Giant Cell tumor of the Zygomatic Arch in a Pediatric Patient: Rare Case Report / Tumor de Células Gigantes do Arco Zigomático em um Paciente Pediátrico: Relato de Caso Raro / Tumor de Células Gigantes del Arco Cigomático en un Paciente Pediátrico: Informe de un Caso Raro

Introduction: A giant cell tumor (GCT) is a rare tumor, usually benign, which can be locally aggressive, with an almost unknown incidence in the skull. Case report: Case report of a pediatric patient diagnosed with Ewing's sarcoma (ES) and later GCT when sought medical care for an increased volume in the zygomatic arch and the right preauricular region. Imaging exams showed an expansive lesion in the zygomatic and squamous portion of the right temporal bone, with an extradural intracranial component in the middle fossa, with no evidence of infiltration. From the anatomopathological view, fusocelullar neoplasia was seen. The CD68 marker was positive and the S100 marker was negative. Tumor resection by microsurgery was performed. The patient remains in follow-up, with imaging exams at neurosurgery and orthopedics, the latest magnetic resonances of the skull showing a reduction in the size of the lesion. In addition, the patient did not submit to adjuvant therapy and is currently without complaints. Conclusion: This is an important case due to its rarity and clinical data that contributes for the understanding of the pathologies presented, allowing that, in the future, new studies are able to optimize the treatment, and the prognosis for these patients

Introdução: O tumor de células gigantes (TCG) é raro, geralmente benigno, e pode ser localmente agressivo, com incidência quase desconhecida no crânio. Relato do caso: Paciente pediátrico com diagnóstico de sarcoma de Ewing (SE) e posteriormente de TCG, quando procurou atendimento médico por conta do aumento de volume em arco zigomático e região préauricular à direita. Os exames de imagem evidenciaram lesão expansiva em porção zigomática e escamosa do osso temporal à direita, com componente intracraniano extradural em fossa média, sem evidências de infiltração. Do ponto de vista anatomopatológico, observou-se neoplasia fusocelular. O marcador CD68 foi positivo e o marcador S100, negativo. Realizouse microcirurgia para ressecção do tumor. O paciente permanece em acompanhamento com exames de imagem nos serviços de neurocirurgia e ortopedia, com as últimas ressonâncias magnéticas de crânio mostrando redução no tamanho da lesão. Além disso, não realiza terapia adjuvante e atualmente não apresenta queixas. Conclusão: Este é um caso importante em razão da sua raridade e dos dados clínicos que agregam informações sobre as patologias apresentadas, permitindo que, no futuro, novas pesquisas possam otimizar o tratamento da referida neoplasia e o prognóstico desses pacientes

Introducción: El tumor de células gigantes (TCG) es un tumor raro, generalmente benigno, que puede ser localmente agresivo, con una incidencia casi desconocida en el cráneo. Relato del caso: Paciente pediátrico diagnosticado con sarcoma de Ewing (SE) y posteriormente con TCG, cuando procuró atención médica por aumento de volumen en arco cigomático y región preauricular derecha. Los exámenes de imagen mostraron una lesión expansiva en la porción cigomática y escamosa del temporal derecho, con componente intracraneal extradural en la fosa media, sin evidencias de infiltración. Desde el punto de vista anatomopatológico, se observó neoplasia fusocelular. El marcador CD68 fue positivo y el marcador S100 negativo. Se realizó microcirugía para resecar el tumor. El paciente permanece en seguimiento con exámenes de imagen en los servicios de neurocirugía y ortopedia, con las últimas resonancias magnéticas de cráneo mostrando una reducción en el tamaño de la lesión. Además, no se somete a terapia adyuvante y actualmente no tiene quejas. Conclusión: Este es un caso importante por su rareza y datos clínicos que agregan informaciones sobre las patologías presentadas, permitiendo, en el futuro, nuevas investigaciones para optimizar el tratamiento de la neoplasia referida, y el pronóstico de estos pacientes

Improvement of Rheumatic Valvular Heart Disease in Patients Undergoing Prolonged Antibiotic Prophylaxis.

Secondary prophylaxis of rheumatic heart diseases is efficient in reducing disease recurrence, heart damage, and cardiac impairment. We aimed to monitor the clinical evolution of a large Brazilian cohort of rheumatic patients under prolonged secondary prophylaxis. From 1986 to 2018, a cohort of 593 patients with rheumatic fever was followed every 6 months by the Reference Center for the Control and Prevention of Rheumatic Fever and Rheumatic Cardiopathy (CPCFR), Paraná, Brazil. In this cohort, 243 (41%) patients did not present cardiac damage (group I), while 350 (59%) were diagnosed with rheumatic heart disease (RHD) (group II) using the latest case definition. Among group II, 233 and 15 patients had impairment of the mitral and aortic valves, respectively, while 102 patients had impairment of both valves. Lesions on the mitral and aortic valves presented a regression in 69.9 and 48.7% of the patients, respectively. Active patient recruitment in the reference center and early detection of oropharyngeal GAS were important factors for optimal adherence to the prophylactic treatment. Patients with disease progression were associated with noncompliance to secondary prophylaxis. No patients undergoing regular prophylaxis presented progression of the rheumatic cardiac disease. Eighteen valvular surgeries were performed, and four (0.7%) patients died. This study confirmed that tailored and active efforts invested in rheumatic heart disease secondary prevention allowed for significant clinical improvement.

Schwanoma Maligno de Nervo Periférico Metastático para Pulmão e Glândula Adrenal: Relato de Caso / Metastatic Peripheral Nerve Malignant Schwannoma of Lungs and Adrenal Gland: Case Report / Schwannoma Maligno Metastásico de Nervio Periférico para Pulmones y Glándula Suprarrenal: Reporte de Caso

Introdução: O schwanoma é um tumor pouco frequente que se origina das células de Schwann que recobrem os prolongamentos nervosos. A malignidade desses tumores é ainda mais rara, correspondendo a 5-10% de todos os sarcomas. O prognóstico é reservado, mesmo após completa ressecção cirúrgica. Relato do caso: Paciente do sexo masculino, 79 anos, apresentou-se com lesão crostosa em perna esquerda com dois meses de evolução. A biópsia da lesão inicialmente revelou sarcoma pleomórfico, mas seu padrão imuno-histoquímico confirmou tumor maligno da bainha do nervo periférico. O tumor desenvolveu metástase linfonodal, pulmonar e adrenal dentro de poucos anos após o diagnóstico inicial. A ressecção do tumor adrenal não foi possível. O paciente desenvolveu síndrome paraneoplásica e teve piora do estado clínico, evoluindo a óbito. Conclusão: Tendo em vista a forma inespecífica do aparecimento do tumor e sua agressividade, é importante ressaltar o papel da ressecção cirúrgica ampla no seu tratamento. Além disso, fica evidente a necessidade de novos relatos desse tipo de neoplasia para melhor definição de uma conduta mais apropriada, principalmente em casos de recorrência ou disseminação metastática

Introduction: Schwannoma is a rare type of tumor which grows from Schwann cells, that protect nerve extensions. The malignancy of these tumors is even rarer, corresponding to 5-10% of all sarcomas. The prognosis is dismal, even after complete surgical resection. Case report: A 79-year-old male patient presented with a crusted lesion in his left leg with two months of evolution. Initially, the biopsy of the lesion revealed pleomorphic sarcoma, but its immunohistochemical pattern was peripheral nerve sheath malignant tumor. The tumor developed lymph node, lung, and adrenal metastasis within a few years after the first diagnosis. Adrenal tumor resection was not possible. The patient developed paraneoplastic syndrome and his clinical status worsened, progressing to death. Conclusion: In view of the nonspecific form of tumor appearance and its aggressiveness, it is important to highlight the role of wide surgical resection in its treatment. In addition, it is clear that new case reports of this type of neoplasia are necessary for better definition of more appropriate conducts, especially in recurrence or metastatic cases

Introducción: El schwannoma es un poco común, el cual se origina en las células de Schwann, que cubren las extensiones nerviosas. La malignidad de estos tumores es aún más rara, y representa 5-10% de todos los sarcomas. El pronóstico es reservado, incluso después de una resección quirúrgica completa. Relato del caso: Varón de 79 años que presenta una lesión costrosa en la pierna izquierda a los dos meses de evolución. La biopsia de la lesión reveló inicialmente un sarcoma pleomórfico, pero su patrón inmunohistoquímico era un tumor maligno de la vaina del nervio periférico. El tumor desarrolló metástasis en los ganglios linfáticos, pulmones y suprarrenales pocos años después del diagnóstico. La resección del tumor suprarrenal no fue posible. El paciente desarrolló síndrome paraneoplásico y empeoró el estado clínico, progresando hasta la muerte. Conclusión: Dada la forma inespecífica de aparición del tumor y su agresividad, es importante destacar el papel de la resección quirúrgica amplia en su tratamiento. Además, existe una clara necesidad de nuevos informes de este tipo de neoplasias para definir mejor un abordaje más adecuado, especialmente en casos de recurrencia o diseminación metastásica

Epidemia de nefrite por Streptococcus equi subsp. zooepidemicus: estudo de caso-controle no município de Monte Santo de Minas, Minas Gerais, 2013 / Outbreak of nephritis by Streptococcus equi subspecies zooepidemicus: case-control study in the municipality of Monte Santo de Minas, Minas Gerais, Brazil, 2013 / Epidemia de nefritis por Streptococcus equi subsp. zooepidemicus: estudio de casos y controles en Monte Santo de Minas, Minas Gerais, Brasil, 2013

OBJETIVO: investigar a epidemia de nefrite por Streptococcus zooepidemicus em Monte Santo de Minas, Minas Gerais, Brasil. MÉTODOS: realizou-se estudo de caso-controle e tentativa de isolamento do agente, no período de janeiro a abril de 2013, utilizando-se dados clínicos, laboratoriais, de entrevistas e de inspeções. RESULTADOS: houve 417 casos notificados e 175 (42,0%) confirmados, dos quais 90,9% residiam no município e 67,4% eram do sexo feminino, com idade mediana de 36 anos; 24% dos casos foram hospitalizados; três sorveterias do município utilizavam leite do laticínio A; houve associação significativa entre adoecimento e consumo do leite A (odds ratio [OR]=4,16/IC95%: 1,55;11,18), um dos sorvetes feito com esse leite (OR=3,09/IC95%: 1,39;6,86) e milk shake de leite não industrializado (OR=3,25/IC95%: 1,13;9,36); não se detectou a bactéria em propriedades rurais. CONCLUSÃO: a epidemia de nefrite por Streptococcus zooepidemicus foi associada ao consumo de leite e derivados.

OBJECTIVE: to investigate an outbreak of nephritis by Streptococcus zooepidemicus in Monte Santo de Minas, Minas Gerais State, Brazil. METHODS: a case-control study and attempt to isolate the bacterial agent were carried out from January to April 2013, using clinical and laboratory data, interviews and inspections. RESULTS: 417 suspected cases were reported, of which 175 (42.0%) were confirmed; 90.9% lived in that municipality, of which 67.4% were female, with median age of 36 years; 24% of cases were hospitalized; three ice cream shops in the municipality used type A milk; there was significant association between the illness and the consumption of type A milk (odds ratio [OR]=4.16/95%CI: 1.55;11.18), one of the ice cream made with this milk (OR=3.09/95%CI: 1.39;6.86) and milk shake of non-processed milk (OR=3.25/95%CI: 1.13;9.36); the bacterium was not detected in rural properties. CONCLUSION: the outbreak of nephritis by Streptococcus zooepidemicus was associated with the consumption of milk and dairy products.

OBJETIVO: investigar la epidemia de nefritis por Streptococcus zooepidemicus en Monte Santo de Minas, Minas Gerais, Brasil. MÉTODOS: fue realizado un estudio de casos y controles e intento de aislamiento del agente, entre enero y abril de 2013, con datos clínicos y de laboratorio, entrevistas e inspecciones. RESULTADOS: fueron notificados 417 casos sospechoso, de los cuales, 175 confirmados; 90,9% eran residentes del municipio, 67,4% de sexo femenino, con mediana de edad de 36 años; 24% casos fueron hospitalizados; tres heladerías del municipio utilizaban leche del lacticinio A; encontramos asociación significativa entre la enfermedad y el consumo de leche A (odds ratio [OR]=4,16/IC95%: 1,55;11,18), en helados hechos con leche (OR=3,09/IC95%: 1,39;6,86) y milk shake de leche no industrializada (OR=3,25/IC95%: 1,13;9,36); la bactéria no fue aislada en zonas rurales. CONCLUSIÓN: la epidemia de nefritis por Streptococcus zooepidemicus se asoció con el consumo de leche y derivados.

[Outbreak of nephritis by Streptococcus equi subspecies zooepidemicus: case-control study in the municipality of Monte Santo de Minas, Minas Gerais, Brazil, 2013]. / Epidemia de nefrite por Streptococcus equi subsp. zooepidemicus: estudo de caso-controle no município de Monte Santo de Minas, Minas Gerais, 2013.

OBJECTIVE: to investigate an outbreak of nephritis by Streptococcus zooepidemicus in Monte Santo de Minas, Minas Gerais State, Brazil. METHODS: a case-control study and attempt to isolate the bacterial agent were carried out from January to April 2013, using clinical and laboratory data, interviews and inspections. RESULTS: 417 suspected cases were reported, of which 175 (42.0%) were confirmed; 90.9% lived in that municipality, of which 67.4% were female, with median age of 36 years; 24% of cases were hospitalized; three ice cream shops in the municipality used type A milk; there was significant association between the illness and the consumption of type A milk (odds ratio [OR]=4.16/95%CI: 1.55;11.18), one of the ice cream made with this milk (OR=3.09/95%CI: 1.39;6.86) and milk shake of non-processed milk (OR=3.25/95%CI: 1.13;9.36); the bacterium was not detected in rural properties. CONCLUSION: the outbreak of nephritis by Streptococcus zooepidemicus was associated with the consumption of milk and dairy products.

Group a streptococcus antibiotic resistance in southern Brazil: a 17-year surveillance study.

BACKGROUND: Scarce data are available about the antimicrobial resistance of Group A Streptococcus in South America. METHODS: This study evaluated the antimicrobial susceptibility profile of 1,112 isolates of Group A Streptococcus during the period from 1993 to 2009 in Curitiba city, Brazil. Macrolide-resistant isolates were characterized by emm typing and pulsed-field gel electrophoresis. RESULTS: All isolates were susceptible to penicillin, vancomycin, and tigecycline. On the contrary, 18.6% of the isolates were resistant to tetracycline, presenting a minimum inhibitory concentration (MIC)(50)/MIC(90) of 32/64 mg/L. Erythromycin resistance rose from 1.9% before 2000 to 4% after 2000 and was associated with a marked increased of MIC levels. Simultaneously, both the phenotype and genotype of macrolide resistance were modified as the M phenotypes (mef(A) genotype) were replaced by the cMLS(B) phenotypes (erm(B) genotype). CONCLUSION: This polyclonal spreading of cMLS(B) macrolide resistance has not been previously observed in South America and should stimulate further epidemiological surveillance in this part of the world.

An outbreak of Streptococcus dysgalactiae subsp equisimilis in a hospital in the south of Brazil

The beta-hemolytic group C streptococci (Lancefield's group) has been considered an emergent human pathogen, showing an important role as an opportunist agent, being responsible for nosocomial infections and outbreaks. This study is reporting the first outbreak of nosocomial infection caused by Streptococcus dysgalactiae subsp. equisimilis in Brazil. From January, 2002, to December, 2004, S. equisimilis was isolated in 67/207 (32.37 percent) samples from secretions of patients' infected wounds, interned at the Hospital of Sanitary Dermatology in the State of Paraná (HDSPR). The prevalence of this microorganism increased from 11/42 (26.19 percent) in 2002, 14/65 (21.54 percent) in 2003 to 42/100 (42.00 percent) in 2004. This increase was statistically significant (p=0.024), and this microorganism became the most frequently isolated in these patients, overtaking the rates of isolation of Pseudomonas aeruginosa. The S. equisimilis grew in pure culture, as a unique microorganism, in six samples (2.9 percent) out of 207. Fresh feces of 15 animals (horses and sheep) living in the proximities of the hospital were also examined and three of them positive for S. equisimilis. The biochemical profile of the strains isolated from the patients and from the animals was the same. These animals might have been the source of the dissemination of the outbreak in the hospital. New studies will be necessary to confirm the genetic relationship between the strains isolated from patients and animals.

O estreptococo beta-hemolítico do grupo C de Lancefield tem sido considerado patógeno humano emergente, mostrando importante papel como agente oportunista, implicado algumas vezes em infecções hospitalares e surtos. Este estudo está relatando o primeiro surto de infecção hospitalar causado pelos Streptococcus dysgalactiae subsp. equisimilis no Brasil. De janeiro de 2002 a dezembro de 2004, isolou-se S. equisimilis em 67/207 (32,37 por cento) das amostras de secreções de lesões de feridas coletadas de pacientes internados no Hospital de Dermatologia Sanitária do Paraná (HDSPR). A prevalência deste microrganismo aumentou de 11/42 (26,19 por cento) em 2002, 14/65 (21,54 por cento) em 2003 para 42/100 (42,00 por cento) em 2004. Este aumento foi estatisticamente significante (p=0.024), tornando este microrganismo o mais freqüentemente isolado nos pacientes, ultrapassando as taxas de isolamento de Pseudomonas aeruginosa. Em seis amostras (2,9 por cento) entre as 207 examinadas, S. equisimilis cresceu em cultivo puro, como único microrganismo. Também foram examinadas fezes frescas de 15 animais (cavalos e ovelhas) que vivem nas proximidades do hospital, e três amostras foram positivas para S. equisimilis. O perfil bioquímico encontrado entre os isolados dos pacientes foi o mesmo encontrado entre os isolados das fezes dos animais. Acredita-se que estes animais possam ter sido a fonte de disseminação do surto no hospital. Novos estudos serão necessários para confirmar o relacionamento genético entre os isolados dos pacientes e animais.