Sarcoma de Ewing atípico: relato de caso e achados de imagem / Atypical Ewing's Sarcoma: a case report and imaging findings

Objetivo: Apresentar um caso atípico de Sarcoma de Ewing, devido a sua dificuldade diagnóstica. Relato do caso: Paciente masculino, 14 anos, com diagnóstico histopatológico e pela imuno-histoquímica de Sarcoma de Ewing de origem clavicular. A tomografia computadorizada revelou formação expansiva localizada na região supraclavicular direita com epicentro em terços médio e extremidade medial da clavícula ipsilateral, determinando lesão lítica nos terços proximal e médio da clavícula direita, com extensa reação periosteal e pequenas áreas de descontinuidade da cortical no terço proximal da clavícula. A ressonância magnética confirmou lesão expansiva centrada nos terços médio e proximal da clavícula direita, apresentando preservação da superfície articular da articulação esternoclavicular sem sinais de invasão vascular ou do plexo braquial, sem evidências de envolvimento das estruturas do plexo braquial. Realizadas sessões de quimioterapia para diminuição das dimensões tumorais e, em sequência, submetido à claviculectomia. Discussão: O diagnóstico por imagem, incluindo raio x, tomografia computadorizada e ressonância magnética, podem ser usados comumente no diagnóstico dessa doença. Os aspectos radiográficos são de extrema importância para o diagnóstico, apesar da aparência radiológica de várias lesões ósseas serem semelhantes, o Sarcoma de Ewing é caracterizado por uma lesão osteolítica altamente agressiva, mal delimitada, associado à reação periosteal, dando origem aos denominados "triangulo de Codman", "raios de sol" e em "casca de cebola". A tomografia computadorizada, assim como a ressonância magnética, são exames úteis para estadiamento dessas lesões, definindo com mais clareza as alterações dos componentes de partes moles, alterações corticais e a presença de metástases. A tomografia computadorizada caracteriza o sarcoma como lesão expansiva, com captação heterogênea ao meio de contraste e extensão da lesão para partes moles, já a ressonância magnética descreve como lesão de baixo sinal de T1, com realce heterogêneo ao contraste e sinal heterogêneo em T2. Conclusão: No paciente em questão, as imagens revelaram informações importantes para o diagnóstico e prognóstico da lesão. Portanto, os radiologistas devem estar atentos aos achados de imagem que possam estar fora do padrão usual da doença

Objective: To present an atypical case of Ewing Sarcoma, due to its diagnostic difficulty. Case report: Male patient, 14 years old, with histopathological and immunohistochemical diagnosis of Ewing Sarcoma of clavicular origin. Computed tomography revealed an expansive formation located in the right supraclavicular region with an epicenter in the middle third and medial end of the ipsilateral clavicle, determining a lytic lesion in the proximal and middle thirds of the right clavicle, with extensive periosteal reaction and small areas of cortical discontinuity in the proximal third of the clavicle. Magnetic resonance imaging confirmed an expansive lesion centered in the middle and proximal thirds of the right clavicle, showing preservation of the articular surface of the sternoclavicular joint without signs of vascular or brachial plexus invasion, without evidence of involvement of the brachial plexus structures. Chemotherapy sessions were carried out to reduce tumor size and, subsequently, he underwent clavicularectomy. Discussion: Diagnostic imaging, including x-ray, computed tomography and magnetic resonance imaging, can be commonly used in the diagnosis of this disease. Radiographic aspects are extremely important for diagnosis, despite the radiological appearance of several bone lesions being similar, Ewing's Sarcoma is characterized by a highly aggressive, poorly delimited osteolytic lesion, associated with periosteal reaction, giving rise to the so-called "triangle of Codman", "rays of sun" and "onion skin". Computed tomography, as well as magnetic resonance imaging, are useful exams for staging these lesions, defining more clearly changes in soft tissue components, cortical changes and the presence of metastases. Computed tomography characterizes the sarcoma as an expansile lesion, with heterogeneous contrast uptake and extension of the lesion to soft tissues, whereas magnetic resonance imaging describes it as a low-signal T1 lesion, with heterogeneous contrast enhancement and heterogeneous signal on T2. Conclusion: In the patient in question, the images revealed important information for the diagnosis and prognosis of the injury. Therefore, radiologists must be alert to imaging findings that may be outside the usual pattern of the disease

Clinical characteristics of 662 patients with pancreatic neuroendocrine tumors receiving antitumoral therapy.

Pancreatic neuroendocrine neoplasia constitute an important subentity of the gastroenteropancreatic neuroendocrine neoplasms accounting for up to 15% of all neuroendocrine neoplasm. Prognosis and oncological behavior of pancreatic neuroendocrine tumors (pNETs) is extremely heterogenous and dependent on the specific tumor stage and differentiation. However, systematic data on the specific epidemiology of pNET are scarce. We identified 662 patients with pNET within the Oncology Dynamics database (IQVIA). Patients were derived from 4 European countries (Germany, France, UK, Spain), 3 Asian countries (Japan, China, South Korea) and 2 South American countries (Mexico and Brazil) and with regard to major patient and tumor related characteristics including patients' age, sex, tumor stage, tumor grading, and differentiation. The mean age of the study cohort was 62 years (SD 12 years) with 53.9.1% of all patients being male. The majority of patients had an Eastern co-operative of Oncology Group 1 performance status (63.3%). The most common Union international contre le cancer tumor stage was stage IV (85%) with liver metastases (89.0%) representing the most common site of extra-pancreatic tumor manifestation. The majority of all patients displayed well or moderate tumor differentiation (9.6% of patients had a Ki-67 expression below 2%. 67.6% of pNET patients had a Ki-67 expression between 2 and 20% and 22.8% of patients showed an expression above 20%). At time point of diagnoses, 93.1% of patients were classified as inoperable. Of note, 93.9 % of patients received systemic anti-tumoral therapy in palliative intention, while treatment was administered in 1.4 % of cases in neoadjuvant and in 4.7% of cases in in an adjuvant setting. Biological therapy was applied to 39.4% of patients, followed by targeted therapies (31.4%) and chemotherapy. Pancreatic neuroendocrine neoplasia are diagnosed in advanced tumor stages, globally. Systemic treatment was the most commonly used treatment modality. Such data may help to better understand the specific epidemiology of pNET worldwide.

Surgery or active surveillance for pNETs < 2 cm: Preliminary results from a single center Brazilian cohort.

BACKGROUND AND OBJECTIVES: Incidence of pancreatic neuroendocrine tumors (pNETS) seems to be rising over the years, with many cases incidentally diagnosed. Surgery and active surveillance are current treatment modalities for small pNETS. We review our institutional series and compare outcomes for small asymptomatic and nonfunctioning tumors. METHODS: This retrospective cohort study included patients with 2 cm or less and well differentiated pNETS at a single Brazilian Cancer Center. From 2002 to 2020, patients received active surveillance or surgery as a treatment strategy. Short and long-term results were compared. RESULTS: Sixty-four patients were included, 41 in surgical strategy and 23 in the active surveillance approach. Baseline group characteristics were comparable. More patients on active surveillance underwent abdominal magnetic resonance imaging (MRI) and had tumors located in the pancreatic head (41% vs. 17%, p = 0.038). Minimally invasive procedure was chosen in 80.1% of the surgical patients. No patient died after surgery. Median follow-up period was 38.6 and 46.4 months for active surveillance and surgery cohorts, respectively. No difference in disease progression rate was observed. CONCLUSION: Both approaches seem to be safe for small pNETs. Long-term outcome and quality of life should be considered when discussing such options with patients.

A case of metastatic non-teratoid ocular medulloepithelioma in an adult horse

This case reports an eight-years-old horse that presented a unilateral tumor in the left ocular orbital region diagnosed on the basis of clinical signs, gross anatomopathological and histopathological investigations. The affected area was exenteration and submitted to histopathological evaluation and was diagnosed as a non-teratoid medulloepithelioma. This is a rare ocular neoplasm that usually affects young individuals – and was composed by neuroepithelium arranged in tubules and Flexner-Wintersteiner rosettes without presence of heteroplastic components. The animals died and was submitted to post-mortem evaluation, revealing presence of medulloepithelioma metastases at the internal region of the head, lymph nodes, lungs, and liver. This neoplasm usually presents a benign frame; however, the case herein described shows that it can be highly malignant and aggressive since it can cause extensive lesions and spread metastases to visceral organs.

A case of metastatic non-teratoid ocular medulloepithelioma in an adult horse

This case reports an eight-years-old horse that presented a unilateral tumor in the left ocular orbital region diagnosed on the basis of clinical signs, gross anatomopathological and histopathological investigations. The affected area was exenteration and submitted to histopathological evaluation and was diagnosed as a non-teratoid medulloepithelioma. This is a rare ocular neoplasm that usually affects young individuals and was composed by neuroepithelium arranged in tubules and Flexner-Wintersteiner rosettes without presence of heteroplastic components. The animals died and was submitted to post-mortem evaluation, revealing presence of medulloepithelioma metastases at the internal region of the head, lymph nodes, lungs, and liver. This neoplasm usually presents a benign frame; however, the case herein described shows that it can be highly malignant and aggressive since it can cause extensive lesions and spread metastases to visceral organs.(AU)

Ocular teratoid medulloepithelioma in a northern red-shouldered macaw: case report and literature review.

A 4-mo-old northern red-shouldered macaw (Diopsittaca nobilis) was admitted to the veterinary hospital of the Arruda Câmara Zoo, in the State of Paraiba, Brazil, for investigation of an orbital mass. Given rapid progression and lack of response to treatment, the bird was euthanized, and an autopsy was performed. Histologically, the mass consisted of a retrobulbar invasive tumor characterized by tubular and rosette-like structures, with interspersed heteroplastic tissues, such as aggregates of neuroglial cells and islands of hyaline cartilage. The tumor was immunopositive for pancytokeratin, GFAP, NSE, and S100. These findings were compatible with an ocular teratoid medulloepithelioma, a neoplasm best described in humans but also reported rarely in young cockatiels and African Grey parrots.

Meduloepitelioma de cuerpo ciliar. Reporte de un caso y revisión de la literatura / Ciliary body medulloepithelioma. Case report and literature review

Introducción: Se presenta el caso de un paciente de 10 años de edad a quien se le realizó el diagnóstico de meduloepitelioma de cuerpo ciliar con posterior enucleación. Objetivo: Presentar las principales características clínicas, abordaje diagnostico y terapéutico de un paciente con meduloepitelioma de cuerpo cilar, realizando una revisión de la literatura existente de esta patología. Diseño del estudio: Reporte de caso. Resumen del caso: Paciente masculino de 10 años de edad que presentó perdida de agudeza visual asociada a dolor ocular, al examen oftalmológico se encontró masa en cámara anterior. Se realizó biopsia de lesión iridiana diagnosticándose meduloepitelioma de cuerpo ciliar con posterior enucleación. Conclusión: Se presentó el caso de un niño con meduloepitelioma de cuerpo ciliar, exponiéndose las manifestaciones clínicas, su diagnóstico y tratamiento, así como la revisión de la literatura existente.

Background: We present the case of a 10-year-old patient who underwent a diagnosis of ciliary body medulloepithelioma with subsequent enucleation. Objective: To present the main clinical characteristics, diagnostic and therapeutic aproach of a patient with a ciliary body medulloepithelioma, carrying out a review of the existing literature of this pathology. Study design: Case report. Case summary: A 10-year-old male patient presented with loss of visual acuity associated with ocular pain. Ophthalmological examination revealed a mass in the anterior chamber. A biopsy of an iris lesion was made, diagnosing a ciliary body medulloepithelioma with subsequent enucleation. Conclusion: The case of a child with ciliary body medulloepithelioma was presented, exposing the clinical manifestations, their diagnosis and treatment, as well as the review of the existing literature.

Tumor neuroectodérmico primitivo de cavidad nasal / Primitive neuroectodermal tumor nasal cavity

Los tumores neuroectodérmicos primitivos son tumores muy infrecuentes. Derivan de células embrionarias de la cresta neural, y comparten características con los sarcoma de Ewing extraóseos. Se presentan habitualmente en niños y adultos jóvenes, su ubicación más frecuente es en la región toracopulmonar, abdomen y extremidades. Son tumores que presentan una agresividad importante, lo que condiciona un pronóstico sombrío. La ubicación en fosas nasales-cavidades perinasales es anecdótica, existiendo el reporte de tan sólo un caso en la literatura mundial de ubicación en cavidad nasal.

Primitive neuroectodermal tumors are rare malignancies arising from embryonic neural crest cells. They share characteristics with extraosseous Ewing sarcoma. They usually occur in children and young adults and the most common location is the thoracopulmonary region, abdomen and extremities. These tumors have significant aggressiveness, which determines their poor prognosis. The location in nasal fossa-paranasal cavities is anecdotal. Until now only one previous case has been reported in the located in the nasal fossa.

Asociación entre diastematomielia y meduloepitelioma: reporte de un caso y revisión de la literatura / Association between diastematomyelia and medullo epithelioma: case report and literature review

Aim: Case report of association between diastematomyelia and medulloepithelioma. Method: 14-year-old patient with lower back pain and recent neurological deficit in extremities. CT and MRI scans of the thoracolumbar spine revealed a diastematomyelia. Intraoperative examination confirmed the presence of a spinal dysraphism and associated tumor, which was almost completely resected. Result: Histopathological and immunohistochemical findings were consistent with medulloepithelioma. Her postoperative course was uneventful. Conclusion: Diastematomyelia may manifest during adolescence as lower back pain and neurological deficit. The association of this malformation with a neoplasia is extremely rare; the present case describes concomitance with medulloepithelioma.

Reporte de un caso de asociación de diastematomielia y meduloepitelioma. Método: Paciente de 14 años con dolor lumbar y déficit neurológico en extremidades de reciente instalación. En TAC y RNM de columna dorsolumbar se pesquisa una diastematomielia. La exploración intraoperatoria, comprueba la presencia de una disrafia espinal y un tumor asociado al defecto, que se reseca casi en su totalidad. Resultado: Los hallazgos histopatológicos e inmunohistoquímicos son concordante con meduloepitelioma. La paciente tuvo un postoperatorio satisfactorio. Conclusión: La diastematomielia se puede manifestar durante la adolescencia como dolor lumbar y déficit neurológico. La asociación de esta malformación, con una neoplasia es muy poco frecuente, el presente caso describe la concomitancia con un meduloepitelioma.

Tumor neuroectodérmico primitivo do terceiro ventrículo associado a metástase leptomeníngea / Primitive Neuroectodermal Tumor of the Third Ventricle Associated with Leptomeningeal Metastases

Os tumores neuroectodérmicos primitivos (PNET) são tumores malignos, não diferenciados, raramente apresentados na idade adulta, principalmente os de localização supratentorial. Apresentamos neste artigo o caso de um paciente de 19 anos, que nos chegou transferido de outro hospital com o diagnóstico de hidrocefalia. A existência do PNET associado a carcinomatose leptomeníngea foi comprovada como causa da hidrocefalia.

Primitive neuroectodermal tumors (PNET) are malignant tumors exceptionally present in adulthood, especially those with supratentorial location. In this article, we present the case of a young man who was transfer fromanother institutionwith the diagnosis of hydrocephalus; during his stay, we corroborated the presence of supratentorial PNET associated to leptomeningeal carcinomatosis that was a cause of hydrocephalus.

Tumor neuroectodérmico primitivo abdominal: presentación de un caso / Abdominal primitive neuroectodermal tumor: case report

El tumor neuroectodérmico primitivo es una lesión muy rara y representa una de las neoplasias malignas más indiferenciadas. Se manifiesta por una complicada diversidad histológica y una expresión genética compleja, con clínica variada siempre maligna; se informan casos en diferentes localizaciones. Se presenta un paciente masculino de 13 meses de edad con distensión abdominal; en un ultrasonido abdominal se le detectó una imagen tumoral heterogénea que medía 86mm, no se definía el órgano de origen, que estaba acompañado de ascitis de moderada cuantía. En la biopsia se definió un tumor de células redondas, de alto grado de malignidad, con patrón de Ewing/tumor neuroectodérmico primitivo extraóseo de localización abdominal. Recibió quimioterapia sistémica e intratumoral, lo que hizo posible la resección quirúrgica de la lesión. En el postoperatorio tardío aparecieron imágenes nodulares en fondo de saco, partes blandas, lóbulo izquierdo hepático e hidronefrosis bilateral por diseminación de la enfermedad(AU)

Biomarcadores moleculares y su asociación con la supervivencia en niños con astrocitomas / Molecular biomarkers and their association with survival in children with astrocytomas

Los tumores del sistema nervioso central (SNC) representan los tumores sólidos más frecuentes en la edad pediátrica, los tumores del tallo suponen un 10% al 25%, y de ellos, los tumores difusos intrínsecos del puente (TDIP) presentan infiltración difusa en su patrón de crecimiento; el 95% de los niños mueren a causa de la enfermedad dentro de los tres primeros años con una mediana de supervivencia de 4 a 15 meses. Existen biomarcadores que se han puesto de manifiesto mediante técnicas de inmunohistoquímica. Objetivo: Determinar la asociación entre los marcadores tumorales Bcl2, CD133, p53 y Ki67 con la histología y la supervivencia de pacientes con tumor difuso intrínseco del puente. Pacientes y métodos: Se realizó un estudio ambilectivo, longitudinal, descriptivo de pacientes diagnosticados con TDIP en un período de 6 años con tipificación de los marcadores CD133, p53, Ki67, Bcl-2 mediante inmunohistoquímica, se analizó la asociación de dichos marcadores con la estirpe histológica y la supervivencia de los pacientes. Resultados: Se incluyeron en total 15 pacientes; por histología 11 (73%) tenían gliomas, y 4 (26.6%) presentaban tumores neuro ectodérmicos primitivos (TNEP). De los gliomas, 8 (72%) eran de bajo grado y 3 (28%) de alto grado, el marcador p53 estuvo sobre expresado en 8 de 14 pacientes (57%), p = 0.3802; Ki67 dio positivo en 7 de 14 pacientes (50%) p = 0.7363; el CD133 no presentó sobre expresión en ninguno de los enfermos, en tanto que Bcl-2 se encontró alterado en 9 de 14 sujetos (64%), p = 0.4858. La mediana de supervivencia en estos pacientes es de 13 meses. Conclusión: El biomarcador que muestra una asociación significativa con la supervivencia es Ki67, lo queda pie a la ideación de medidas terapéuticas de forma individualizada...

Supratentorial primary neuroectodermal tumor (PNET) inside the third ventricle in adult: an rare case report / Tumor neuroectodérmico primário (PNET) supratentorial no terceiro ventrículo em adulto: um raro relato de caso

The PNET of CNS are considered malignant undifferentiated tumors, and it represents about 2,8% of all tumors found on infants and teenagers, more rarely found on adults. In the present article will report the case of a patient, male, 23 years-old, with nodular lesion inside the third ventricle, admitted on emergency room with acute intense headache, drowsiness, vomiting and visual clouding, started three days before. Although there have been advances in diagnosis and treatment of PNET in children, few publications were found on the efficiency of available treatment options on adults. In our patient the lesion was completely removed by a anterosuperior interhemispheric transcallosal craniotomy, and subsequently diagnosed as PNET by anatomopathological. Postoperative hydrocephaly was installed and reverted with a ventriculoperitoneal shunt, with clinical and neurological status improvement. The patient died 18 months after diagnosis, due to respiratory hospitalar infection...

Os PNET do SNC são considerados tumores malignos indiferenciados e representam cerca de 2,8% de todos os tumores encontrados em crianças e adolescentes, sendo mais raramente encontrados em adultos. Neste presente artigo, relatamos o caso de um paciente, do sexo masculino, com 23 anos de idade, com lesão nodular dentro do terceiro ventrículo, que foi admitido na emergência com cefaleia intensa e aguda, sonolência, vômitos e turvação visual, iniciados três dias antes da admissão. Embora tenha havido avanços no diagnóstico e tratamento de PNET em crianças, poucas publicações foram encontradas sobre a eficiência de opções de tratamento disponíveis em adultos. No nosso paciente a lesão foi completamente removida por uma craniotomia ântero-transcalosa inter-hemisférica; posteriormente, ele foi diagnosticado como PNET por meio do exame anatomopatológico. A hidrocefalia instalada no pós-operatório foi revertida com uma derivação ventrículo-peritoneal, com melhora do quadro clínico e neurológico. O paciente morreu 18 meses após o diagnóstico, em decorrência de uma infecção hospitalar respiratória...

Tumor neuroectodérmico primitivo renal: presentación de una paciente / Primitive neuroectodermal tumor of the kidney: report of a patient

Las neoplasias renales representan entre el 2-3 por ciento del total de las neoplasias; entre ellas el tumor neuroectodérmico primitivo es poco frecuente. Se presenta el caso de una paciente femenina de 28 años de edad con antecedentes de asma bronquial que, accidentalmente, notó un aumento de volumen del lado izquierdo del abdomen, por lo que acudió al médico. Después de algunos estudios se le planteó el diagnóstico presuntivo de tumor renal izquierdo y se realizó una nefrectomía radical izquierda; en el postoperatorio la paciente presentó una evolución favorable. El informe histológico informó un tumor neuroectodérmico primitivo renal(AU)