment, we analyzed the treatment outcome of patients who had received radiotherapy alone. Between Jan 1995 and May 2001, 6 patients with low- grade MLS were treated with radiotherapy alone. The median radiation dose was 30.6 Gy (range; 30 - 39 Gy) in a daily fractions of 1.5 - 1.8 Gy. Each patient had an endoscopic esophago-gastro-duodenoscopy with biopsy, 4 weeks after the completion of radiotherapy and every 6 months thereafter. A complete response was obtained in all patients. All patients were followed-up without evidence of disease, and no patient suffered a relapse. There was neither perforation nor hemorrhage of the stomach in any of the patients. No renal or hepatic toxicity were noted, and no secondary malignancies developed. In conclusion, radiotherapy should be considered as the preferred treatment method for low-grade MLS, in patients with no evidence of H. pylori infection, or who do not respond to antibiotic therapy, due to the significant benefits in gastric preservation and low morbidity.

Clinical management of six cases of low-risk primary tonsillar non-Hodgkin's lymphoma

CONTEXT: There have been many reports that favor aggressive systemic treatment with chemotherapy and radiotherapy, even for well-localized lymphomas, avoiding the need for tonsillectomy of the normal tonsil. CASE REPORT: We report six cases of primary tonsillar lymphoma with a median patient age of 42 years. There were two lymphoma cases with diffuse large cells, two cases with mixed small and large cells, one with small cells and one indeterminate. They were treated with six cycles of chemotherapy and cervical radiotherapy. All patients achieved durable complete remission. Our data agree with previous reports that suggested that primary tonsillar high-grade B-cell NHL has a good prognosis if aggressively treated.

Linfoma no Hodgkin de células grandes no hendidas de la cavidad bucal: reporte de un caso / Big-cell non-Hodgkin lymphoma of the oral cavity: report of a case

Los Linfomas No Hodgkin de la cavidad bucal son un grupo representativo de neoplasias que tienen diversidad en antecedentes, manera de presentación, respuesta al tratamiento y pronóstico. Se expone un caso evaluado en el Servicio de Clínica Estomatológica y en el Servicio de Cirugía Bucal de la Facultad de Odontología de la U.C.V., correspondiente a un paciente masculino de 81 años, con lesión de aspecto clínico tumoral en el reborde alveolar superior izquierdo. Basado en el estudio clínico, histopatológico e inmunohistoquímico, fue diagnosticado como Linfoma No Hodgkin de Células Grandes no Hendidas. La conducta a seguir después de realizado el diagnóstico fue, referencia al Hospital Oncológico Luis Razetti en donde le planificaron una combinación de tratamientos (Quimioterapia, Radioterapia)

Linfoma mamario primario: comunicación de un caso / Primary breast lymphoma: report of a case

EL linfoma mamario es frecuente y constituye menos del 1 por ciento de las neoplasias malignas de esta glándula. La sospecha clínica es poco corriente debido a su rareza y ausencia de signos específicos. Usualmente la histopatología no es suficiente para realizar el diagnóstico definitivo, debiéndose realizar estudios inmunohistoquímicos complementarios. Comunicamos el caso de una mujer derivada desde el hospital de Punta Arenas con diagnóstico de carcinoma mamario derecho T2N, Mx con el objeto de realizar tratamiento quirúrgico y radiante. En nuestros centro se efectuó una mastectomía conservadora cuyo estudio de la pieza operatoria reveló la presencia de un linfoma mamario nodular difuso que fue tratado con el esquema de linfoma extranodal que utiliza nuestro hospital. Se analizan algunos aspectos relevantes de estas infrecuentes neoplasia maligna con especial énfasis en el diagnóstic, histopatología y terapéutica

Angiosarcomas radioinducidos / Radioinducted angiosarcomas

Se contribuye a la casuística con la presentación de 2 casos de angiosarcoma radioinducido, diagnosticados ambos, en pacientes de sexo femenino que previamente habían recibido dosis total de radioterapia, uno por presentar un linfoma tipo Hodgkin, y otro por carcinoma ductal de mama. En una de las pacientes el tiempo de latencia fue de 10 años, mientras que en la otra fue de 25 años. La evolución fue dispar, ya que mientras una tiene un año de evolución sin evidencias de enfermedad, la otra falleció debido a metástasis pulmonares y complicaciones cardíacas al año de ser operada

Non Hodgkin's lymphoma in oral-cavity-report of two cases

Non hodgkin's lymphomas are tumours of the immune system that uncommonly evolves as primary lesion of the oral cavity. Two cases of primary lymphoma in the oral cavity are presented. One case was treated by radiotherapy and the second one died because of the neoplastic infiltration to the orbit and brain. In the first case the patient survived with no recurrence of the tumour