[Epidural extraskeletal Ewing sarcoma. Case report and literature review]. / Sarcoma de Ewing extraesquelético epidural. Caso clínico y revisión de la literatura.

Ewing sarcoma is a malignant tumour of the bone that sometimes presents extraskeletal involvement, with the epidural location being rare. We report the case of a 45-year-old woman with paresthesia, paresis and urinary retention. Magnetic resonance imaging showed an epidural mass from C6 to D3. Laminectomy from C7 to D2 and partial resection of the lesion was performed. Pathological analysis was consistent with Ewing sarcoma. The patient received chemotherapy and radiotherapy, without evidence of disease at 8 months follow-up. A review of the literature on all published cases of extraskeletal Ewing sarcoma with epidural involvement is presented.

Primary epidural lumbar Ewing sarcoma presenting as cauda equina syndrome in an adult patient who underwent surgical decompression: A case report.

Ewing sarcoma (ES) is a rare primary neoplasm in the lumbar adult spine and may mimic a benign tumor. In this case, after a patient's three-month history of lower back pain and rapidly progressing leg numbness and weakness, magnetic resonance imaging (MRI) showed a mass in the third lumbar vertebra. At a two-month follow-up, imaging showed a fracture, compression and lesion enlargement. Decompression and fixation confirmed ES, and the patient began combined radiotherapy and chemotherapy. Two months postoperatively, residual ES was suspected on MRI. The patient underwent a second surgery, and histopathology confirmed necrosis. A six-month follow-up after the first surgery showed no tumor recurrence. This case supports the inclusion of ES in the differential diagnosis of pathologic spinal fracture. Early decompression and spinal fixation are critical for preserving neurologic and spinal functions in ES complicated by a compression fracture. Combined adjuvant radiotherapy and chemotherapy remain the standard therapeutic strategy.

Intestinal Ewing sarcoma: An unusual presentation in the pediatric age.

BACKGROUND: Ewing's Sarcoma (ES) is the second most common type of bone cancer, with an annual incidence of 2.9:100,000. Extraosseous cases represent 15%; however, there are no reported cases of ES located in the intestine in the pediatric population. CASE REPORT: We describe the case of a 14-year-old male patient, previously healthy, who started with an anemic syndrome, weight loss, and diaphoresis of 8 weeks of evolution. After visiting a physician, who documented the presence of anemia, the patient was referred to the National Institute of Pediatrics. Physical examination showed grade III-IV systolic murmur, splenomegaly, and pain in the left hemiabdomen with no irradiation. Computed axial tomography showed a mass-dependent on the peritoneum and intestinal loop. A biopsy of the lesion showed intestinal ES. The lesion was completely resected, and the patient was treated with chemotherapy and radiotherapy. Thirty months after diagnosis, the patient has no evidence of tumor activity. CONCLUSIONS: Extraosseous presentation of ES in pediatric age is rare. There are no reports of intestinal ES in the Latin American pediatric population, although eight case reports were found in adults. ES is curable by a combination of chemotherapy, radiotherapy, and surgery. The medical literature indicates that the extraosseous presentation should receive the same treatment as the osseous presentation, which can provide a survival rate of up to 70% if there is no evidence of metastasis (which most frequently is observed in the lung).

INTRODUCCIÓN: El sarcoma de Ewing (SE) es el segundo tipo cáncer más común de hueso, cuya incidencia anual es de 2.9:100,000. Los casos extraóseos representan el 15%; sin embargo, no existen reportes en la literatura de casos de SE ubicados en el intestino en la población pediátrica. CASO CLÍNICO: Se describe el caso de un paciente de sexo masculino de 14 años, previamente sano, que inició con síndrome anémico, pérdida de peso y diaforesis de 8 semanas de evolución. Acudió con un médico, quien documentó la presencia de anemia y lo refirió al Instituto Nacional de Pediatría. A la exploración física presentaba soplo sistólico grado III-IV, esplenomegalia y dolor en hemiabdomen izquierdo sin irradiaciones. La tomografía axial computarizada mostró una masa dependiente del peritoneo y asa intestinal. La biopsia de la lesión reportó SE intestinal. Se resecó por completo la lesión y el paciente recibió tratamiento con quimioterapia y radioterapia. Después de 30 meses del diagnóstico, el paciente se encuentra sin datos de actividad tumoral. CONCLUSIONES: La presentación extraósea del SE en edad pediátrica es rara. No existen reportes de presentación de SE intestinal en la población pediátrica latinoamericana, aunque se encontraron ocho reportes de caso en adultos. El SE es curable mediante la combinación de quimioterapia, radioterapia y cirugía. La literatura médica indica que la presentación extraósea debe recibir el mismo tratamiento que la ósea, lo cual puede proporcionar una sobrevida de hasta el 70% si no hay evidencia de metástasis (que ocurre más frecuentemente a pulmón).

Primary epidural lumbar Ewing sarcoma presenting as cauda equina syndrome in an adult patient who underwent surgical decompression: A case report.

Ewing sarcoma (ES) is a rare primary neoplasm in the lumbar adult spine and may mimic a benign tumor. In this case, after a patient's three-month history of lower back pain and rapidly progressing leg numbness and weakness, magnetic resonance imaging (MRI) showed a mass in the third lumbar vertebra. At a two-month follow-up, imaging showed a fracture, compression and lesion enlargement. Decompression and fixation confirmed ES, and the patient began combined radiotherapy and chemotherapy. Two months postoperatively, residual ES was suspected on MRI. The patient underwent a second surgery, and histopathology confirmed necrosis. A six-month follow-up after the first surgery showed no tumor recurrence. This case supports the inclusion of ES in the differential diagnosis of pathologic spinal fracture. Early decompression and spinal fixation are critical for preserving neurologic and spinal functions in ES complicated by a compression fracture. Combined adjuvant radiotherapy and chemotherapy remain the standard therapeutic strategy.

Primary vertebral tumor of axis: A clinical and surgical challenge. / Tumor vertebral primario de axis: un desafío clínico y quirúrgico.

We report the case of a 17-year-old male presented to the emergency department with a six weeks history of neck pain and no neurogical deficit. Computed tomography and magnetic resonance imaging revealed an expansile lesion in the axis, with soft tissue and spinal cavity invasion, without mielopathy signs. Tomography-guided mass biopsy was taken. Waiting for histopathologic results, the pacient developed acute tetraparesis and sphincter incompetence. Magnetic resonance revealed that the bone mass had grown with epidural compromise, mielopathy and new vertebral lesions. Medular decompression with laminectomy, excision of the posterior elements of axis with the involved soft tissue mass and occipito-cervical fixation was performed. Neurological recovery was complete. Inmunochemistry revealed an Ewing Sarcoma. Chemoteraphy treatment was given, with partial response. Primary Ewing sarcoma of atlas-axis is a rare entity with poor prognosis. Multidisciplinary approach treatment is needed, with a total surgical resection if it is possible, a real challenge for the surgeon.

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.

[Neuroectodermic renal tumor. Case report and bibliographic review.] / Tumor renal neuroectodérmico. A propósito de un caso tratado en nuestra unidad. Revisión de la literatura.

We report the case of a 16 year old female who underwent left radical nephrectomy for a 13.2 cm neuroectodermic renal tumor and posterior chemo and radiotherapy in the Quiron Hospitals in Madrid and Complejo Hospitalario de Toledo, with a current survival of 14 months. Medical literature was reviewed for the historical, clinical, etiopathogenic, diagnostic, prognostic, therapeutic and survival features. We estimate the number of published cases to date. We also address the treatment performed and its results under a critical view.

Se presenta el caso de una adolescente de 16 años de edad a la que practicamos nefrectomia radical izquierda por un tumor neuroectodérmico renal de 13,2 cm y tratada posteriormente mediante quimioterapia y radioterapia en los Hospitales Quirón de Madrid y Complejo Hospitalario de Toledo, con una supervivencia actual de 14 meses. Se revisa la literatura médica en los aspectos históricos, clínicos, etiopatogénicos, diagnósticos, pronósticos, terapéuticos y de supervivencia. Se realiza una estimación de la cantidad de casos publicados hasta la fecha. Abordamos además con una visión descriptiva y crítica el tratamiento realizado y su resultado.

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.

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.

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.

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.

[Histopathology and molecular profile of a case of prostatic Ewing sarcoma]. / Histopatología y perfil molecular de un caso de sarcoma de Ewing prostático.

We report a case of Ewing sarcoma localized in the prostate gland of a 33-year-old patient without bone or soft tissue involvement. Evidence of EWS and FLI1 gene translocation was detected by fluorescence in situ hybridization (FISH). This is an unusual case with an interesting clinical presentation; indeed, only a few cases have been reported to date and not all have the supporting biological studies now considered essential for the diagnosis.

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.

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

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.

Intestinal Ewing sarcoma: An unusual presentation in the pediatric age / Sarcoma de Ewing intestinal: presentación inusual en la edad pediátrica

Abstract Background: Ewing's Sarcoma (ES) is the second most common type of bone cancer, with an annual incidence of 2.9:100,000. Extraosseous cases represent 15%; however, there are no reported cases of ES located in the intestine in the pediatric population. Case report: We describe the case of a 14-year-old male patient, previously healthy, who started with an anemic syndrome, weight loss, and diaphoresis of 8 weeks of evolution. After visiting a physician, who documented the presence of anemia, the patient was referred to the National Institute of Pediatrics. Physical examination showed grade III-IV systolic murmur, splenomegaly, and pain in the left hemiabdomen with no irradiation. Computed axial tomography showed a mass-dependent on the peritoneum and intestinal loop. A biopsy of the lesion showed intestinal ES. The lesion was completely resected, and the patient was treated with chemotherapy and radiotherapy. Thirty months after diagnosis, the patient has no evidence of tumor activity. Conclusions: Extraosseous presentation of ES in pediatric age is rare. There are no reports of intestinal ES in the Latin American pediatric population, although eight case reports were found in adults. ES is curable by a combination of chemotherapy, radiotherapy, and surgery. The medical literature indicates that the extraosseous presentation should receive the same treatment as the osseous presentation, which can provide a survival rate of up to 70% if there is no evidence of metastasis (which most frequently is observed in the lung).

Resumen Introducción: El sarcoma de Ewing (SE) es el segundo tipo cáncer más común de hueso, cuya incidencia anual es de 2.9:100,000. Los casos extraóseos representan el 15%; sin embargo, no existen reportes en la literatura de casos de SE ubicados en el intestino en la población pediátrica. Caso clínico: Se describe el caso de un paciente de sexo masculino de 14 años, previamente sano, que inició con síndrome anémico, pérdida de peso y diaforesis de 8 semanas de evolución. Acudió con un médico, quien documentó la presencia de anemia y lo refirió al Instituto Nacional de Pediatría. A la exploración física presentaba soplo sistólico grado III-IV, esplenomegalia y dolor en hemiabdomen izquierdo sin irradiaciones. La tomografía axial computarizada mostró una masa dependiente del peritoneo y asa intestinal. La biopsia de la lesión reportó SE intestinal. Se resecó por completo la lesión y el paciente recibió tratamiento con quimioterapia y radioterapia. Después de 30 meses del diagnóstico, el paciente se encuentra sin datos de actividad tumoral. Conclusiones: La presentación extraósea del SE en edad pediátrica es rara. No existen reportes de presentación de SE intestinal en la población pediátrica latinoamericana, aunque se encontraron ocho reportes de caso en adultos. El SE es curable mediante la combinación de quimioterapia, radioterapia y cirugía. La literatura médica indica que la presentación extraósea debe recibir el mismo tratamiento que la ósea, lo cual puede proporcionar una sobrevida de hasta el 70% si no hay evidencia de metástasis (que ocurre más frecuentemente a pulmón).

Primary Extraskeletal Ewing Sarcoma of the Thoracolumbar Epidural Space: Rare Case Report in a Child / Sarcoma de Ewing extraesquelético primário no espaço epidural toracolombar: Raro relato de caso em criança

Introduction Ewing sarcomas are a family of tumors that can be of skeletal or extraskeletal origin. We report a rare case of a child with extraskeletal Ewing sarcoma in the thoracolumbar epidural space. Case Report The patient was a 1-year-old female child with sphincter alteration, flaccid paraplegia, and areflexia. A magnetic resonance imaging (MRI) scan showed a large extensive epidural lesion with compression of the dural sac in the D6­L2 segment, and a left paravertebral extension through the L1­L2 foramen. Laminotomy was performed, with subtotal resection of the lesion. The histopathological and immunohistochemical analyses indicated Ewing sarcoma. Due to the child's age, radiotherapy was not performed, only chemotherapy, due to the aggressiveness of the neoplasm. The patient showed rapid tumor recurrence and ended up dying. Discussion Extraskeletal Ewing sarcoma can appear in different locations in the body. They are aggressive tumors with local recurrence and distant metastases. In our case, a combination of MRI and positron-emission tomography­computed tomography scan presented a clearer result, especially in the presence of metastasis. In the histopathological analysis, small blue cells with a clear cytoplasm and indistinct nucleoli were observed. In the immunohistochemical analysis, CD99 (MIC2) expression is highlighted. The best treatment outcome would have been surgical resection with chemotherapy and radiotherapy. Conclusion We reported a rare case of thoracolumbar epidural Ewing sarcoma in which, despite surgery and chemotherapy, the tumor behaved very aggressively, leading to an unfavorable prognosis.

Introdução Os sarcomas de Ewing constituem uma família de tumores que podem ser de origem óssea ou extraesquelética. O caso aqui relatado é o de uma criança com raro sarcoma de Ewing extraesquelético no espaço epidural toracolombar. Relato de caso Criança do sexo feminino, de 1 ano de idade, com quadro de alteração de esfincter, paraplegia flácida, e arreflexia. O exame de ressonância magnética (RM) revelou volumosa lesão expansiva epidural com compressão do saco dural no segmento de D6-L2, e extensão paravertebral esquerda através do forame L1-L2. Realizou-se laminotomia, com ressecção subtotal da lesão. O exame histopatológico e a imunohistoquímica indicavam sarcoma de Ewing. Por conta da idade da criança, ela não foi submetida a radioterapia, apenas a quimioterapia, e, devido à agressividade da neoplasia, a paciente evoluiu com rápida recidiva tumoral e terminou falecendo. Discussão O sarcoma de Ewing extraesquelético pode aparecer em diferentes locais do corpo. São tumores agressivos com recorrência local e metástase à distância. Na investigação, a combinação de RM e tomografia por emissão de pósitrons­tomografia computadorizada apresenta melhor resultado, principalmente na presença de metástase. No exame histopatológico, observaram-se pequenas células azuis com citoplasma claro e nucléolos indistintos. Na imunohistoquímica, se destaca a expressão principalmente do CD99 (MIC2). A conduta com melhor resultado no tratamento seria ressecção cirúrgica, com quimioterapia e radioterapia. Conclusão Relatamos um caso raro de uma criança com sarcoma de Ewing epidural na coluna, e apesar da realização da cirurgia e de quimioterapia, o tumor se comportou de modo extremamente agressivo, o que levou a um prognóstico desfavorável.

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.

Respuesta patológica completa al tratamiento multimodal en sarcoma de Ewing del adulto / Pathological complete response after multimodal treatment of adult Ewing sarcoma

Resumen El sarcoma de Ewing es una neoplasia rara y altamente agresiva que afecta con cierta predilección adolescentes varones. La incorporación de terapia neoadyuvante y nuevas técnicas quirúrgicas ha mejorado la supervivencia. Presentamos el caso de un varón de 41 años con sarcoma de Ewing de pared torácica, quien recibió tratamiento multimodal consistente en quimio-radioterapia concurrente y tratamiento qui rúrgico, y alcanzó respuesta patológica completa. El sarcoma de Ewing rara vez se presenta en la edad adulta y, cuando lo hace, suele tener mal pronóstico. El tratamiento multimodal de pacientes mayores de 40 años ha probado mejorar los resultados oncológicos.

Abstract Ewing sarcoma is a rare and highly aggressive neoplasm that occurs most frequently in male adolescents. The incorporation of neoadjuvant therapy and new surgical techniques has improved survival. We present the case of a 41-year-old man diagnosed with Ewing sarcoma of the chest wall, whose tumor showed a pathological complete response to a multimodal treatment consisting of concurrent chemotherapy, radiotherapy, and surgical resection. Ewing sarcoma rarely occurs in adults, who generally have a worse prognosis. A multimodal approach for the treatment of patients older than 40 years has proven to improve oncological results.

Sarcoma de ewing nasosinusal en la infancia / Nasosinusalewing sarcoma in childhood / Sarcoma de ewing nasossinusal na infância

RESUMEN Introducción: dentro de los tumores de cabeza y cuello, el Sarcoma de Ewing es uno de los menos frecuente, afecta la mandíbula y la maxila, pero es más raro aún en tejidos blandos originados en la cavidad nasal o los senos paranasales; comparten características con los tumores neuroectodérmicos primitivos. Se presentan habitualmente en niños y adultos jóvenes, presentándose de inicio con una gran agresividad. Presentación de caso: Presentamos una niña de 4 años que acudió a consulta con una inflamación en el piso de fosa nasal derecha, sin fiebre, sin dolor, escasa rinorrea con obstrucción nasal derecha. Discusión: se realizó resonancia magnética visualizando una masa tumoral hacia el piso nasal derecho, de gran intensidad, isointenso y sin rotura de paredes del seno maxilar u otra estructura vecina. Se tomó muestra incisional para biopsia de la lesión que informa positivo a un sarcoma de Ewing. Conclusiones: se aplicó tratamiento con radioterapia y quimioterapia.

ABSTRACT Introduction: within the head and neck tumors, Ewing's Sarcoma is one of the least frequent, it affects the jaw and maxilla, but it is even more rare in soft tissues originated in the nasal cavity or sinuses; share characteristics with primitive neuroectodermal tumors. They usually occur in children and young adults, presenting themselves with great aggressiveness. Case presentation: We present a 4-year-old girl who went to the office with an inflammation in the floor of the right nostril, without fever, without pain, poor rhinorrhea with right nasal obstruction. Discussion: MRI was performed visualizing a tumor mass towards the right nasal floor, of great intensity, isointense and without rupture of the walls of the maxillary sinus or other neighboring structure. Incisional sample was taken for biopsy of the lesion that positively reports Ewing's sarcoma. Conclusions: treatment with radiotherapy and chemotherapy was applied.

RESUMO Introdução: nos tumores de cabeça e pescoço, o sarcoma de Ewing é um dos menos frequentes, afeta a mandíbula e a maxila, mas é ainda mais raro em tecidos moles originados na cavidade nasal ou seios da face; compartilham características com tumores neuroectodérmicos primitivos. Geralmente ocorrem em crianças e adultos jovens, apresentando-se com grande agressividade. Apresentação do caso: Apresentamos uma menina de 4 anos que foi ao consultório com inflamação no assoalho da narina direita, sem febre, sem dor, rinorréia ruim com obstrução nasal direita. Discussão: A RM foi realizada visualizando uma massa tumoral em direção ao assoalho nasal direito, de grande intensidade, isointensa e sem ruptura das paredes do seio maxilar ou de outra estrutura vizinha. Amostra incisional foi coletada para biópsia da lesão que relata positivamente o sarcoma de Ewing. Conclusões: o tratamento com radioterapia e quimioterapia foi aplicado.