Sarcoma de Ewing extraesquelético: reporte de caso / Extraskeletal Ewing's Sarcoma: a Case Report

Introducción: El sarcoma de Ewing es un tumor maligno de alto grado con localización principalmente ósea; se han reportado aproximadamente 12% con presentación extra-esquelética. Actualmente, existen alrededor de 20 casos descritos en la literatura con origen mediastinal y 10 casos con origen pulmonar. Caso clínico: Se presenta el caso de una mujer de 25 años con un mes de disnea y dolor torácico, con el hallazgo de derrame pleural masivo y tumoración mediastinal en hemitórax derecho. Se le realiza toracotomía anterior bilateral con esternotomía transversa de Clamshell, con resección parcial que demuestra, por patología, sarcoma monomórfico de alto grado e inmunohistoquímica concluyente de sarcoma de Ewing. Conclusión: Este caso es una entidad rara y conlleva un reto diagnóstico para el clínico; sin embargo, debe sospecharse considerando la presentación clínica y radiológica del paciente, buscando incrementar la tasa de supervivencia mediante el diagnóstico y tratamiento oportuno.

Introduction: Ewing's sarcoma is a high-grade malignant tumor with mainly bony lo-calization; approximately 12% have been reported with extraskeletal presentation. Currently, there are about 20 cases described in the literature with mediastinal origin and 10 pulmonary cases. Case Report: We present the case of a 25-year-old woman with one month of dysp-nea and chest pain, with massive pleural effusion and mediastinal tumor in the right hemithorax who underwent bilateral anterior thoracotomy with Clamshell transverse sternotomy, with partial resection demonstrating, by pathology, high-grade monomorphic sarcoma and conclusive immunohistochemistry of Ewing's sarcoma. Conclusion: This case is a rare entity and involves a diagnostic challenge for the clinician; however, it should be suspected considering the clinical and radiological presentation of the patient, seeking to increase the survival rate through timely diagnosis and treatment.

Sarcoma de Ewing/tumor neuroectodérmico primitivo hepático / Ewing sarcoma/primitive hepatic neuroectodermal tumor

Resumen El sarcoma de Ewing (ES) y el tumor neuroectodérmi co primitivo (PNET) pertenecen al grupo de neoplasias denominadas tumores de células pequeñas y redondas. Los PNET se dividen en centrales y periféricos. El ES y los PNET periféricos surgen del tejido óseo, de los tejidos blandos o nervios periféricos. Presentamos un caso de ES/PNET hepático en un hombre sano que inició cuatro meses antes de la consulta con síntomas abdominales y pérdida de peso. La endoscopia digestiva alta y la analí tica no revelaron hallazgos relevantes. En la tomografía de abdomen se evidenció hígado aumentado de tamaño a expensas de lesión sólida que comprometía todos sus segmentos con realce al contraste endovenoso y grandes áreas de necrosis. Comprimía y desplazaba estructuras vecinas. Se realizó biopsia con aguja gruesa de la lesión hepática: neoplasia de células pequeñas y redondas. La inmunohistoquímica reveló negatividad para CD45, CKA1/A3, cromogranina, sinaptofisina y citoqueratinas CK7 y CK20. Expresión tenue de CD56 y positividad de CD99, FLI-1 y NKX2. Realizó tratamiento quimioterápico con carboplatino y etopósido por 6 ciclos con mejoría clínica y tolerancia al mismo. En imágenes de control se evidenció reducción de la masa con afección del lóbulo hepático derecho, compromiso de la vena cava inferior, infiltración de la glándula suprarrenal y polo superior del riñón derechos. Se remitió a cirugía hepatobiliar para resección quirúrgica de la lesión residual. El paciente rechazó el procedimiento quirúrgico. Nuestro objetivo es destacar el desafío diagnóstico clínico e histológico de esta entidad que obliga a descartar otras entidades clínicas.

Abstract Ewing sarcoma (ES) and primitive neuroectodermal tumor (PNET) belong to the group of neoplasms called small round cell tumors. PNETs have been divided into central and peripheral. ES and peripheral PNETs arise from bones, soft tissues, or peripheral nerves. We pres ent a case of hepatic ES/PNET in a healthy man that began four months before consultation with abdominal symptoms and weight loss. Upper gastrointestinal en doscopy and laboratory tests revealed no notable find ings. The abdominal tomography revealed an enlarged liver due to a solid lesion that involved all its segments with intravenous contrast enhancement and large areas of necrosis. It compressed and displaced neighboring structures. Core needle biopsy of the liver lesion was per formed: small round cell neoplasm. Immunohistochemis try revealed negativity for CD45, CKA1/A3, chromogranin, synaptophysin, and cytokeratins CK7 and CK20. Dim CD56 expression and CD99, FLI-1, and NKX2 positivity. He underwent chemotherapy treatment with carboplatin and etoposide for 6 cycles with clinical improvement and tolerance. Control images showed reduction of the mass with involvement of the right hepatic lobe, involvement of the inferior vena cava, infiltration of the right adrenal gland and upper pole of the right kidney. He was referred to hepatobiliary surgery for surgical resection of the residual lesion. The patient rejected the proposed surgi cal procedure. Our objective is to highlight the clinical and histological diagnostic challenge of this entity that requires ruling out other clinical entities.

Clinical analysis of 21 cases of primary Ewing sarcoma of the thoracic wall / 中国癌症杂志

Background and Purpose:Primary Ewing sarcoma of the thoracic wall(PEST)is a rare extraosseous Ewing sarcoma that occurs in the chest wall or thoracic cavity with a short survival,poor prognosis and a high rate of recurrence.Early diagnosis and treatment are the best way to prolong survival time since the cause of PEST is not clear.This study aimed to explore the clinicopathologic characteristics,diagnosis and treatment of PEST to improve clinical understanding of this disease.Methods:A total of 21 cases with PEST were treated at The First Affiliated Hospital of Soochow University,and reviews were published from 2018 to 2023.Clinical data,pathological features,treatment and follow-up of the patients were analyzed respectively.The survival was from the start of treatment to the death of the patient or the end of the follow-up.Cumulative survival was estimated by Kaplan-Meier method.Results:A total of 21 cases with PEST(male/female ratio,13∶8;sites of left/right chest ratio,6∶15;median age,20 years;mean age,28 years;median diameter of the tumor,8.0 cm;mean diameter of the tumor,18.1 cm)met the inclusion criteria.65.2%of the patients presented with the pain in the ipsilateral thoracic and abdominal area.In 47.1%of cases,the ipsilateral ribs were invaded with pleural effusion.Pathological morphology microscopy showed most tumor cells were tightly packed or lobular distribution of small blue round cells.In immunohistochemistry,CD99 and vimentin were positive in 100%and 80%cases respectively while neurogenic markers were expressed to varying degrees.EWSR1 separated signal was found by fluorescence in situ hybridization(FISH),and the EWSR1-FLI1 fusion was detected by next-generation sequencing(NGS)in two cases at our hospital.Two cases received neoadjuvant chemotherapy,10 patients received chemotherapy and radiotherapy after operation,5 cases were treated with radiotherapy only,1 case received surgery only,and 3 cases had no surgical data.A total of 14 cases were followed up for 3-38 month while 7 cases were lost to visit.Cumulative survival correlates with age at disease.The mean survival time was 19.98 months,and the median survival time was 13.00 months.Conclusion:Young males,right chest and the mass larger than 8 cm are more often found.Most cases can be initially diagnosed using histopathology and immunohistochemical markers.FISH or NGS of the EWSR1 gene test are a highly accurate method for diagnosis.The prognosis of PEST is extremely poor,and the cumulative survival rate is negatively correlated with the age of onset.Surgery,radiotherapy and chemotherapy are the main treatments for this disease.

Pelvic Ewing Sarcoma: The Great Mimicker / Sarcoma de Ewing pélvico: o grande imitador

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.

Ewing’s sarcoma or peripheral primitive neuroectodermal tumor at the base of tongue: a rare location case report

Peripheral primitive neuroectodermal tumor now termed as Ewings sarcoma/PNET, representing a family of tumors with varying degrees of neuronal differentiation and genetic rearrangements. Most common sites are extraosseous involving trunk and lower limb. Base of tongue is a very rare site for Ewing’s/PNET, hence in view of rarity of the location as an uncommon presentation, the case has been reported for the better understanding and supporting the literature with the similar finding. A 68 years old female patient presented with the complaint of difficulty in swallowing since 3 months. On examination growth was identified at the left side base of tongue. Biopsy was processed and stained with H&E and other relevant markers. Differentials on the basis microscopic examination were lymphoma, neuroendocrine tumor and primitive neuroectodermal tumor. Various immunohistochemical markers to rule out the given differential were used. The tumor was positive for CD 99 and vimentin. The case was repored as PNET. Peripheral PNET is a challenging topic. Wide range of extraosseous locations have been observed but head and neck being less reported needs to be studied for understanding the behavior of this highly malignant disease in this rare location so that patient can be benefitted by advanced multimodality treatments including surgery, chemotherapy and radiotherapy.

Ewing’s sarcoma or peripheral primitive neuroectodermal tumor at the base of tongue: a rare location case report

Peripheral primitive neuroectodermal tumor now termed as Ewings sarcoma/PNET, representing a family of tumors with varying degrees of neuronal differentiation and genetic rearrangements. Most common sites are extraosseous involving trunk and lower limb. Base of tongue is a very rare site for Ewing’s/PNET, hence in view of rarity of the location as an uncommon presentation, the case has been reported for the better understanding and supporting the literature with the similar finding. A 68 years old female patient presented with the complaint of difficulty in swallowing since 3 months. On examination growth was identified at the left side base of tongue. Biopsy was processed and stained with H&E and other relevant markers. Differentials on the basis microscopic examination were lymphoma, neuroendocrine tumor and primitive neuroectodermal tumor. Various immunohistochemical markers to rule out the given differential were used. The tumor was positive for CD 99 and vimentin. The case was repored as PNET. Peripheral PNET is a challenging topic. Wide range of extraosseous locations have been observed but head and neck being less reported needs to be studied for understanding the behavior of this highly malignant disease in this rare location so that patient can be benefitted by advanced multimodality treatments including surgery, chemotherapy and radiotherapy.

Sarcoma de Ewing multifocal: presentación de un caso pediátrico / Multifocal Ewing sarcoma: presentation of a pediatric case / Sarcoma de Ewing Multifocal: apresentação de um caso pediátrico

Introducción: el Sarcoma de Ewing es una neoplasia maligna de origen mesenquimático. Al momento del diagnóstico el 75% se presentan en forma localizada. Objetivo: comunicar un caso que por su presentación multifocal, generó dificultades diagnósticas. Caso clínico: niña de 6 años. Consulta por traumatismo de mano derecha tras caída de su altura 24 horas previas, constatándose en mano y puño derecho edema, calor y eritema, movilidad conservada. No fiebre. Radiografía: aumento del diámetro del tercer metacarpiano, imagen esmerilada, no trazos de fracturas. Ingresa con planteo de celulitis. Anemia leve microcítica, hipocrómica. Proteína C reactiva 82 mg/l. Recibe clindamicina intravenosa 72 horas, completa 14 días vía oral. Persistencia de alteraciones en puño y mano derecha, agrega tumoración de raíz nasal con desviación del eje, indolora. Fosfatasa alcalina, lactato deshidrogenasa, fosfatemia, calcemia normales. Resonancia magnética: alteración morfoestructural de radio, olecranon y tercer metacarpiano, fractura de olecranon y radio, reacción perióstica. Pet-Scan: lesión extensa ósea en macizo facial, tibias, cúbitos, humero derecho y clavícula. Biopsia 3er metacarpiano: tumor de células pequeñas, redondas azules, CD99 y vimentina positivo. Comienza poliquimioterapia y radioterapia sin complicaciones. Conclusiones: es frecuente que las manifestaciones clínicas iniciales sean confundidas con entidades más frecuentes, como post-traumáticas y/o inflamatorias, tal como ocurrió en este caso. Posteriormente, la aparición de nuevas lesiones y compromiso del estado general orientó el abordaje diagnóstico de la patología tumoral. La confirmación exige el estudio anatomopatológico con estudio inmunohistoquímico. La presencia de metástasis óseas constituye un factor de mal pronóstico y dificulta el abordaje terapéutico.

Introduction: Ewing's sarcoma is a malignant neoplasm of mesenchymal origin. At the time of diagnosis 75% of the cases are localized. Objective: to report a case that, due to its multifocal presentation, generated diagnostic difficulties. Clinical case: 6-year-old girl. She consulted for right hand trauma after a fall from her height 24 hours earlier, with edema, warmth and erythema in the right hand and fist, with preserved mobility. No fever. X-ray: increase in the diameter of the 3rd metacarpal, frosted image, no traces of fractures. Admitted with cellulitis. Mild microcytic anemia, hypochromic. C-reactive protein 82mg/l. Receives intravenous clindamycin 72 hours, completes 14 days orally. Persistence of alterations in fist and right hand, adds tumor of nasal root with deviation of the axis, painless. Alkaline phosphatase, lactate dehydrogenase, phosphatemia, normal calcemia. MRI: morphostructural alteration of radius, olecranon and 3rd metacarpal, fracture of olecranon and radius, periosteal reaction. Pet-Scan: extensive bone lesion in facial mass, tibiae, ulnae, right humerus and clavicle. Biopsy 3rd metacarpal: small cell tumor, blue round, CD 99 and vimentin positive. Polychemotherapy and radiotherapy were started without complications. Conclusions: it is frequent that the initial clinical manifestations are confused with more frequent entities, such as post-traumatic and/or inflammatory, as occurred in this case. Subsequently, the appearance of new lesions and compromise of the general condition guided the diagnostic approach of the tumor pathology. Confirmation requires anatomopathological study with immunohistochemical study. The presence of bone metastases constitutes a poor prognostic factor and hinders the therapeutic approach.

Introdução: O sarcoma de Ewing é um neoplasma maligno de origem mesenquimatosa. No momento do diagnóstico, 75% dos casos são localizados. Objetivo: Relatar um caso que, devido a sua apresentação multifocal, causou dificuldades diagnósticas. Caso clínico: Menina de 6 anos. Ela consultou por traumatismo à mão direita após cair de sua altura 24 horas antes, com edema, calor e eritema na mão direita e punho, com mobilidade preservada. Sem febre. Raio-X: aumento do diâmetro do 3º metacarpo, imagem fosca, sem vestígios de fraturas. Admitido com a sugestão de celulite. Anemia microcítica leve, hipocrómica. Proteína C reativa 82mg/l. Recebe clindamicina intravenosa por 72 horas, completa 14 dias por via oral. Persistência de alterações no punho e mão direita, tumor indolor da raiz nasal com desvio do eixo. Fosfatase alcalina, desidrogenase láctica, fosfataemia, calcemia normal. IRM: alteração morfo-estrutural do rádio, olecrânio e 3º metacarpo, fratura do olecrânio e do rádio, reação periosteal. Pet-Scan: extensa lesão óssea na massa facial, tíbia, ulnae, úmero direito e clavícula. Biópsia do 3º metacarpo: tumor de pequenas células, redondo azul, CD 99 e vimentina positiva. Ela iniciou a poli-quimioterapia e radioterapia sem complicações. Conclusões: É comum que as manifestações clínicas iniciais sejam confundidas com entidades mais freqüentes, tais como pós-traumáticas e/ou inflamatórias, como ocorreu neste caso. Posteriormente, o aparecimento de novas lesões e o envolvimento do quadro geral levaram a uma abordagem diagnóstica da patologia tumoral. A confirmação requer um estudo anatomopatológico com estudo imuno-histoquímico. A presença de metástases ósseas é um fator de mau prognóstico e dificulta a abordagem terapêutica.

Correlation NKX2.2 IHC and EWSR1 break-apart FISH in the diagnosis of Ewing sarcoma: Can combined NKX2.2 and CD99 immunoexpression obviate or minimize the need of FISH testing? First assessment study from Indian tertiary cancer care center

Context: Ewing sarcoma (ES) are malignant small round cell tumors (MSRCT) characterized by rearrangements of EWSR1 gene. Although gold standard for diagnosis is detection of specific fusion genes by molecular testing, these ancillary tests are costly and only available in limited number of settings. There is a persuasive evidence for reliability of NKX2.2 immunohistochemistry (IHC) as a surrogate marker for EWSR1 gene rearrangement in ES. Aims: The aim of this study is to correlate the NKX2.2 immuno-expression with genetically confirmed ES cases and also to assess the reliability and accuracy of NKX2.2 along with combined positivity of NXX2.2 and CD99 in diagnosing ES and differentiating it from other relevant histological mimics. Settings and Design: The present study is a retrospective study conducted over a period of 6-year duration in a tertiary cancer care center. Methods and Material: We evaluated NKX2.2 immunoexpression in 35 genetically confirmed cases of ES and also in pertaining differential entities (n = 58) of ES including rhabdomyosarcoma (n = 20), lymphoblastic lymphoma (n = 14), Wilms tumor (n = 10), poorly differentiated synovial sarcoma (n = 4), small-cell osteosarcoma (n = 4), neuroblastoma (n = 5), and mesenchymal chondrosarcoma (n = 1). CD99 was performed in the category of MSRCTs showing NKX2.2 positivity to evaluate combined specificity for the diagnosis of ES. Results: Of the 35 genetically confirmed cases of ES, 29 cases (83%) showed NKX2.2-positive expression (83% sensitivity). Compared to ES, NKX2.2 was positive in only 05% cases (3/58 cases) of non-ES MSRCT. Only two of five cases of neuroblastomas and one case of mesenchymal chondrosarcoma showed NKX2.2 positivity. CD99 positivity was seen in 100% of ES and in the single case of mesenchymal chondrosarcoma. All five cases (100%) of neuroblastoma were negative for CD99. Conclusions: The presented study, which is the first from an Indian oncology center, showed NKX2.2 IHC is quite reliable in diagnosis of ES in the right clinicopathological context. With remarkable sensitivity and specificity of NKX2.2 IHC for diagnosis of ES, we propose that combined positivity of CD99 and NKX2.2 IHC can obviate or minimize the need of EWSR1 gene rearrangement molecular testing for diagnosis of ES.

Desafíos en el manejo del sarcoma de Ewing en una paciente testigo de Jehová / Challenges in the care of Ewing's sarcoma in a Jehovah's Witness patient

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.

Tailored therapy and bloodless supportive care measures for a Jehovah’s witness with metastatic Ewing sarcoma

A seventeen-year-old Jehovah’s witness with metastatic Ewing sarcoma initially declined cancer therapy due to the risk of life-threatening bone marrow suppression. He then subsequently agreed to other treatment options with bloodless supportive care measures in order to achieve quality of life and long-term control of his disease. This case report adds to the collective knowledge of providing treatment options including bloodless supportive care measures for Jehovah’s witness patients with cancer.

Primary renal ewing sarcoma in a 15-year-old female: Case report

@#Extraskeletal Ewing sarcoma (EES) is a rare tumor that primarily affects children and lacks specific clinical signs. Diagnosis is confirmed through imaging techniques, histology, and molecular diagnostics. Treatment typically involves surgical intervention and chemotherapy. We present the case of a 15-year-old female with a history of abnormal mass enlargement in the right flank area. An initial diagnosis of Wilms tumor was made, and the patient underwent a right open radical nephrectomy. However, the tumor recurred eight months after nephrectomy, necessitating a metastasectomy. Chemotherapy was started to immediately target the tumor recurrence. Next-generation sequencing done on the open radical nephrectomy and metastasectomy samples revealed the presence of the EWSR1-FLI1 fusion gene in both specimens, confirming the final diagnosis to be primary renal Ewing sarcoma. Despite undergoing a right open radical nephrectomy for the primary tumor site, a metastasectomy during tumor recurrence, and chemotherapy, the patient’s prognosis remained poor.

Clinical and imaging features of eight cases of Ewing sarcoma of the jaw / 华西口腔医学杂志

OBJECTIVES@#This study investigate the clinical and imaging features of Ewing sarcoma (ES) of the jaw.@*METHODS@#Eight cases of pathologically diagnosed ES of the jaw from January 2010 to June 2022 were included in the study. Clinical and radiological features were retrospectively analyzed.@*RESULTS@#Among the eight cases, the mean age at onset was 29.4 years, and the male to female ratio was 7∶1. The predilecting site was the posterior part of mandible, accounting for 75% of the cases. The lesions often exhibited early numbness of the lower lip and lymphadenopathy. The main radiographic manifestation of mandibular lesions was ill-defined radiolucency, mixed with fibrous or brush-like tumor matrix, and soft tissue mass. The maxillary ES lesions mainly presented as lytic bone destruction accompanied by adjacent soft tissue mass. Periosteal ossification was rarely seen.@*CONCLUSIONS@#The clinical and imaging characteristics of ES in the jaw are helpful for its diagnosis.

A Surgical Case of Sarcomas with BCOR Genetic Alternations Invading the Heart through the Pulmonary Veins / 日本心臓血管外科学会雑誌

Sarcomas with BCOR genetic alternations, formerly treated as Ewing-like sarcomas, are malignant tumors with a poor prognosis. They have been classified under the category of undifferentiated small round cell sarcomas of bone and soft tissue since 2020. There are only a few reports on surgical treatment for these sarcomas, as they are extremely rare, and no specific treatment has been established. Among them, there have been no reports on the treatment of patients with intracardiac invasion. We report herein the case of intracardiac invasion of a rare sarcomas with BCOR genetic alternations. The patient is a 14-year-old girl who presented to the hospital with a chief complaint of left upper arm pain. Computed tomography (CT) showed tumors in the left axilla and left thoracic cavity, and after biopsies of each, we diagnosed the patient with sarcomas with BCOR genetic alternations. Although chemotherapy was planned, echocardiography revealed a mobile tumor in the left atrium, we decided to perform surgical procedure before chemotherapy to reduce the risk of embolism and sudden death. The tumor invaded directly the left upper pulmonary vein and extended into the left atrium. Since right lung metastasis was suspected, we considered the en bloc tumor resection while preserving the left lung. However it was difficult because the tumor invaded into the vicinity of the lower lobe bronchus. Concerned about extracardiac seeding, we resected the tumor as much as possible intravascularly. Although there was residual tumor, chemotherapy was started immediately after surgery, and the tumor has shrunk in size. We are planning to remove the entire tumor after several courses of chemotherapy.

Sarcoma de Ewing extraesquelético: relato de um caso raro e exuberante simulando lipoma / Extraskeletal Ewing's sarcoma: report of a rare and exuberant case simulating lipoma

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.

Ewing Sarcoma in the Sciatic Nerve: Case Report / Sarcoma de Ewing no Nervo Ciático: Relato de Caso

Ewing sarcoma (ES) is a malignant neoplasm that affects bones and soft tissues, usually in young patients. Currently, ES is grouped with other tumors that share the same histological and genotypic characteristics, forming the Ewing Sarcoma Family of Tumors (ESFT), which includes ES of bone, extraosseous ES (peripheral neuroepithelioma), Askin tumor, and peripheral primitive neuroectodermal tumor (PNET). Its origin in peripheral nerves is extremely rare, making its diagnosis and treatment very challenging. We describe a case of a 27-year-old male with extraosseous ES originating in the sciatic nerve, which was surgically removed, and discuss the difficulties encountered in the management of this patient.

O sarcoma de Ewing (SE) é uma neoplasia maligna que acomete ossos e partes moles, geralmente em pacientes jovens. Atualmente, o SE é agrupado com outros tumores que compartilham as mesmas características histológicas e genotípicas, formando os Tumores da Família do Sarcoma de Ewing (TFSE), que incluem o SE ósseo, o SE extraósseo (neuroepitelioma periférico), o tumor de Askin, e o tumor neuroectodérmico primitivo (TNEP) periférico. Sua origem em nervos periféricos é extremamente rara, o que torna o seu diagnóstico e tratamento um grande desafio. Descrevemos o caso de um homem de 27 anos com SE extraósseo originário no nervo ciático, que foi removido cirurgicamente, e discutimos as dificuldades encontradas no manejo desse paciente.

Ewing sarcoma: A rare report from the Indian pediatric population

Ewing抯 sarcomas are rare, aggressive tumors with a tendency toward recurrence following resection, and early metastasis. Although patients of younger or older age account for almost 30% of instances, peak incidences occur between the ages of 10 and 20. We, hereby, report the case of a 10-year-old girl who presented with a 3-month history of pain in her right hip that was unable to be relieved by non-steroidal anti-inflammatory medicine and physical therapy. On examination, bone mar

Sarcoma de Ewing mesencefálico: reporte de caso / Mesencephalic Ewing sarcoma: Case report

El sarcoma de Ewing es una neoplasia de origen más frecuentemente óseo; otras localizaciones son excepcionales. En el caso de las presentaciones primarias intracraneales, resulta imprescindible descartar que se trate de un secundarismo así como también de otros tumores neuroectodérmicos que puedan requerir distintos abordajes diagnósticos y terapéuticos. Se presenta a una paciente de 14 años que consultó por ptosis palpebral de ojo izquierdo asociado a diplopía de 2 meses de evolución; los estudios por imágenes mostraron una lesión tumoral extraaxial situada a nivel de la cisterna interpeduncular. Se realizó la exéresis completa, con diagnóstico anatomopatológico de sarcoma de Ewing de ubicación mesencefálica

Ewing's sarcoma is a malignant neoplasm mainly occurring in the bone, with other locations being exceptional. In the case of primary intracranial presentations, it is essential to rule out metastatic lesions as well as other neuroectodermal tumors that may require different diagnostic and therapeutic approaches. We present a 14-year-old patient who consulted for upper eyelid ptosis of left eye associated with a 2-month history of diplopia, with imaging evidence of extra-axial tumor lesion, located at the level of the interpeduncular cistern. Complete excision was performed, with a pathological diagnosis of Ewing's sarcoma of midbrain location.

Sarcoma de Ewing extraesquelético en cavidad nasal y senos paranasales, reporte de caso / Extra-skeletal Ewing sarcoma in the nasal cavity and paranasal sinus, case report

El sarcoma de ewing es un tumor maligno de rápido crecimiento, con prevalencia de 1-5 casos por cada 1.000.000 habitantes, su forma extraesquelética en la cavidad sinonasal o senos paranasales es inusual. Objetivo: describir la localización atípica de esta neoplasia y la importancia de lograr un diagnóstico oportuno. Paciente femenina, con una masa en la cavidad nasal derecha de dos meses de evolución, cefalea y epistaxis. Con asimetría en región orbitaria derecha y deformidad del tabique nasal, senos paranasales con sintomas de obstrucción. La tomografía reveló una masa que invade senos paranasales. La biopsia mostró un sarcoma de Ewing. Se confirmó con CD99. La paciente recibió quimioterapia y plan de resección quirúrgica, pero falleció. El diagnóstico y tratamiento oportuno del sarcoma de ewing en cavidad sinonasal debe apoyarse con examenes tomográficos, histopatológicos, inmunohistoquímicos y de ser posible citogenéticos para llegar al diagnóstico definitivo en etapas tempranas del tumor

Ewing's sarcoma is a rapidly growing malignant tumor, with a prevalence of 1-5 cases per 1,000,000 inhabitants, its extraskeletal shape in the sinonasal cavity or paranasal sinuses is unusual. Objective: to describe the atypical location of this neoplasm and the importance of achieving a timely diagnosis. Female patient, with a mass in the right nasal cavity of two months of evolution, headache and epistaxis. With asymmetry in the right orbital region and deformity of the nasal septum, paranasal sinuses with symptoms of obstruction. Tomography revealed a mass that invades the paranasal sinuses. The biopsy showed Ewing's sarcoma. It was confirmed with CD99. The patient received chemotherapy and a surgical resection plan, however she died. The timely diagnosis and treatment of Ewing's sarcoma in the sinonasal cavity should not be based solely on clinical evaluation, it requires a tomographic, histopathological, immunohistochemical and, if possible, cytogenetic examination to reach a definitive diagnosis in the early stages of the tumor.

Fractura, más allá del trauma / Fracture, beyond trauma

Se expone el caso de un paciente de 19 años, sin antecedentes médicos, con historia de traumatismo en el muslo derecho, que tuvo acortamiento, edema, rotación del miembro inferior derecho y dolor. En la radiografía se identificó una fractura en el tercio proximal de fragmentos múltiples de la diáfisis con engrosamiento y reacción perióstica que generaron una sospecha de un tumor óseo. La resonancia magnética confirmó una neoplasia ósea de características malignas en el tercio superior del fémur con destrucción de la cortical e invasión del canal endomedular sin signos de lesiones metastásicas. La biopsia confirmó el diagnóstico de sarcoma de Ewing localizado. El manejo intrahospitalario consistió en antiinflamatorios e inmovilización del miembro inferior derecho por 21 días. Luego, recibió tres ciclos de quimioterapia con el esquema para sarcoma de Ewing fase I. Además, se indicó terapia física, tratamiento ambulatorio con analgésico, radioterapia y finalmente se practicará la resección parcial de cadera. Se evidenció disminución del edema local, control del dolor con medicamentos orales y recuperación de la movilidad, aunque mantuvo la limitación funcional del miembro inferior derecho que imposibilita la bipedestación y la deambulación

A 19-year-old patient, with no previous medical history, with a history of trauma to the right thigh, presented with shortening, edema, rotation of the right lower limb and pain. Radiography identified a fracture in the proximal third of multiple fragments of the diaphysis with thickening and periosteal reaction that generated a suspicion of a bone tumor. MRI confirmed a bone neoplasm of malignant characteristics in the upper third of the femur with destruction of the cortex and invasion of the end medullary canal without signs of metastatic lesions. The biopsy confirmed the diagnosis of localized Ewing's sarcoma. The intrahospital management consisted of anti-inflammatory drugs and immobilization of the right lower limb for 21 days. Then, she received three cycles of chemotherapy with the Ewing sarcoma phase 1 scheme. In addition, physical therapy, outpatient treatment with analgesic, radiotherapy and finally partial hip resection was indicated. There was a decrease in local edema, pain control with oral medications, mobility was recovered, although the functional limitation of the right lower limb was maintained, making it impossible to stand and walk