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.

Extraskeletal Ewing sarcoma of thyroid gland: A case report / 中南大学学报(医学版)

Extraskeletal Ewing sarcoma is a rare event, and extraskeletal Ewing sarcoma of the thyroid gland is even rarer. It has non-specific clinical manifestation and difficulty in early diagnosis. The diagnosis mainly depends on histology and immunohistochemistry. It possesses the features of high malignancy, high rate of local recurrence, and distant metastasis. Currently, the aggressive multimodal treatment contains surgery, chemotherapy, and radiotherapy. This study presented a case of extraskeletal Ewing sarcoma arising in the thyroid gland of a 30-year-old woman, who presented with supraclavicular mass and sense of dysphagia obstruction in Department of Otolaryngology, Head and Neck Surgery, Second Xiangya Hospital, Central South University in 2018. Imaging studies demonstrated a cystic-solid mass in inferior of the left leaf of thyroid gland and in the posterior of the trachea and esophagus. The patient underwent localized tumor resection. The pathological diagnosis revealed that it was a small round cell tumor, and the immunohistochemistry results were considered to be extraskeletal Ewing sarcoma. Subsequently, the patient was given chemotherapy and local radiation therapy. There was no evidence of tumor recurrence or metastasis.

Sarcoma de Ewing óseo: Enfoque multidisciplinario y resultados oncológicos en 88 pacientes / Ewing sarcoma of the bone. Multidisciplinary approach and oncological results in 88 patients

El sarcoma de Ewing óseo es un tumor poco frecuente, agresivo, que afecta principalmente a niños y adultos jóvenes. Existe ausencia de registros en nuestro país respecto de la prevalencia de esta enfermedad, los esquemas de tratamiento utilizados y sus resultados. El objetivo fue analizar, en un grupo de pacientes con sarcoma de Ewing óseo tratados con quimioterapia y cirugía de conservación de miembro, las tasas de supervivencia global, de recurrencia local y los factores de riesgo oncológicos. Se incluyó a 88 pacientes. La edad media de la serie fue de 14.5 años y el seguimiento promedio de 8.8 años. La tasa de supervivencia global fue de 79.5% a los 2 años, de 69% a 5 años y de 64% a 10 años. Los factores pronósticos negativos asociados a menor supervivencia fueron: mala respuesta a la quimioterapia, edad ≥ de 16 años, localización central, y recurrencia local. En el análisis multivariable únicamente la respuesta a la quimioterapia tuvo significancia estadística. La tasa libre de recurrencia local a 2 y 5 años fue del 87%. La mala respuesta a la quimioterapia fue el único factor significativo para la recurrencia local. Consideramos que la cirugía de conservación de miembro asociada a quimioterapia pre y postoperatoria debe ser el tratamiento para el sarcoma de Ewing óseo, alcanzando de esta manera una supervivencia global a 5 años del 69%. En nuestra serie, la respuesta a la quimioterapia ha sido el factor pronóstico más relevante para supervivencia y recurrencia local.

Ewing sarcoma of the bone is a rare, highly aggressive tumor that typically affects children and young adults. In Argentina, the lack of Ewing's sarcoma registries reflects in the absence of information regarding prevalence, treatment protocols and patient´s outcome. The purpose of this study was to analyze, in a group of patients diagnosed with Ewing sarcoma of the bone, treated with chemotherapy and limb-conserving surgery, their overall survival rate, local recurrence rate, and oncological risk factors. A retrospective research was conducted between 1990 and 2017. Eighty-eight patients with Ewing sarcoma of the bone matched the inclusion criteria. Median age was 14.5 years and median follow-up was 8.8 years. Overall survival rate was 79.5%, 69% and 64% at 2, 5 and 10 years respectively. Negative prognostic factors, associated with less survival rate after univariate analysis, were: bad response to chemotherapy (tumoral necrosis 0-89%), age > 16 years-old, central tumor localization and local recurrence. Gender and tumor size were not significant prognostic factors. After multivariate analysis, response to chemotherapy remained statistical significant. Local recurrence-free survival rate at 2 and 5 years was 87%. Tumor response to chemotherapy (0-89%) was the only significant factor for local recurrence. We consider that limb-salvage surgery, with neoadjuvant and adjuvant chemotherapy, are the mainstays of treatment for Ewing's sarcoma, with an overall survival rate, at 5 years, of 69%. In this population, response to chemotherapy is the most relevant prognostic factor, being associated with both local recurrence and overall survival.

Delays in the health care system for children, adolescents, and young adults with bone tumors in Brazil / Atrasos no sistema de saúde para crianças, adolescentes e adultos jovens com tumores ósseos no Brasil

ABSTRACT Objective: To identify delays in the health care system experienced by children and adolescents and young adults (AYA; aged 0-29 years) with osteosarcoma and Ewing sarcoma using information from the Brazilian hospital-based cancer registries. Methods: Patient data were extracted from 161 Brazilian hospital-based cancer registries between 2007 and 2011. Hospital, diagnosis, and treatment delays were analyzed in patients without a previous histopathological diagnosis. Referral, hospital, and health care delays were calculated for patients with a previous histopathological diagnosis. The time interval was measured in days. Results: There was no difference between genders in overall delays. All delays increased at older ages. Patients without a previous histopathological diagnosis had the longest hospital delay when compared to patients with a previous histopathological diagnosis before first contact with the cancer center. Patients with Ewing sarcoma had longer referral and health care delays than those with osteosarcoma who had a previous histopathological diagnosis before first contact with the cancer center. The North and Northeast regions had the longest diagnosis delay, while the Northeast and Southeast regions had the longest treatment delay. Conclusion: Health care delay among patients with a previous diagnosis was longer, and was probably associated with the time taken for to referral to cancer centers. Patients without a previous histopathological diagnosis had longer hospital delays, which could be associated with possible difficulties regarding demand and high-cost procedures. Despite limitations, this study helps provide initial knowledge about the healthcare pathway delays for patients with bone cancer inside several Brazilian hospitals.

RESUMO Objetivo: Identificar atrasos no sistema de saúde em crianças e adolescentes e adultos jovens (AAJ; até 29 anos) com osteossarcoma e sarcoma de Ewing com informações dos registros de câncer de base hospitalar do Brasil. Métodos: Os dados dos pacientes foram extraídos de 161 registros de câncer de base hospitalar brasileiros entre 2007 e 2011. Os atrasos no hospital, no diagnóstico e no tratamento foram analisados em pacientes sem um diagnóstico histopatológico anterior. Os atrasos no encaminhamento, no hospital e no sistema de saúde foram calculados para pacientes com diagnóstico histopatológico anterior. O intervalo de tempo foi medido em dias. Resultados: Não houve diferença entre os sexos nos atrasos em geral. Todos os atrasos aumentaram na faixa etária mais velha. Os pacientes sem um diagnóstico histopatológico anterior apresentaram o atraso hospitalar mais longo em comparação com os pacientes com diagnóstico histopatológico anterior antes do primeiro contato com o centro de câncer. Os pacientes com sarcoma de Ewing apresentaram atrasos no encaminhamento e no sistema de saúde mais longos do que os com osteossarcoma, que apresentaram diagnóstico histopatológico anterior antes do primeiro contato com o centro oncológico. As regiões Norte e Nordeste apresentaram o atraso mais longo no diagnóstico, ao passo que as regiões Nordeste e Sul apresentaram o atraso mais longo no tratamento. Conclusão: O atraso no sistema de saúde entre os pacientes com diagnóstico anterior foi maior e provavelmente associado ao tempo de encaminhamento para os centros oncológicos. Os pacientes sem um diagnóstico histopatológico anterior apresentaram atrasos mais longos no hospital, o que pode ser associado a possíveis dificuldades com relação à demanda e aos procedimentos de alto custo. Apesar das limitações, nosso estudo ajuda a fornecer um conhecimento inicial sobre os atrasos no sistema de saúde para tratamento de pacientes com câncer em vários hospitais brasileiros.

De la patología estructural a la patología molecular: un modelo en el sarcoma de Ewing / From structural pathology to molecular pathology. A model of Ewing sarcoma

La existencia de un grupo distintivo de neoplasias de células redondas de hueso y partes blandas altamente malignas fue reconocida desde el siglo pasado. Este grupo de tumores de células redondas y pequeñas comprenden un conjunto heterogéneo de neoplasias pobremente diferenciadas formadas por células pequeñas, redondas u ovales con alto índice nuclear-citoplasmático, núcleos redondos con cromatina dispersa o agrupada y nucléolo poco evidente. En el año 1921, James Ewing, profesor de Patología de la Universidad de Cornell, USA, describió estas características histológicas en un tumor que denominó endotelioma difuso de hueso. Charles Oberling, en Paris, en el año 1928, denominó la neoplasia como Sarcoma de Ewing. De manera resumida podemos plantear la evolución de las técnicas diagnósticas aplicadas en el estudio de la siguiente forma: 1) Aplicacion de técnicas convencionales histológicas: H&E. 2) Histoquímica convencional: PAS, Carmin de Best. 3) Microscopía Electrónica. 4) Inmunohistoquímica: marcadores mesenquimales, neurales, epiteliales y otros. 5) Cultivos celulares. 6) Xenotrasplante de tumores. 7) Estudios citogenéticos convencionales: traslocaciones cromosómicas principales. 8) FISH. 9) Biología molecular: reordenamiento genético y tipos de genes de fusión. Se presenta una visión global de los distintos aspectos de la morfología, ultra estructura inmunohistoquímica, genética convencional y molecular asi como nuevas aproximaciones a la terapéutica de este tumor óseo.

Juvenile Ewing sarcoma presenting as a pelvic mass

A teen aged girl with rapidly developing pelvic mass, associated with weight loss and left leg weakness, was evaluated and found to have Ewing's sarcoma of non-osseous origin from pelvis, which is a rare situation. She was treated by a multidisciplinary approach by surgery, chemotherapy and rehabilitation by physiotherapy to effective response

Primary Ewing's sarcoma of the spine: case report / Sarcoma de Ewing primário da coluna vertebral: relato de caso

Primary malignant sarcomas of the spine are extremely rare. Because of biological heterogeneity, these tumors have variable sensitivity to radiation and chemotherapy. Adequate local control through complete tumor removal is an important therapeutic goal. However, aggressive resection of tumors in the spinal column must be coupled with restoration of spinal column stability and minimization of neural deficits. The balance of these factors makes treatment of primary sarcomas of the spine challenging, and dictates an individual approach to treatment. We report on a 18 years old man with primary Ewing's sarcoma of the nonsacral spine. The clinical picture and imaging characteristics were analyzed as well as the management modalities and outcome.

Sarcomas malignos primários da coluna são extremamente raros. Devido sua heterogeneidade biológica, estes tumores apresentam sensibilidade variada à radioterapia e à quimioterapia.O controle local adequado através da ressecção tumoral completa é um objetivo terapêutico importante. Contudo, a ressecção completa dos tumores da coluna vertebral deve ser realizada com a restauração da estabilidade espinhal e minimização do déficit neurológico. O equilíbrio entre estes fatores torna o tratamento dos sarcomas da coluna desafiador, exigindo-se uma abordagem individualizada para o tratamento de cada paciente. Relatamos um caso de sarcoma de Ewing da coluna não-sacral em um homem de 18 anos. O quadro clínico e características de imagem foram analisados, bem como a modalidades de manejo e o prognóstico.

Ewing's sarcoma of the nose

Nasal bleeding is a common complaint familiar to all otolaryngologists. But, sino-nasal primary Ewing's Sarcoma is extremely rare. This is a case of a 16-year old male patient who suffers from epistaxis, in addition to his left eye proptosis. After examination, it has been revealed that he had a nasal mass in the left side of his nose. The case was managed initially as a vascular mass, but then, under surgery, a frozen section was subjected to examination using histology; the examination revealed the existance of malignant cells. After all, definite histopathological diagnosis by immunohistochemical stains proved it to be Ewing's Sarcoma

The possible cost effectiveness of peripheral blood stem cell mobilization with clophosphamide and the late addition of G-CSF

The purpose of this study was to develop a cost-effective protocol for the mobilization of peripheral blood stem cells (PBSC) in patients with malignancy. Thirty consecutive patients were randomized to mobilize PBSC with the late addition of a standard 250 microg dose of G-CSF (Neutrogen) from day 8 or early addition of the same dose of G-CSF from day 2, following cyclophosphamide (CY) 4 g/m2. The median yield of CD34+ cells from evaluated patients was 7.87 x 10(6)/kg (range, 2.06-27.25), collected in a median of four apheresis (range, 2-9). Target CD34 + cell doses > or = 2.0 x 10(6)/kg were achieved in all patients able to be evaluated. There were no statistically significant differences in CD34+ cell yields or toxicities. Overall engraftment occurred with median days to neutrophils > or = 0.5 x 10(9)/L or platelets > 20 x 10(9)/L of 11 and 17 days, respectively. However, the duration of G-CSF administration was markedly shorter in the late use of G-CSF group than in the early use of G-CSF group, with a median of 9 days compared with 15 days (p>0.001). PBSC harvesting after priming with CY plus delayed use of G-CSF made it a safe and cost-effective procedure.

Primitive peripheral neuroectodermal tumors Mayagüez Medical Center experience

Primitive neuroectodermal or neuroepithelial tumors are names used to describe neoplasias composed of undifferentiated cells resembling germinal cells of the embryonic neural tube. These tumors are small round cell malignancies of the neural crest origin arising outside the central and sympathetic nervous system. They are described as peripheral and central neuroectodermal tumors related to the original malignant cell. A great number of tumors are described under this classification in spite of the fact that there is no an universal acceptance that these small-cell neoplasms, regardless of their primary site, are derived from immature neuroectoderm tissue. Because one tumor resembles others in terms of its phenotypic expression, multiple specific studies such as clinical profile, ultrastructural, immunocytochemical, and cytogenetic features should be studied, since no single clinical or laboratory marker is by itself diagnostic. However, there is a chromosomal reciprocal translocation, t(11;22)(q24;q12), which is unique to Primitive Neuroectodermal Tumor (PNET)

Tumor de Ewing: análise do tratamento em doze pacientes do Hospital do Câncer de Pernambuco / Ewing's Tumor: surgical treatment of twelve patients in the Hospital do Câncer de Pernambuco

Os autores relatam os resultados do tratamento em doze pacientes portadores de tumor de Ewing no Hospital do Câncer de Pernambuco, no período de 1991 a 1996, concluindo a prevalência no sexo masculino do tumor e a maior incidência nas duas primeiras décadas de vida. A principal localizaçÝo encontrada foi o úmero, seguida pelo fêmur e tíbia. A conduta adotada com relaçÝo ao tratamento foi quimioterapia, cirurgia com preservaçÝo do membro ou amputaçÝo seguido de quimioterapia, havendo uma sobrevida, em cinco anos, de 66,75 dos pacientes tratados

Sarcoma de células redondas de la gotera costovertebral del adolescente y del adulto joven / Round cell sarcomas of the costovertebral roof in adolescent and young patients

El objetivo de este trabajo es mostrar la evolución de seis pacientes jóvenes portadores de tumores poco diferenciado de la gotera costovertebral durante siete años. Se hará un breve comentario de esta entidad poco,frecuente en el campo de la oncologia. Analizaremos las dificultades diagnósticas así como los diagnósticos diferenciales más comúnes. Por último, comentaremos las opciones terapéuticas que se utilizaron en estos casos y su relación con la bibliografía

Traitement conservateur des tumeurs osseuses primitives malignes de l'enfant Resultats preliminaire a' propos de 14 cas

Progress of neoadjuvant chemotherapy has trasformed prognosis of squelettal malignant tumor in infants. This has led to a great improvement in limb sparing surgery and in disease-free survival. The authors report their experience of limb salvage about their 14 first cases. The nature of tumor is osteosarcoma in 7 cases and ewing sarcoma in the other 7 cases. This review allows some conslusions: 1/ necesity of multidisciplinary cooperation in management of such patients. 2/ the worst prognosis of Ewing sarcoma. 3/ inter relation between tumor sensibility to chemotherapy and survival of patients. 4/ fiability of autograft in intercalary reconstruction and good fonctionnal result of knee reconstruction with massive prothesis

Avanços em oncologia na última década e perspectivas futuras em tumores sólidos pediátricos / Advances in oncology on the last decada and future perspectives in pediatric solid tumors

Nos últimos anos o prognósticos das crianças com câncer melhorou sensivelmente. Isto foi devido a tratamentos padronizados por meio de grupos cooperativos. Atualmente säo conhecidos fatores prognósticos que permitem orientar o tratamento, reduzindo-os em casos de melhor prognóstico e intensificando-os nos casos de pior prognóstico. O objetivo atual näo é somente curar a criança mas curar com o menor efeito colateral

Experiencia con el tratamiento del sarcoma de Ewing en el Hospital Nacional de Niños / The treatment of Ewing sarcoma at the National Children's Hospital

Entre 1965 y 1987 han sido tratados en el Hospital Nacional de Niños 15 pacientes con diagnóstico histológico de sarcoma de Ewing, 8 hombres y 7 mujeres, con una edad promedio de 9 años. Los signos clínicos que presentaron fueron dolor, tumor y fiebre y confundieron y retardaron el diagnóstico en 6 de ellos. El fémur fue sitio de origen en 27%, pelvis 50%, húmero, peroné y tibia en el 13% respectivamente y escápula y tejido blandos en 6%. Diez casos presentaban el tumor localizado, cuatro ingresaron con metástasis a pulmon y uno extraóseo, diseminado. Siete de los casos ingresaron entre 1965 y 1977 y fueron tratados con radioterapía y en algunos casos quimioterapia con C Act y V, en ninguno se hizo resección del tumor; de este grupo sobreviven dos, uno de ellos curado espontaneamente. Del grupo tratado a partir de 1978 con radioterapia y quimioterapia con 5 drogas (V A C Act y MTX) por 18 meses y resección del tumor en 6 casos, sobreviven 4 con un tiempo promdeio en remisión de 83 meses. Se considera que la quimioterapia múltiple, la resección quirúrgica amplia y radioterapia postoperatoria han mejorado el pronóstico de este tumor en nuestro medio. La presencia de metástasis al ingreso constituye un signo de mal pronóstico, lo mismo que la recidiva local, pero otros esquemas terapeputicos y el uso de quimioterapia intra arterial pueden cambiar las perspectivas en estos casos