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Abstract Ewing sarcoma is the most common malignant bone tumor of the pelvis in children and young adults. Even with aggressive treatment, its survival rate is amongst the poorest. Classical presentation may not be the rule. It may simulate clinically, imagiologically and histopathologically other nonmalignant entities. Therefore, its suspicion should not be overlooked. We report two cases of pelvic Ewing sarcoma: the first mimicking eosinophilic granuloma, and the second mimicking osteomyelitis. In the latter, we also report an atypical finding of its natural history: an initial response to antibiotic and anti-inflammatory treatment. In both cases, we highlight the possibility of an inconclusive percutaneous bone biopsy and the importance of immunochemistry and cytogenetics for the definitive diagnosis.
Resumo O sarcoma de Ewing é o tumor ósseo maligno da pelve mais comum em crianças e adultos jovens. Mesmo com tratamento agressivo, sua taxa de sobrevivência está entre as piores. A apresentação clássica pode não ser a regra. Ele pode simular clinicamente, imaginologicamente e histopatologicamente outras entidades não malignas. Portanto, sua suspeita não deve ser negligenciada. Relatamos dois casos de sarcoma pélvico: o primeiro imitando granuloma eosinofílico e o segundo imitando osteomielite. Neste último, também relatamos um achado atípico de sua história natural: uma resposta inicial ao antibiótico e ao tratamento anti-inflamatório. Em ambos os casos, destacamos a possibilidade de uma biópsia óssea percutânea inconclusiva e a importância da imunoquímica e da citogenética para o diagnóstico definitivo.
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Humanos , Masculino , Adulto , Osteomielite , Neoplasias Pélvicas , Sarcoma de Ewing , Anti-Inflamatórios não Esteroides , Doença de KimuraRESUMO
El sarcoma de Ewing es una neoplasia de hueso y tejidos blandos, cuyo manejo se relaciona con toxicidad hematológica. Este aspecto representa un desafío médico y ético en los pacientes testigos de Jehová quienes, por sus creencias religiosas, rechazan la aplicación de hemoderivados, con riesgo de que se descontinúe la quimioterapia o de que se utilicen dosis subóptimas. Se presenta el caso de una mujer colombiana de 34 años, testigo de Jehová, con diagnóstico de sarcoma de Ewing con estadificación clínica IIB (T1N0M0) en las regiones maxilar y mandibular izquierdas, tratada con quimioterapia, quien presentó un valor mínimo de hemoglobina de hasta 4,5 g/dl y tuvo indicación quirúrgica como parte del tratamiento. En estos pacientes, la decisión de practicar una transfusión comprende implicaciones éticas que requieren alternativas terapéuticas y un abordaje multidisciplinario.
Ewing's sarcoma is a bone and soft tissue neoplasm, whose management is related to hematological toxicity. This aspect represents a medical and ethical challenge in Jehovah's Witnesses patients, who, due to their religious beliefs, reject the blood component transfusion, with the risk of discontinuing chemotherapy or using suboptimal doses. We present the case of a 34-year-old Colombian woman, Jehovah's Witness, diagnosed with Ewing's sarcoma with clinical stage IIB (T1N0M0) in the left maxillary and mandibular regions, treated with chemotherapy, who presented a hemoglobin nadir of up to 4.5 g/dL, and surgical indication as part of the treatment. In these patients, the transfusion decision has ethical implications that require therapeutic alternatives and a multidisciplinary approach.
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Sarcoma de Ewing , Transfusão de Componentes Sanguíneos , Tratamento Farmacológico , AnemiaRESUMO
Sarcoma de Ewing extraesquelético (EES) é tumor raro e agressivo, acomete mais homens entre 10-20 anos e representa 25% dos sarcomas de Ewing. Caso relatado pelo diagnóstico prévio de lipoma de crescimento acelerado, destacando a importância do diagnóstico histopatológico. Masculino, 14 anos, apresentando tumoração de 10x10cm no ombro direito há um ano, com ultrassonografia sugestiva de lipoma. Realizada exérese da lesão, sendo confirmado EES pela imuno-histoquímica. O diagnóstico de EES em jovens é desafiador frente a outras tumorações subcutâneas, sendo a histopatologia imprescindível. A rápida progressão do tumor e os elevados índices metastáticos evidenciam a importância da terapêutica precoce.
Extraskeletal Ewing sarcoma (EES) is a rare tumor that affects men between 10-20 years old and represents 25% of Ewing sarcomas. We report a case due to the previous diagnosis of a fast-growing lipoma to highlight the importance of histopathological diagnosis. A 14-year-old boy presented 10x10 cm tumor in the right shoulder for a year with ultrasonography suggesting lipoma. The lesion was excised and EES was confirmed by immunohistochemical analysis. EES diagnosis in young people is challenging compared to subcutaneous tumors, and histopathology is essential. The rapid progression of the tumor and high metastatic rates highlight the significance of early treatment.
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Ewing sarcoma in the head and neck is rare, and metastasis from other bones to the mandible accounts for 0.7% of cases. This report presents a case of oral metastasis in a 24-year-old male patient diagnosed with Ewing sarcoma of the femur (p53 gene mutation and EWSR1-ERG fusion). The chief complaint was numbness in the mandible and pain for 1 month and a hardened, ulcerated exophytic lesion in the right retromolar region. Imaging exams revealed an unspecified thinning of the cortical bone of the inferior alveolar canal in the right mandibular ramus, associated with erosion of the alveolar bone. Histopathological analysis confirmed metastasis of Ewing sarcoma. The patient presented an aggressive disease progression and died 1 month after the oral diagnosis. It is important to recognize the signs and symptoms compatible with rare clinical outcomes, leading to an early diagnosis that can improve patients' quality of life and survival.
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The use of positron emission tomography with computed tomography (PET/CT) for adult cancer patients is widespread, however, its use in pediatric patients is limited by fear of radiation, monetary cost and lack of awareness of its greater sensitivity in the evaluation of some types of tumors. Ewing's sarcoma is one of the primary pediatric malignancies in which PET/CT with 18F-labeled fluorodeoxyglucose (18F-FDG) has demonstrated greater sensitivity in the evaluation of bone metastases compared to scintigraphy, as well as in the evaluation of treatment response. We report a 13 years old female consulting for retrosternal pain. A chest CT scan showed an infiltrating mass originating in the sternum. A biopsy confirmed the presence of an Ewing sarcoma. The tumor was staged with PET/CT which showed multiple bone lesions not visible in previous studies.
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Adolescente , Feminino , Humanos , Sarcoma de Ewing , Neoplasias Ósseas , Sarcoma de Ewing/patologia , Sarcoma de Ewing/diagnóstico por imagem , Neoplasias Ósseas/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Sensibilidade e Especificidade , Compostos Radiofarmacêuticos , Fluordesoxiglucose F18 , Tomografia por Emissão de Pósitrons , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Estadiamento de NeoplasiasRESUMO
RESUMEN Fundamento: los tumores óseos algunas veces son difíciles de diagnosticar, estos se dividen en benignos y malignos, los benignos son lo más frecuente y entre de ellos el osteocondroma es el principal. Los malignos a pesar de su baja frecuencia tienen importancia por su alta mortalidad, que varía entre 80 a 95 %, y las graves secuelas físicas y emocionales que acarrea su tratamiento en los pacientes. Los tipos de tumor óseo maligno más frecuente son el sarcoma de Ewing y el sarcoma osteogénico. Objetivo: profundizar los conocimientos de los tumores óseos en edad pediátrica. Métodos: la búsqueda de la información se realizó en un periodo de cinco meses (primero de agosto de 2017 al 31 de diciembre de 2017) y se emplearon las siguientes palabras claves: bone neoplasms y Childhood, a partir de la información obtenida se realizó una revisión bibliográfica de un total de 246 artículos publicados en las bases de datos PubMed, Hinari, Medline y SciELO y mediante el gestor de búsqueda y administrador de referencias EndNote, de ellos se utilizaron 50 citas seleccionadas para realizar la revisión, 40 de ellas de los últimos cinco años, donde se incluyeron dos libros. Resultados: se abordan formas de presentación clínica de los tumores óseos y los estudios de tipo imagenológicos, de laboratorio e histológicos. Se describen modalidades de tratamiento tanto conservador como quirúrgico. Conclusiones: los tumores óseos benignos en edad pediátrica son más frecuente y en ellos predomina los osteocondroma y entre los malignos el tumor de Ewing y el sarcoma osteogénico.
ABSTRACT Background: bone tumors are sometimes difficult to diagnose, they are divided into benign and malignant, benign tumors are the most frequent and among them the Osteochondroma is the main one. The malignant ones in spite of their low frequency are important for their high mortality, which varies between 80 to 95%, and the serious physical and emotional consequences that their treatment brings to patients. The most common malignant bone tumor types are Ewing's sarcoma and osteogenic sarcoma. Objective: to deepen the knowledge of bone tumors in pediatric age. Methods: the search of the information was made in a period of five months (from August 1st, 2017 to December 31st, 2017) and the following key words were used: bone neoplasms and Childhood, from the information obtained a bibliographical review of a total of 246 articles published in PubMed, Hinari, Medline, and SciELO databases was made through the EndNote search manager and reference manager, of which 50 selected citations were used to perform the review, 40 of them from the last five years, where two books were included. Results: forms of clinical presentation of bone tumors and imaging, laboratory and histological studies are addressed. Both conservative and surgical treatment modalities are described. Conclusions: benign osseous tumors in the pediatric age are more frequent and osteochondroma predominates in them and malignant tumors include Ewing tumor and osteogenic sarcoma.
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ABSTRACT BACKGROUND: The aim here was to elucidate the current survival condition of patients diagnosed with Ewing's sarcoma of the bones and joints and determine independent risk factors associated with the prognosis. DESIGN AND SETTING: Retrospective cohort study based on the Surveillance, Epidemiology and End Results (SEER) database in the United States. METHODS: We identified 397 patients who were diagnosed with Ewing's sarcoma of the bones and joints between January 2004 and December 2013. The multivariate Cox proportional hazards model was used to determine factors associated with the risk of death by adjusting for various factors. RESULTS: The one, two and five-year disease-specific survival rates were 89.08%, 78.08% and 62.47%, respectively. The factors related to death were age (≥ 18 years versus < 18 years; hazard ratio, HR = 1.77; 95% confidence interval, CI: 1.38-2.31); tumor site (extremity versus spine and pelvis; HR = 2.03; 95% CI: 1.31-2.62); tumor size (> 10 cm versus ≤ 10 cm; HR = 1.78; 95% CI: 1.34-2.56); and type of treatment (surgery alone versus radiotherapy with surgery; HR = 0.51; 95% CI: 0.38-0.89; or radiotherapy alone versus radiotherapy with surgery; HR = 1.61; 95% CI: 1.10-2.39; or no treatment versus radiotherapy with surgery; HR = 1.86; 95% CI: 1.23, 2.58). CONCLUSIONS: Patients with Ewing's sarcoma showed poor survival in situations of age ≥ 18 years, tumor size > 10 cm, receiving radiotherapy alone and receiving no treatment. Patients undergoing surgery alone had better survival.
Assuntos
Humanos , Masculino , Feminino , Adolescente , Sarcoma de Ewing/mortalidade , Neoplasias Ósseas/mortalidade , Prognóstico , Brasil/epidemiologia , Métodos EpidemiológicosRESUMO
El sarcoma de Ewing forma parte de una familia de tumores que se caracterizan por presentar translocaciones que involucran al gen EWS y algún miembro de la familia ETS que posee un dominio de unión al ADN. Se presenta el caso de un paciente de dos años de edad con una masa cervical de crecimiento rápido que por compresión local comprometió estructuras nerviosas manifestándose inicialmente con un retardo en el neurodesarrollo. Se diagnosticó Sarcoma de Ewing/Tumor neuroectodérmico primitivo por biopsia. Este es un tipo de tumor raro con una presentación inusual a nivel cervical; el cual debe tenerse en cuenta al momento de evaluar pacientes con masas cervicales en especial las de crecimiento rápido con el fin de dar un tratamiento preciso y oportuno.
Ewing's sarcoma is part of a family of tumors that is characterized by translocations that involve the EWS gene and a member of the ETS family that has a DNA binding domain. The case of a two-year-old patient who was admitted in our institution because of a rapidly growing cervical mass associated to neurodevelopment setback and functional gradual loss due to nerve compression. Ewing's sarcoma / primitive neuroectodermal tumor was diagnosed by biopsy. This is a rare type of tumor with an unusual presentation in this location; which should be taken into account when assessing a patient with cervical masses, especially those of rapid growth in order to provide an accurate and opportune treatment for improving outcomes.
Assuntos
Humanos , Sarcoma de Ewing , Neoplasias de Tecidos Moles , Tumores Neuroectodérmicos Primitivos Periféricos , Neoplasias de Cabeça e PescoçoRESUMO
Fundamento: los tumores óseos malignos en el niño son bastante raros. Comparado con el resto de los tumores infantiles ocupa solo un 3 a 4 %. A pesar de su baja frecuencia tienen importancia por su alta mortalidad, que varía entre 80 a 95 %, y las graves secuelas físicas y emocionales que acarrea su tratamiento en los pacientes. Los tipos de tumor óseo maligno más frecuente a esta edad son el sarcoma de Ewing y el Sarcoma osteogénico. Objetivo: determinar cuáles son los tumores óseas malignos más frecuentes con un estudio de cinco años de los casos ingresados en el Hospital Pediátrico Universitario Eduardo Agramonte Piña de Camagüey en menores de 19 años. Métodos: se realizó un estudio observacional, descriptivo, longitudinal y retrospectivo a través de la revisión del archivo de anatomía patología del Hospital Pediátrico Universitario Eduardo Agramonte Piña de Camagüey. Se estudiaron todos los casos con diagnóstico histopatológico de tumor óseo maligno desde el 1ro de enero 2012 a 31 de diciembre de 2016, con edades inferiores a los 19 años. La fuente de información fue el libro de registro de biopsias del departamento de Anatomía Patológica, los datos obtenidos se compilaron en un formulario elaborado para los mismos. El análisis estadístico se realizó con el paquete estándar profesional S.P.S.S versión 21 2012. Los resultados se presentaron en tablas. Resultados: los tumores óseos malignos de mayor incidencia fueron el sarcoma de Ewing y el Sarcoma osteogénico. El grupo de edad más frecuente de tumores óseos fue entre 10 a 14 años. Conclusiones: el resultado del estudio es comparable con lo reportado en la bibliografía médica donde los tumores óseos malignos, el sarcoma de Ewing y el sarcoma osteogénico son los másfrecuentes.
Background: malignant bone tumors in children are quite rare. When compared to the rest of the childhood tumors it occupies only 3 to 4%. Despite their low frequency, they are important because of their high mortality, ranging from 80 to 95%, and the serious physical and emotional sequelae that their treatment entails in patients. The most frequent types of malignant bone tumor at that age are Ewing's sarcoma and osteogenic sarcoma. Objective: to determine the most frequent malignant bone tumors with a five-year study of the cases admitted to the Eduardo Agramonte Piña University Pediatric Hospital in Camagüey under the age of 19 years. Methods: an observational, descriptive, longitudinal and retrospective study was carried out through a review of the anatomy pathology file of the Eduardo Agramonte Piña University Pediatric Hospital in Camagüey. We studied all cases with histopathological diagnosis of malignant bone tumor from January 1, 2012 to December 31, 2016, with ages below 19 years. The source of information was the biopsy record book of the Department of Pathology. The data obtained were compiled in a form prepared for them. Statistical analysis was performed using the standard professional package S.P.S.S version 21 2012. The results were presented in tables. Results: Ewings sarcoma and osteogenic sarcoma were the most frequent malignant tumors. The most frequent age group of bone tumors was between 10 and 14 years. Conclusions: the results of the study are comparable to those reported in the medical literature where malignant bone tumors, Ewing's sarcoma and osteogenic sarcoma are the most common.
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OBJECTIVE: To evaluate the performance of magnetic resonance imaging (MRI) in detecting periosteal reactions and to compare MRI and conventional radiography (CR) in terms of the classification of periosteal reactions. MATERIALS AND METHODS: Retrospective study of 42 consecutive patients (mean age, 22 years; 20 men) with a confirmed diagnosis of osteosarcoma or Ewing's sarcoma, MRI and CR images having been acquired pretreatment. Three blinded radiologists detected periosteal reactions and evaluated each periosteal reaction subtype in CR and MRI images: Codman's triangle; laminated; and spiculated. The CR was used as a benchmark to calculate the diagnostic performance. We used the kappa coefficient to assess interobserver reproducibility. A two-tailed Fisher's exact test was used in order to assess contingency between CR and MRI classifications. RESULTS: In the detection of periosteal reactions, MRI showed high specificity, a high negative predictive value, and low-to-moderate sensitivity. For CR and for MRI, the interobserver agreement for periosteal reaction was almost perfect, whereas, for the classification of different subtypes of periosteal reaction, it was higher for the Codman's triangle subtype and lower for the spiculated subtype. There was no significant difference between MRI and CR in terms of the classifications (p < 0.05). CONCLUSION: We found no difference between MRI and CR in terms of their ability to classify periosteal reactions. MRI showed high specificity and almost perfect interobserver agreement for the detection of periosteal reactions. The interobserver agreement was variable for the different subtypes of periosteal reaction.
OBJETIVO: Avaliar o desempenho da ressonância magnética (RM) na detecção de reação periosteal e comparar a classificação de presença ou ausência de reações periosteais entre a RM e a radiografia convencional (RC). MATERIAIS E MÉTODOS: Estudo retrospectivo incluindo 42 pacientes consecutivos (idade média, 22 anos; 20 homens) com diagnóstico confirmado de osteossarcoma ou sarcoma de Ewing, tendo exames de RM e RC adquiridos pré-tratamento. Três radiologistas avaliaram às cegas a presença ou ausência de reação periosteal e de cada subtipo de reação periosteal nas imagens de RC e RM: triângulo de Codman, multilamelada e espiculada. A RC foi usada como padrão de referência para cálculo do desempenho diagnóstico. Foi utilizado o coeficiente kappa para reprodutibilidade interobservador. Adicionalmente, foi realizado teste exato de Fisher bicaudal para avaliar se houve diferença significativa entre as leituras da RC e RM. RESULTADOS: A RM mostrou alta especificidade, alto valor preditivo negativo e baixa sensibilidade na detecção de reação periosteal. A concordância interobservador para a reação periosteal foi quase perfeita para a RC e RM. A concordância interobservador para a classificação dos diferentes subtipos de reação periosteal foi maior para o subtipo triângulo de Codman e menor para o subtipo espiculada. Não houve diferença na detecção por RM e RC (p < 0,05). CONCLUSÃO: Não houve diferença significativa entre as classificações da presença ou ausência de reações periosteais entre os métodos RC e RM. A RM apresentou alta especificidade e concordância interobservador quase perfeita para a detecção de reação periosteal. A concordância interobservador para os diferentes subtipos de reação periosteal foi variável.
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Abstract Objective: To evaluate the performance of magnetic resonance imaging (MRI) in detecting periosteal reactions and to compare MRI and conventional radiography (CR) in terms of the classification of periosteal reactions. Materials and Methods: Retrospective study of 42 consecutive patients (mean age, 22 years; 20 men) with a confirmed diagnosis of osteosarcoma or Ewing's sarcoma, MRI and CR images having been acquired pretreatment. Three blinded radiologists detected periosteal reactions and evaluated each periosteal reaction subtype in CR and MRI images: Codman's triangle; laminated; and spiculated. The CR was used as a benchmark to calculate the diagnostic performance. We used the kappa coefficient to assess interobserver reproducibility. A two-tailed Fisher's exact test was used in order to assess contingency between CR and MRI classifications. Results: In the detection of periosteal reactions, MRI showed high specificity, a high negative predictive value, and low-to-moderate sensitivity. For CR and for MRI, the interobserver agreement for periosteal reaction was almost perfect, whereas, for the classification of different subtypes of periosteal reaction, it was higher for the Codman's triangle subtype and lower for the spiculated subtype. There was no significant difference between MRI and CR in terms of the classifications (p < 0.05). Conclusion: We found no difference between MRI and CR in terms of their ability to classify periosteal reactions. MRI showed high specificity and almost perfect interobserver agreement for the detection of periosteal reactions. The interobserver agreement was variable for the different subtypes of periosteal reaction.
Resumo Objetivo: Avaliar o desempenho da ressonância magnética (RM) na detecção de reação periosteal e comparar a classificação de presença ou ausência de reações periosteais entre a RM e a radiografia convencional (RC). Materiais e Métodos: Estudo retrospectivo incluindo 42 pacientes consecutivos (idade média, 22 anos; 20 homens) com diagnóstico confirmado de osteossarcoma ou sarcoma de Ewing, tendo exames de RM e RC adquiridos pré-tratamento. Três radiologistas avaliaram às cegas a presença ou ausência de reação periosteal e de cada subtipo de reação periosteal nas imagens de RC e RM: triângulo de Codman, multilamelada e espiculada. A RC foi usada como padrão de referência para cálculo do desempenho diagnóstico. Foi utilizado o coeficiente kappa para reprodutibilidade interobservador. Adicionalmente, foi realizado teste exato de Fisher bicaudal para avaliar se houve diferença significativa entre as leituras da RC e RM. Resultados: A RM mostrou alta especificidade, alto valor preditivo negativo e baixa sensibilidade na detecção de reação periosteal. A concordância interobservador para a reação periosteal foi quase perfeita para a RC e RM. A concordância interobservador para a classificação dos diferentes subtipos de reação periosteal foi maior para o subtipo triângulo de Codman e menor para o subtipo espiculada. Não houve diferença na detecção por RM e RC (p < 0,05). Conclusão: Não houve diferença significativa entre as classificações da presença ou ausência de reações periosteais entre os métodos RC e RM. A RM apresentou alta especificidade e concordância interobservador quase perfeita para a detecção de reação periosteal. A concordância interobservador para os diferentes subtipos de reação periosteal foi variável.
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El sarcoma de Ewing es una neoplasia maligna que afecta fundamentalmente a niños y adolescentes. Cerca del 95 % de estos sarcomas ocurre entre los 5-25 años. Es más frecuente en el sexo masculino y muy raro en la raza negra. Tiene predilección por las diáfisis de los huesos largos, las costillas y los huesos planos, como la escápula y la pelvis. Se presenta a una paciente de 37 años de edad con lesión tumoral en la mano la izquierda. Como tratamiento de primera línea se le realizó amputación del primer y segundo dedos de la mano. La biopsia informó sarcoma de Ewing. Posteriormente la paciente fue sometida a esquema de quimioterapia. Se considera de interés la presentación de este caso por la localización poco usual de la neoplasia.
Ewing’s sarcoma is a malignant tumor that primarily affects children and adolescents. Nearly 95% of these sarcomas occur between the ages of 5 to 25 years. It is more common in males and extremely rare among blacks. It presents most often in the diaphyses of long bones, the ribs and flat bones, such as the scapula and pelvis. We present the case of a 37 year-old woman with a tumor in the left hand. The amputation of the first and second fingers was performed as first-line treatment. The biopsy revealed a Ewing’s sarcoma. Subsequently, the patient underwent chemotherapy regimen. This case is of interest given the unusual location of the tumor.
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El sarcoma de Ewing representa el segundo tumor primario de hueso más común en niños y adolescentes (3 % de todos los tumores óseos pediátricos), solo excedido en prevalencia por el osteosarcoma. Respecto al sitio de compromiso, el sarcoma de Ewing afecta la pelvis, las extremidades y las costillas en el 86 % de los casos, seguido por la escápula (de 4 a 5 %). Este artículo presenta un caso de sarcoma de Ewing en la escápula; se describen sus características clínicas e imaginológicas, así como su tratamiento.
Ewing´s sarcoma is the second most common primary bone tumor in children and adolescents (3% of all pediatric bone tumors), its prevalence is only exceeded by osteosarcoma. Regarding the site of involvement, Ewing's sarcoma involves pelvis, limbs and the ribs in 86% of cases, followed by the scapula (4-5%). This article presents a case of Ewing's sarcoma in the scapula; clinical and imaging characteristics are described, as well as treatment.
Assuntos
Humanos , Sarcoma de Ewing , Escápula , Neoplasias Ósseas , Imageamento por Ressonância MagnéticaRESUMO
Fundamento: el sarcoma de Ewing es una neoplasia altamente maligna que afecta fundamentalmente a niños y adolescentes. Cerca del 95 % de estos sarcomas ocurren entre los 5-25 años, es frecuente en el sexo masculino y muy raro en la raza negra. Objetivo: actualizar y sistematizar los conocimientos sobre el sarcoma de Ewing de mayor utilidad para en el desempeño clínico-quirúrgico. Método: se realizó una revisión de la literatura de los últimos cinco años. Se insistió en la epidemiología, histopatología, diagnóstico y tratamiento. Desarrollo: el sarcoma de Ewing presenta una incidencia anual de 2, 73 casos por cada millón de habitantes. Predomina en las diáfisis y metáfisis, los huesos largos de las extremidades y huesos planos. Clínicamente puede presentarse como un cuadro pseudoinflamatorio caracterizado por dolor con exacerbación nocturna y una masa dolorosa asociados fiebre, malestar general y pérdida de peso; las que semejan una osteomielitis. El diagnóstico requiere técnicas de inmunohistoquímica y estudios moleculares. El tratamiento ha pasado por una serie de modalidades: cirugía, cirugía y radioterapia, radioterapia y quimioterapia y quimioterapia, para retomar en la actualidad la asociación de la cirugía con la quimioterapia y eventualmente la radioterapia. Conclusiones: la heterogeneidad histológica, favorece la diversidad de formas clínicas de presentación y hacen complejo su diagnóstico. La estrecha similitud con otras enfermedades, hacen del sarcoma de Ewing uno de los tumores de más difícil tratamiento, por lo que la detección temprana, la cirugía de resección seguida de la quimioterapia aseguran los mejores resultados.
Background: Ewing´s sarcoma is a highly malignant neoplasia that mainly affects children and adolescents. Around 95 % of these sarcomas appear between 5-25 years of age, are frequent in male sex and very infrequent in black people. Objective: to systematize and bring up to date the most useful information about the Ewing´s sarcoma for the clinical-surgical performance. Method: a review of the literature from the last five years was conducted, making emphasis on epidemiology, histopathology, diagnosis, and treatment. Development: Ewing´s sarcoma presents an incidence of 2.73 cases per million per year in the general population. From the clinical point of view the symptoms can include pseudo-inflammatory problems characterized by a painful mass and pain with nighttime aggravations, along with fever, discomfort, and weight loss; these manifestations resemble an osteomyelitis. The diagnosis requires the application of immunohistochemistry techniques and molecular studies. The treatment has passed through a series of modalities: surgery, surgery and radiotherapy, radiotherapy and chemotherapy; nowadays, the surgery-chemotherapy association and eventually, the radiotherapy have been taken up again. Conclusions: histological heterogeneity favors the diversity of clinical ways of presentation and makes more complex its diagnosis. The close similarity to other diseases make of the Ewing´s sarcoma one of the tumors of most difficult treatment; that is why the early detection and the removal surgery fallowed by chemotherapy guarantee the best results.
RESUMO
The Sarcomas Ewing/PNET is highly aggressive and both tumors present/display he himself phantom of genetic anomalies reason for which the term of Tumors of the family of sarcoma of Ewing is used (TFSE) that includes to a group of tumors with a phantom of neuroectodermic differentiation. Case of feminine adolescent of eleven years, that referred sharp pain, in hemitorax appears left, in Rx of the thorax heterogenous dense image of diffuse edges projected on third basal means in left pulmonary field received antibiotics, remained asymptomatic up to eight months later when chest pain reappeared, made Rx of the thorax where demonstrate at level of the eighth costal arc bony erosion, in addition to the image previously described to greater size. Normal physical examinations. normal examinations of laboratory, computerized axial Tomography of the chest demonstrate bony erosion, and commitment of the local pleura biopsy of the injury reported: Malignant Tumor of round cells, extensive necrosis. Bony scintygraphy indicated osteoblastic activity altered in D8, suggestive of secondary bony infiltration. Made resection of tumor like injury in chest wall with reconstruction, positioning of prosthesis and central catheter biopsy of bony Marrow: refusal. The morphologic and inmunohistochemical findings with positivity for compatible FLy and CD99 with tumor of the family of sarcoma of Ewing/primtive neuroectodermic tumor. At the moment in treatment programmed with standard chemotherapy every 21 days X-ray in week 12.
Los Sarcomas Ewing/PNET son altamente agresivos y ambos tumores presentan el mismo espectro de anomalías genéticas razón por la que se usa el término de Tumores de la familia del sarcoma de Ewing (TFSE) que abarca a un grupo de tumores con un espectro de diferenciación neuroectodérmica. Se presenta caso de adolescente femenina de once años, quien refirió dolor punzante, en hemitorax izquierdo, en Rx del tórax imagen densa heterogénea de bordes difusos proyectada sobre tercio medio basal en campo pulmonar izquierdo. Recibió antibióticos, permaneció asintomática hasta ocho meses después cuando reapareció dolor torácico, y en Rx del tórax se evidencio a nivel del octavo arco costal erosión ósea, además de la imagen anteriormente descrita de mayor tamaño. Examen físico y de laboratorios fueron normales, en Tomografía axial computarizada del tórax se evidencio erosión ósea, y compromiso de la pleura local. Biopsia de la lesión reportó: Tumor maligno de células redondas, extensa necrosis. Gammagrama Óseo señaló actividad osteoblástica alterada en D8 sugestivo de infiltración ósea secundaria. Se realizó resección de lesión tumoral, reconstrucción, colocación de prótesis, catéter central biopsia de Médula ósea: negativa. Los hallazgos morfológicos e inmunohistoquímicos con positividad para FLy y CD99 compatible con tumor de la familia del sarcoma de Ewing/tumor neuroectodérmico primitivo. Actualmente en tratamiento programado con quimioterapia estándar cada 21 días Radioterapia en la semana 12.
Assuntos
Humanos , Feminino , Criança , Neoplasias Ósseas/diagnóstico , Sarcoma de Ewing/diagnóstico , Diagnóstico Diferencial , Dor no Peito/etiologia , Imuno-Histoquímica , Neoplasias Ósseas/cirurgia , Radiografia Torácica , Sarcoma de Ewing/cirurgia , ToracotomiaRESUMO
OBJECTIVE: To outline the epidemiological profile and prognosis for Ewing's sarcoma in the Brazilian population. MATERIAL AND METHODS: The medical records of 64 patients with intraosseous Ewing's sarcoma who were treated at the Pediatric Oncology Institute, IOP-GRAACC-Unifesp, between 1995 and 2010, were retrospectively evaluated. RESULTS: The statistical analysis on the data obtained did not correlate factors such as sex, trauma, pathological fracture and time taken for case diagnosis with the treatment outcome. Factors such as initial metastasis, lung metastasis, tumor site, age, recurrence and type of surgery showed results corroborating what has been established in the literature. CONCLUSION: The prognosis in cases of Ewing's sarcoma was mainly influenced by the presence of metastases at the time of diagnosis.
RESUMO
OBJETIVO: Traçar o perfil epidemiológico e o prognóstico do sarcoma de Ewing na população brasileira. Material e MÉTODO: Foram avaliados, retrospectivamente, os prontuários de 64 pacientes tratados, com sarcoma de Ewing intraósseo, no Instituto de Oncologia Pediátrica, IOP-GRAACC-Unifesp, no período de 1995 a 2010. RESULTADOS: A análise estatística dos dados obtidos não correlacionou fatores como sexo, trauma, fratura patológica e tempo ao diagnóstico com o desfecho do tratamento. Fatores como metástase inicial, metástase pulmonar, local do tumor, idade, recidiva e tipo de cirurgia indicam resultados que corroboram a literatura consagrada. CONCLUSÃO: O prognóstico no sarcoma de Ewing foi influenciado principalmente pela presença de metástase ao diagnóstico.
OBJECTIVE: To outline the epidemiological profile and prognosis for Ewing's sarcoma in the Brazilian population. Material and METHODS: The medical records of 64 patients with intraosseous Ewing's sarcoma who were treated at the Pediatric Oncology Institute, IOP-GRAACC-Unifesp, between 1995 and 2010, were retrospectively evaluated. RESULTS: The statistical analysis on the data obtained did not correlate factors such as sex, trauma, pathological fracture and time taken for case diagnosis with the treatment outcome. Factors such as initial metastasis, lung metastasis, tumor site, age, recurrence and type of surgery showed results corroborating what has been established in the literature. CONCLUSION: The prognosis in cases of Ewing's sarcoma was mainly influenced by the presence of metastases at the time of diagnosis.
Assuntos
Humanos , Masculino , Feminino , Pré-Escolar , Criança , Adolescente , Adulto Jovem , Prognóstico , Sarcoma de Ewing/epidemiologia , Neoplasias ÓsseasRESUMO
OBJETIVO: As endopróteses parciais de joelho para as ressecções em sarcomas ósseos demonstram serem boa solução para o tratamento de pacientes com imaturidade esquelética. O objetivo deste estudo é avaliar o escore funcional, as vantagens, as desvantagens e indicações para esta técnica cirúrgica em quatorze pacientes em um protocolo brasileiro de osteossarcoma e sarcoma de Ewing. MÉTODOS: Análise retrospectiva realizada para identificar a evolução funcional e as possíveis complicações do procedimento. 14 pacientes com idade entre 10 e 22 anos avaliados funcionalmente pelos critérios de Enneking/ISOLS (International Society of Limb Salvage), sendo todos operados na mesma Instituição e pelo mesmo cirurgião. Foram utilizadas endopróteses parciais das extremidades distal do fêmur e proximal da tíbia com reconstrução ligamentar. ReSULTADOS: A análise do escore funcional de Enneking/ISOLS demonstrou 78,6 por cento de excelentes resultados e 21,4 por cento de bons. Dos 14 pacientes, todos portadores de tumores primitivos ósseos em protocolo de quimioterapia, nove não apresentaram nenhum tipo de complicação e cinco indivíduos evoluíram com complicações relacionadas ao procedimento, sendo que houve relação estatística positiva entre os maus resultados e a presença de complicações (p=0,027). CONCLUSÃO: As endopróteses parciais de joelhos são menos prejudiciais ao estoque ósseo de pacientes com esqueleto imaturo. As críticas sobre os maus resultados funcionais estão sendo suplantadas pelas novas técnicas de reconstrução, corretos protocolos de reabilitação, qualidade e tecnologia dos implantes, e o aumento da curva de aprendizado. Essa opção de tratamento per-mite a preservação do estoque ósseo e a possibilidade de revisão da artroplastia não convencional de modo menos agressivo.
OBJECTIVE: Partial knee endoprosthesis to bone sarcomas resections seems to be a good solution to treat this immature skeletal patients. The purpose of this study is to evaluate the functional score in fourteen patients, advantages and the technique indications. METHODS: Retrospective analysis was done to assess in this group of patients the functional evolution and the possible complications of the procedure. 14 patients between 10 and 22 years functionally evaluated in Ennekin/ISOLS (International Society of Limb Salvage) criteria, being all of them operated in the same institution by the same surgeon. Were used distal femur and proximal tibia partial endoprosthesis. RESULTS: General analysis demonstrated that the functional results were over than 67 percent (ISOLS criteria) in 78,6 percent of the patients, being considered excellent. 21,4 percent were considered good results, being between 50 and 66 percent. Bone storage was preserved when avoiding the adjacent segment resection. Surgery time was not prolonged in ligament reconstruction. CONCLUSION: Knee partial endoprosthesis are less damage to bone storage in young patients. The critics about the bad functional results are being supplied by new surgical techniques, excellent rehabilitation protocols, implants technology and the consequent learning curve. This option of treatment permits the preservation of healthy bone and provides the possibility of a revision replacement less aggressive.
Assuntos
Humanos , Masculino , Feminino , Adolescente , Adulto , Joelho , Prótese do Joelho , Osteossarcoma , Estudos Retrospectivos , Sarcoma de EwingRESUMO
UNLABELLED: Partial knee endoprosthesis to bone sarcomas resections seems to be a good solution to treat this immature skeletal patients. The purpose of this study is to evaluate the functional score in fourteen patients, advantages and the technique indications. METHODS: Retrospective analysis was done to assess in this group of patients the functional evolution and the possible complications of the procedure. 14 patients between 10 and 22 years functionally evaluated in Ennekin/ISOLS (International Society of Limb Salvage) criteria, being all of them operated in the same institution by the same surgeon. Were used distal femur and proximal tibia partial endoprosthesis. RESULTS: General analysis demonstrated that the functional results were over than 67 percent (ISOLS criteria) in 78,6 percent of the patients, being considered excellent. 21,4 percent were considered good results, being between 50 and 66 percent. Bone storage was preserved when avoiding the adjacent segment resection. Surgery time was not prolonged in ligament reconstruction. CONCLUSION: Knee partial endoprosthesis are less damage to bone storage in young patients. The critics about the bad functional results are being supplied by new surgical techniques, excellent rehabilitation protocols, implants technology and the consequent learning curve. This option of treatment permits the preservation of healthy bone and provides the possibility of a revision replacement less aggressive.