RESUMO
Burkitt lymphoma is a non-Hodgkin B-cell lymphoma with a high prevalence in the pediatric population. Abdominal manifestations are well known in sporadic Burkitt lymphoma and vary from nonspecific symptoms to intestinal obstruction due to intussusception; however, mass-like splenic involvement has been scarcely described. OBJECTIVE: To present a case of a patient with a splenic mass whose histopathological analysis revealed Burkitt lymphoma. CLINICAL CASE: A 13-year-old female patient presented with abdominal pain, progressive weight loss, and fever. Imaging studies showed a splenic mass, intestinal thickening, and ileal intussusception. Histopathological analysis of spleen biopsy revealed Burkitt lymphoma. After the first cycle of chemotherapy (BFM95-NHL protocol), abdominal symptoms resolved; no other signs suggestive of intussusception were observed, as well as a significant reduction of the splenic mass was observed. CONCLUSIONS: Burkitt lymphoma in pediatric patients can present as a well-defined splenic tumor, causing no splenomegaly. In addition, its management does not require surgery since it can be resolved with chemotherapy.
Assuntos
Linfoma de Burkitt , Neoplasias Esplênicas , Humanos , Linfoma de Burkitt/diagnóstico , Linfoma de Burkitt/patologia , Linfoma de Burkitt/complicações , Linfoma de Burkitt/tratamento farmacológico , Feminino , Adolescente , Neoplasias Esplênicas/patologia , Neoplasias Esplênicas/diagnóstico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Intussuscepção/diagnóstico , Intussuscepção/etiologia , Dor Abdominal/etiologiaRESUMO
El linfoma es la neoplasia maligna de cabeza y cuello más común en la población pediátrica. Las anifestaciones clínicas de linfoma en amígdala palatina son generalmente asimetría amigdalina, alteración en la apariencia de la mucosa y adenopatías cervicales. Ante una hipertrofia amigdalina unilateral, se debe diferenciar de cuadros infecciosos, procesos crónicos, otros tumores o simplemente una hiperplasia amigdalina benigna. El seguimiento estricto de la asimetría amigdalina es un pilar importante a la hora del diagnóstico de los pacientes con linfoma amigdalino, debido a que un tratamiento temprano es fundamental para el pronóstico. Se presenta una niña de 14 años con hipertrofia amigdalina unilateral y odinofagia, de aproximadamente 21 días de evolución sin respuesta al tratamiento antibiótico, con diagnóstico de linfoma de Burkitt. Presenta remisión completa de la enfermedad al año del diagnóstico.
Lymphoma is the most common childhood malignancy in the head and neck. The most common clinical manifestations of lymphoma in palatine tonsil are the tonsils asymmetry, alteration in the appearance of the mucous and cervical lymphadenopathy. The unilateral tonsillar hypertrophy must be differentiated with infectious processes, with chronic processes, other tumors or benign tonsillar hyperplasia. The monitoring of tonsillar hypertrophy is strict, because early diagnosis and treatment are of great importance in the prognosis of tonsillar lymphoma patients. A 14 year-old girl presents unilateral tonsillar enlargement and odynophagia. After 21 days of antibiotic treatment, she has not clinical improvement. The diagnosis is Burkitt's lymphoma. One year after diagnosis, she presents complete remission of the disease.
Assuntos
Humanos , Feminino , Adolescente , Neoplasias Tonsilares , Linfoma de Burkitt/diagnóstico , Linfoma de Burkitt/terapiaRESUMO
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.
Assuntos
Linfoma de Burkitt , Infecções por Vírus Epstein-Barr , Adolescente , Adulto , Brasil/epidemiologia , Linfoma de Burkitt/diagnóstico , Linfoma de Burkitt/epidemiologia , Criança , Pré-Escolar , Herpesvirus Humano 4 , Humanos , Lactente , Masculino , África do Sul/epidemiologia , Adulto JovemRESUMO
Abdominal angiostrongyliasis is a parasitic zoonosis, endemic in the American continent. Its etiological agent is Angiostrongylus costaricensis, a nematode whose definitive hosts are rats and other rodents and the intermediate hosts, slugs. Mammals acquire the infection by consuming vegetables contaminated with L3 larvae. The disease shows a heterogeneous clinical spectrum and given its low incidence its diagnosis is a great challenge. In Colombia, the first case was reported in 1979 and until 1998, only five additional cases have been reported. However, in the last two decades, no new cases were reported. Here we discuss two cases of children from Huila and Caquetá departments who developed the disease. Both cases required long in-patient care and multiple surgical interventions. The diagnosis was achieved by histopathological observation of parasitic elements inside the mesenteric arteries. One of the children died while the other fully recovered. We discuss the epidemiology, pathogenic cycle, clinical presentation, diagnosis, and prevention strategies of this disease paying particular attention to our patients' features and the Colombian context.
La angiostrongiloidiasis abdominal es una zoonosis parasitaria endémica en el continente americano. Su agente etiológico es el nematodo Angiostrongylus costaricensis, cuyos huéspedes definitivos son los roedores y, los intermediarios, los caracoles y las babosas, por lo que se adquiere al consumir vegetales contaminados con larvas en estadio 3. La presentación clínica es muy variada y, dada su rareza, su diagnóstico es un desafío. En Colombia el primer caso se reportó en 1979 y, desde entonces hasta 1998, se han informado cinco casos más, aunque en las últimas dos décadas no se había reportado ningún caso. Se describen aquí dos casos de angiostrongiloidiasis en niños provenientes de los departamentos de Huila y Caquetá que requirieron una larga hospitalización y múltiples intervenciones quirúrgicas. El diagnóstico se logró al observar en los especímenes quirúrgicos larvas and huevos cuya morfología sugería una infección por nematodos; uno de los pacientes murió y el otro se recuperó satisfactoriamente. Se discuten la epidemiología, la patogenia, la presentación clínica, el diagnóstico y las estrategias de prevención de esta parasitosis, con énfasis en las características particulares de los casos descritos y en el contexto colombiano.
Assuntos
Angiostrongylus/isolamento & purificação , Enteropatias Parasitárias/diagnóstico , Infecções por Strongylida/diagnóstico , Albendazol/uso terapêutico , Angiostrongylus/crescimento & desenvolvimento , Angiostrongylus/fisiologia , Animais , Anti-Helmínticos/uso terapêutico , Apendicite/diagnóstico , Linfoma de Burkitt/diagnóstico , Criança , Pré-Escolar , Colômbia/epidemiologia , Diagnóstico Diferencial , Erros de Diagnóstico , Síndrome de Down/complicações , Eosinofilia/etiologia , Evolução Fatal , Hepatomegalia/etiologia , Humanos , Enteropatias Parasitárias/parasitologia , Enteropatias Parasitárias/patologia , Larva , Masculino , Estações do Ano , Infecções por Strongylida/epidemiologia , Infecções por Strongylida/parasitologia , Infecções por Strongylida/patologia , ZoonosesRESUMO
Introduction: Burkitt's lymphoma is a non- Hodgkin B-cell lymphoma whose cranial location is extremely rare. Clinical case: The case of a 35-year-old Peruvian man with a progressive parietal left cranial tumor is described. A biopsy with immunohistochemistry was performed for the diagnostic confirmation of Burkitt's lymphoma. First-line outpatient treatment with CODOX-M/IVAC was initiated, however, he stopped attending his treatment for 2 months. He returns, he is shown progression of the disease and is treated with rituximab, ifosfamide carboplatin and etoposide. Does not respond satisfactorily and dies. Conclusions: Burkitt's lymphoma is a very aggressive tumor that, if not treated in a timely manner, has high mortality, as happened with the patient in the case.
Introducción: El linfoma de Burkitt es un linfoma no Hodgkin de células B cuya ubicación craneal es extremadamente rara. Caso clínico: Se describe el caso de un hombre peruano de 35 años con una tumoración craneal parietal izquierda de crecimiento progresivo. Para la confirmación diagnóstica de linfoma de Burkitt se realizó una biopsia con inmunohistoquímica. Se inició tratamiento ambulatorio de primera línea con CODOX-M/IVAC, sin embargo, deja de acudir a su tratamiento por 2 meses. Retorna, se le evidencia progresión de la enfermedad y se le trata con rituximab, ifosfamida carboplatino y etopósido. No responde de forma satisfactoria y fallece. Conclusiones: El Linfoma de Burkitt es un tumor muy agresivo que si no es tratado oportunamente presenta alta mortalidad, tal como ocurrió con el paciente del caso.
Assuntos
Linfoma de Burkitt/diagnóstico , Neoplasias Cranianas/diagnóstico , Adulto , Biópsia , Evolução Fatal , Humanos , Imageamento por Ressonância Magnética , MasculinoRESUMO
The aim of this study was to integrate the available data regarding pediatric Burkitt's lymphoma (BL) of the oral cavity. A systematic review was conducted following PRISMA guidelines, through a specific search strategy. Twenty-nine publications were included in this study, resulting in a total of 144 cases. Oral BL was predominantly found in males (75.7%). The mandible was the most involved site (37.5%), and all cases clinically exhibited a swelling. Presence of EBV was observed in 33.3% of the cases, and 4 cases reported HIV-positive patients (33.3%). Chemotherapy was the leading treatment choice for oral BL (94.9%), and the overall 5-year survival was 54.3%. Regarding the quality assessment of the studies, most (19 studies; 65.5%) were classified as an overall moderate risk of bias. In conclusion, the clinicopathological characteristics of oral BL in the pediatric population comprise the sporadic and intermediate subtypes. Despite its aggressiveness, this malignancy presents a moderate overall survival.
Assuntos
Linfoma de Burkitt/diagnóstico , Linfoma de Burkitt/patologia , Neoplasias Bucais/diagnóstico , Neoplasias Bucais/patologia , Adolescente , Linfoma de Burkitt/mortalidade , Linfoma de Burkitt/terapia , Criança , Pré-Escolar , Terapia Combinada , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Neoplasias Bucais/mortalidade , Neoplasias Bucais/terapia , Prognóstico , Análise de Sobrevida , Adulto JovemRESUMO
BACKGROUND: Although the increased use of combined antiretroviral therapy (cART) has decreased the incidence of lymphomas HIV-associated, Burkitt lymphoma (BL) incidence remains stable. Reported outcomes on HIV-associated BL from developed countries seem to corroborate that the regimens do not need to be tailored to the HIV-positive population. MATERIALS AND METHODS: This is a retrospective multicenter cohort study from Brazil, including HIV-positive patients aged 15 years and above diagnosed with BL. RESULTS: A total of 54 patients were included. Median age was 39 years (range, 15-64). At diagnosis, advanced disease was found in 86% and 52% had a CD4+ count lower than 200 cells/mm3. Five patients died before starting any regimen. Among the remaining 49 patients, most were treated with Hyper-CVAD (53%) and CODOX-M IVAC (18%). Rituximab was used in frontline in only 16% of the patients. Primary refractory disease was found in 14%. A treatment-related mortality of 38.7% and a complete response rate of 44.9% were found. At 4 years, estimated overall survival (OS) was 39.8%. All relapsed and primary refractory patients eventually died. Remaining patients died from infections (24/34), despite antimicrobial prophylaxis and associated cART. CONCLUSION: Early mortality and toxicity were higher in our cohort than in developed countries. A faster diagnosis, better understanding of the biology of the disease, establishment of low toxicity regimens, inclusion of rituximab and improvement of supportive care may decrease the mortality of HIV-associated BL in developing countries.