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1.
Medicine (Baltimore) ; 100(2): e24038, 2021 Jan 15.
Artigo em Inglês | MEDLINE | ID: mdl-33466151

RESUMO

RATIONALE: Prostatic sarcoma (PS) is a very rare malignant tumor that accounts for <0.1% of prostate malignancies, and Ewing's sarcoma is an extremely rare form of PS. PATIENT CONCERNS: We reported on a 64-year-old patient with PS and a 36-year-old patient with Ewing's sarcoma, both of whom were examined by contrast-enhanced ultrasonography (CEUS) before surgery. DIAGNOSES: The 2 cases were proven to be prostatic stromal sarcoma, which was confirmed by imaging manifestations and histopathological findings. INTERVENTIONS: The 64-year-old patient underwent radical prostatectomy, and the 36-year-old patient underwent chemotherapy combined with local radiotherapy. OUTCOMES: PS showed diffuse enlargement of the prostate on sonography, and the necrotic liquefying area within the large vessels could be clearly displayed by CEUS. CEUS can be advocated as a valuable noninvasive and safe imaging diagnosis method for PS.


Assuntos
Neoplasias da Próstata/diagnóstico por imagem , Neoplasias da Próstata/patologia , Sarcoma/diagnóstico por imagem , Sarcoma/patologia , Adulto , Humanos , Masculino , Pessoa de Meia-Idade , Prostatectomia , Neoplasias da Próstata/diagnóstico , Neoplasias da Próstata/cirurgia , Sarcoma/diagnóstico , Sarcoma/cirurgia , Sarcoma de Ewing/patologia , Sarcoma de Ewing/cirurgia , Ultrassonografia
2.
Rev. bras. ortop ; 55(5): 649-652, Sept.-Oct. 2020. graf
Artigo em Inglês | LILACS | ID: biblio-1144209

RESUMO

Abstract Primary Ewing sarcoma in the spine is very rare, and the treatment for it is multidisciplinary. There is no consensus regarding the optimal method of local control; however, en bloc resection is associated with an improvement in survival rates. The authors report a case of a 5-year-old girl who initially presented low back pain, and was diagnosed with Ewing sarcoma after being submitted to imaging studies by radiography, magnetic resonance and bone biopsy. A spondylectomy was performed in accordance with the Euro Ewing protocol. At the three-year follow-up, the patient had no restrictions regarding her daily activities, and there has been no evidence of recurrence to date.


Resumo O sarcoma de Ewing da coluna vertebral é raro, e seu tratamento é multidisciplinar. Não há consenso sobre o método ideal de controle local do tumor; no entanto, a ressecção em bloco com margens negativas está associada a uma melhora da sobrevida. Os autores relatam um caso de uma paciente de 5 anos do sexo feminino que inicialmente se apresentou com dor lombar, tendo sido diagnosticada com sarcoma de Ewing de acordo com o estudo imagiológico por radiografia, ressonância magnética e biópsia óssea. A paciente foi submetida a vertebrectomia após quimioterapia, de acordo com o protocolo Euro Ewing. Aos três anos de seguimento, não apresentou restrições nas atividades da vida diária, e, até o momento, não houve evidência de recidiva.


Assuntos
Humanos , Feminino , Pré-Escolar , Sarcoma de Ewing/cirurgia , Neoplasias da Coluna Vertebral , Biópsia , Espectroscopia de Ressonância Magnética , Osteossarcoma/cirurgia , Dor Lombar , Vértebras Lombares , Neoplasias
3.
Hinyokika Kiyo ; 66(9): 297-302, 2020 Sep.
Artigo em Japonês | MEDLINE | ID: mdl-32988166

RESUMO

A woman in her 40s visited our hospital for further examination and treatment of a left renal tumor. Imaging studies showed that the patient had a large left renal tumor, 10.5 cm in diameter, which contained foci of necrosis. Open left radical nephrectomy was performed. In pathological examination, hematoxylin-eosin staining showed uniform small round cells with alveolar growth. Immunohistochemistry findings showed that CD99 and NKX2. 2 were positive, and synaptophysin was focally positive. Fluorescence in situ hybridization confirmed (11 ; 22) (q24 ; q12) chromosomal translocation which led to diagnosis of primary Ewing sarcoma of the kidney. Two months after the surgery, new tumors were found inside and outside of the left psoas muscles and in the left paracolic gutter. Six courses of chemotherapy were administered with VDC (vincristine/doxorubicin/cyclophosphamide) and IE (ifosfamide/etoposide). After completing the combined chemotherapy, the recurrent tumors disappeared completely on imaging. To prevent further recurrence, external radiation was administered to the left retroperitoneal region. About 16 months after the surgery, numerous new tumors appeared in the left retroperitoneal, left pleura, and erector spinae muscles. Chemotherapy was resumed following radiotherapy, and then trabectedin was administered. However, she eventually died of progressive disease at 26 months after the surgery.


Assuntos
Sarcoma de Ewing/diagnóstico por imagem , Sarcoma de Ewing/cirurgia , Sarcoma de Ewing/terapia , Neoplasias de Tecidos Moles , Feminino , Humanos , Ifosfamida , Hibridização in Situ Fluorescente , Recidiva Local de Neoplasia
4.
Ideggyogy Sz ; 73(7-08): 286-288, 2020 Jul 30.
Artigo em Inglês | MEDLINE | ID: mdl-32750246

RESUMO

Intracranial localization of Ewing's sarcoma is considerably very rare. Herein, we present clinical and neuroimaging findings regarding a 4-year-old boy with intracranial Ewing's sarcoma. He was born prematurely, suffered intraventricular haemorrhage, posthaemorrhagic hydrocephalus developed, and a ventriculoperitoneal shunt was inserted in the newborn period. The patient endured re-gular follow ups, no signs of shunt malfunction nor increased intracranial pressure were observed. The last neuroima-ging examination was performed at 8 months of age. Upon reaching the age of 4 years, repeated vomiting and focal seizures began, and symptoms of increased intracranial pressure were detected. A brain MRI depicted a left frontoparietal space-occupying lesion infiltrating the superior sagittal sinus. The patient underwent a craniotomy resulting in the total excision of the tumour. The histological examination of the tissue revealed a small round blue cell tumour. The diagnosis was confirmed by the detection of EWSR1 gene translocation with FISH (fluorescent in situ hybridization). No additional metastases were detected during the staging examinations. The patient was treated in accordance to the EuroEwing 99 protocol. Today, ten years onward, the patient is tumour and seizure free and has a reasonably high quality of life.


Assuntos
Encéfalo/diagnóstico por imagem , Sarcoma de Ewing/diagnóstico , Convulsões/etiologia , Vômito/etiologia , Neoplasias Ósseas/genética , Pré-Escolar , Craniotomia , Humanos , Hibridização in Situ Fluorescente , Pressão Intracraniana , Imagem por Ressonância Magnética , Masculino , Neuroimagem , Qualidade de Vida , Proteína EWS de Ligação a RNA , Sarcoma de Ewing/complicações , Sarcoma de Ewing/genética , Sarcoma de Ewing/cirurgia , Translocação Genética , Resultado do Tratamento
5.
Br J Radiol ; 93(1115): 20200257, 2020 Nov 01.
Artigo em Inglês | MEDLINE | ID: mdl-32706980

RESUMO

OBJECTIVE: To evaluate the multiparametric MRI in predicting chemotherapy response in pathologically proven cases of osteosarcoma and Ewing's sarcoma. Correlation between the tumor size changes and internal breakdown using RECIST 1.1, modified RECIST, quantitative apparent diffusion coefficient (ADC) and tumor volume as well as dynamic contrast-enhanced MRI (DCE-MRI). METHODS: The study included 104 patients pathologically proved osteosarcoma (53) and Ewing`s sarcoma (51) underwent MRI examinations; before and after chemotherapy. All patients were assessed using the RECIST 1.1 criteria, m-RECIST, quantitative ADC, and tumor volume evaluation. 21 patients underwent DCE-MRI curve type with quantitative parameters. Correlation between the different evaluations was carried out. Results were correlated with the post-operative pathology in 42 patients who underwent surgery and for statistical evaluation, Those patients were classified into responders (≥90% necrosis) and non-responders (<90% necrosis). RESULTS: The initial mean ADC of 104 patients of osteosarcoma and Ewing's sarcoma (0.90 ± 0.29) and (0.71 ± 0.16) respectively, differed significantly from that after treatment (1.62 ± 0.46) and (1.6 ± 0.39) respectively with (p<0.001).ADC variations (ADC%) in the non-progressive group were higher than those of the progressive group (128.3 ± 63.49 vs 36.34 ± 78.7) % with (p<0.001).ADC values and ADC variations were inversely correlated with morphologic changes, regardless of the effectiveness of chemotherapy expressed as changes in tumor size based on (RECIST 1.1, RECIST, and 3D volume). Linear regression analysis revealed a Pearson correlation coefficient of r=-0.427, -0.498 and -0.408, respectively with (p<0.001).An increase in the ADC value was not always associated with a reduction in tumor volume. The disease control rate (defined as the percentage of CR+PR+SD patients) was 89.4% and 93.9% according to RECIST 1.1 and m-RECIST respectively.42 out of the 104 patients had postsurgical histological evaluation as regards the chemotherapeutic response divided into two groups. ADC values showed a statistically significant difference between Group A and Group B being more evident with minimum ADC% (p<0.001). CONCLUSION: Quantitative diffusion-weighted imaging with ADC mapping and ADC % after chemotherapy allows a detailed analysis of the treatment response in osteosarcoma and Ewing's sarcoma. The therapeutic response can be underestimated using RECIST 1.1, so the modified RECIST should be also considered. ADVANCES IN KNOWLEDGE: Quantitative ADC especially ADC% provided an accurate non-invasive tool in the assessment of post-therapeutic cases of osteosarcoma and Ewing's sarcoma.


Assuntos
Neoplasias Ósseas/tratamento farmacológico , Imagem de Difusão por Ressonância Magnética , Imageamento por Ressonância Magnética Multiparamétrica , Osteossarcoma/tratamento farmacológico , Sarcoma de Ewing/tratamento farmacológico , Adolescente , Adulto , Área Sob a Curva , Neoplasias Ósseas/diagnóstico por imagem , Neoplasias Ósseas/patologia , Neoplasias Ósseas/cirurgia , Criança , Pré-Escolar , Meios de Contraste , Progressão da Doença , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Osteossarcoma/diagnóstico por imagem , Osteossarcoma/patologia , Osteossarcoma/cirurgia , Estudos Prospectivos , Curva ROC , Critérios de Avaliação de Resposta em Tumores Sólidos , Sarcoma de Ewing/diagnóstico por imagem , Sarcoma de Ewing/patologia , Sarcoma de Ewing/cirurgia , Resultado do Tratamento , Carga Tumoral/efeitos dos fármacos , Adulto Jovem
6.
Acta Orthop Traumatol Turc ; 54(3): 234-244, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32544060

RESUMO

OBJECTIVE: This study is an evaluation of the overall survival rate and factors affecting survival in patients with osteosarcoma, chondrosarcoma, or Ewing's sarcoma. This study aimed to determine the effect of factors related to the preoperative period, patient, tumor, treatment, and postoperative period on survival. METHODS: A total of 114 patients (64 male and 50 female) with osteosarcoma, chondrosarcoma, or Ewing's sarcoma treated between 2005 and 2013 were included in this study. All the patients received standard treatment and were followed up regularly. In all, 44 cases of (conventional and telangiectatic) osteosarcoma, 30 cases of Ewing's sarcoma, and 40 cases of high-grade chondrosarcoma were identified using the Bone and Soft Tissue Tumor Registry. Gender, age, tumor size and localization, pathological fractures, histopathological type, grade, surgical treatment, adjuvant treatments, relapse of the disease, and postoperative complication data were obtained from follow-up forms. The learning curve of institutional expertise was also evaluated. The patient survival rate was calculated using the Kaplan-Meier method, and log-rank statistical methods were used to compare survival rates. RESULTS: The mean length of survival of the patients was 72 months. There was a 56% 5-year survival rate, and the event-free survival rate was 53%. The survival of patients with Ewing's sarcoma whose prodromal period was less than 12 weeks was significantly higher than that of the other groups (p=0.031). The survival of patients with tumor size greater than 150 cc, with local recurrence and distant metastases was low for all groups. Survival rates were significantly lower in osteosarcoma and Ewing's sarcoma patients with stage III tumor or metastasis at diagnosis. The survival of patients with osteosarcoma diagnosed between 2010 and 2013 was significantly higher than that of the earlier group (p=0.02). CONCLUSION: Decreasing the prodromal period (early diagnosis) can improve survival by preventing the local and systemic spread of the tumor. Increase in the surgical experience is likely to have a positive effect on survival rates, especially for patients with osteosarcoma. The relapse of the disease is a poor prognostic factor for survival despite aggressive surgery and adjuvant therapies. LEVEL OF EVIDENCE: Level IV, Therapeutic study.


Assuntos
Neoplasias Ósseas , Condrossarcoma , Extremidades , Procedimentos Ortopédicos , Osteossarcoma , Ossos Pélvicos , Sarcoma de Ewing , Adolescente , Adulto , Neoplasias Ósseas/epidemiologia , Neoplasias Ósseas/patologia , Neoplasias Ósseas/cirurgia , Condrossarcoma/epidemiologia , Condrossarcoma/patologia , Condrossarcoma/cirurgia , Terapia Combinada , Extremidades/patologia , Extremidades/cirurgia , Feminino , Humanos , Masculino , Gradação de Tumores , Estadiamento de Neoplasias , Procedimentos Ortopédicos/métodos , Procedimentos Ortopédicos/estatística & dados numéricos , Osteossarcoma/epidemiologia , Osteossarcoma/patologia , Osteossarcoma/cirurgia , Ossos Pélvicos/patologia , Ossos Pélvicos/cirurgia , Estudos Retrospectivos , Fatores de Risco , Sarcoma de Ewing/epidemiologia , Sarcoma de Ewing/patologia , Sarcoma de Ewing/cirurgia , Taxa de Sobrevida , Turquia
7.
Pediatrics ; 145(6)2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32398328

RESUMO

A 15-year-old girl is scheduled to undergo an upper lobectomy to debulk metastatic Ewing sarcoma. The anesthesiologist recommended placement of a thoracic epidural catheter to provide postoperative analgesia. The patient did not want a needle to be placed near her spine. She was terrified that the procedure would be painful and that it might paralyze her. Although the anesthesiologist reassured her that sedation and local anesthesia would make the procedure comfortable, she remained vehemently opposed to the epidural procedure. The parents spoke privately to the anesthesiologist and asked for placement of the epidural after she was asleep. They firmly believed that this would provide optimal postoperative analgesia and thus would be in her best interest. Experts discuss the pros and cons of siding with the patient or parents.


Assuntos
Comportamento do Adolescente/ética , Anestesia Epidural/ética , Dor Pós-Operatória/prevenção & controle , Relações Pais-Filho , Relações Médico-Paciente/ética , Recusa do Paciente ao Tratamento/ética , Adolescente , Comportamento do Adolescente/psicologia , Anestesia Epidural/métodos , Anestesia Epidural/psicologia , Neoplasias Ósseas/psicologia , Neoplasias Ósseas/cirurgia , Feminino , Humanos , Dor Pós-Operatória/psicologia , Pais/psicologia , Sarcoma de Ewing/psicologia , Sarcoma de Ewing/cirurgia , Recusa do Paciente ao Tratamento/psicologia
8.
Surgery ; 168(2): 335-339, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32434659

RESUMO

BACKGROUND: Adrenal lesions diagnosed during pregnancy remain rare, and their management is challenging because of maternal physiologic modifications, restricted imaging investigations, and contraindications to several treatments. Surgical issues of adrenalectomy during pregnancy and consequences on perinatal outcomes are poorly described. We therefore aimed to report maternal and fetal outcomes after adrenalectomy during pregnancy. METHODS: All pregnant women who underwent adrenalectomy over a 15-year inclusion period were identified from a prospectively maintained database. Surgical management and maternal and fetal outcomes were reviewed. RESULTS: From January 2003 to July 2018, a total of 12 women underwent adrenalectomy at a median gestation of 20 weeks. Of these women, 11 had hyper-secreting lesions, including 8 with cortisol oversecretion, and 11 had benign lesions, including cortisol-secreting adenoma (n = 5), pheochromocytoma (n = 2), primary pigmented, nodular adrenal disease (n = 1), severe Cushing's disease (n = 2), and hematoma (n = 1). A total of 3 patients with severe Cushing's disease (n = 2) and primary pigmented, nodular adrenal disease (n = 1) required bilateral adrenalectomy. One patient presented with a malignant adrenal Ewing sarcoma. Adrenalectomy during pregnancy was performed by the lateral laparoscopic transabdominal laparoscopic route in 9 patients. Postoperative morbidity occurred in 3 women. Maternal mortality was nil, but preterm birth occurred in 7 cases and intrauterine growth retardation was observed in 3 cases. Finally, among the 12 women, 10 had a child in good health. CONCLUSION: During pregnancy, a lateral laparoscopic transabdominal approach is a feasible procedure. Maternal outcome is acceptable but fetal outcome is determined by the underlying disease, with a worse outcome when the adrenalectomy is indicated for malignant lesions or Cushing's syndrome.


Assuntos
Adrenalectomia , Complicações na Gravidez/cirurgia , Adenoma/cirurgia , Doenças do Córtex Suprarrenal/cirurgia , Neoplasias das Glândulas Suprarrenais/cirurgia , Adulto , Estudos de Casos e Controles , Síndrome de Cushing/cirurgia , Feminino , Retardo do Crescimento Fetal/epidemiologia , França/epidemiologia , Humanos , Laparoscopia , Tempo de Internação , Feocromocitoma/cirurgia , Gravidez , Nascimento Prematuro/epidemiologia , Estudos Retrospectivos , Sarcoma de Ewing/cirurgia , Centros de Atenção Terciária
10.
Bone Joint J ; 102-B(1): 64-71, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31888359

RESUMO

AIMS: The purpose of this study was to report the long-term results of extendable endoprostheses of the humerus in children after the resection of a bone sarcoma. METHODS: A total of 35 consecutive patients treated with extendable endoprosthetic replacement of the humerus in children were included. There were 17 boys and 18 girls in the series with a median age at the time of initial surgery of nine years (interquartile range (IQR) 7 to 11). RESULTS: The median follow-up time was 10.6 years (IQR 3.9 to 20.4). The overall implant survival at ten years was 75%. Complications occurred in 13 patients (37%). Subluxation at the proximal humerus occurred in 19 patients (54%) but only six (17%) were symptomatic. Subluxation was seen more commonly in children under the age of nine years (86%) than in those aged nine years or more (33%) (p = 0.002). Implant failure occurred in nine patients (26%): the most common cause was aseptic loosening (four patients, 11%). Lengthening of the implant was carried out in 23 patients (66%). At final follow up, three patients had a limb that was shortened by 5 cm or more. The mean Musculoskeletal Tumor Society (MSTS) functional score was 79% (73% to 90%). CONCLUSION: Extendable endoprosthetic replacement is a reliable method of reconstructing humerus after excision of a bone sarcoma. Children who are less than nine years old have a high risk of subluxation. Cite this article: Bone Joint J 2020;102-B(1):64-71.


Assuntos
Neoplasias Ósseas/cirurgia , Úmero/cirurgia , Osteossarcoma/cirurgia , Membros Artificiais , Neoplasias Ósseas/diagnóstico por imagem , Neoplasias Ósseas/mortalidade , Criança , Condrossarcoma/diagnóstico por imagem , Condrossarcoma/mortalidade , Condrossarcoma/cirurgia , Feminino , Humanos , Úmero/diagnóstico por imagem , Masculino , Margens de Excisão , Metástase Neoplásica , Osteossarcoma/diagnóstico por imagem , Osteossarcoma/mortalidade , Estudos Prospectivos , Próteses e Implantes , Desenho de Prótese , Falha de Prótese , Implantação de Prótese/métodos , Implantação de Prótese/mortalidade , Amplitude de Movimento Articular/fisiologia , Sarcoma de Ewing/diagnóstico por imagem , Sarcoma de Ewing/mortalidade , Sarcoma de Ewing/cirurgia , Resultado do Tratamento
11.
World Neurosurg ; 133: e327-e341, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31520760

RESUMO

BACKGROUND: Medical implications of 3-dimensional (3D) printing technology have evolved and are increasingly used. Surgical spine oncology involves at times complex resection using various surgical approaches and unique spinal reconstruction. As high general complication rates, including hardware failure, are reported, careful preoperative planning and optimized fixation techniques should be performed. 3D printing technology allows the improvement of preoperative planning, practice and exploration of various surgical approaches, and designing customized surgical tools and patient specific implants. OBJECTIVE: To investigate the use of 3D printing technology in complex spine surgeries. METHODS: Between 2015 and 2018, all complex spine oncological cases were evaluated and assessed for the possible benefit of use of 3D printing technology. Following high-quality imaging, a computerized integrated 3D model was created. Based on the planned procedure considering the various surgical steps, a customized 3D model was planned and printed, and in select cases a 3D custom-made implant was designed and printed in various sizes with matching trials. RESULTS: A total of 7 cases were selected for the use of a 3D printing technology. For all, a custom-made model was created. In 3 of these cases, a customized 3D-printed implant was used. Special customized intraoperative instruments were made for 2 cases, and a simulated surgical approach was performed in 5 cases. In 2 cases, pre-bent rods were made based on the model created and were used in surgery later on. CONCLUSIONS: For complex spine oncology cases, the use of 3D printing allowed better preoperative planning, simplified the operative procedure, and enabled improved reconstruction.


Assuntos
Imagem por Ressonância Magnética , Modelos Anatômicos , Impressão Tridimensional , Próteses e Implantes , Procedimentos Cirúrgicos Reconstrutivos/métodos , Neoplasias da Coluna Vertebral/cirurgia , Cirurgia Assistida por Computador/métodos , Tomografia Computadorizada por Raios X , Adolescente , Adulto , Transplante Ósseo , Carcinoma Ductal de Mama/diagnóstico por imagem , Carcinoma Ductal de Mama/secundário , Carcinoma Ductal de Mama/cirurgia , Carcinoma de Células Renais/diagnóstico por imagem , Carcinoma de Células Renais/secundário , Carcinoma de Células Renais/cirurgia , Condrossarcoma/diagnóstico por imagem , Condrossarcoma/patologia , Condrossarcoma/cirurgia , Desenho de Equipamento , Feminino , Tumores de Células Gigantes/diagnóstico por imagem , Tumores de Células Gigantes/patologia , Tumores de Células Gigantes/cirurgia , Hemangioma/diagnóstico por imagem , Hemangioma/patologia , Hemangioma/cirurgia , Humanos , Vértebras Lombares/diagnóstico por imagem , Vértebras Lombares/patologia , Vértebras Lombares/cirurgia , Masculino , Pessoa de Meia-Idade , Osteoma Osteoide/diagnóstico por imagem , Osteoma Osteoide/patologia , Osteoma Osteoide/cirurgia , Procedimentos Cirúrgicos Reconstrutivos/instrumentação , Sarcoma de Ewing/diagnóstico por imagem , Sarcoma de Ewing/secundário , Sarcoma de Ewing/cirurgia , Neoplasias da Coluna Vertebral/diagnóstico por imagem , Neoplasias da Coluna Vertebral/patologia , Neoplasias da Coluna Vertebral/secundário , Cirurgia Assistida por Computador/instrumentação , Instrumentos Cirúrgicos , Vértebras Torácicas/diagnóstico por imagem , Vértebras Torácicas/patologia , Vértebras Torácicas/cirurgia , Adulto Jovem
13.
BMC Cancer ; 19(1): 1168, 2019 Dec 02.
Artigo em Inglês | MEDLINE | ID: mdl-31791278

RESUMO

BACKGROUND: Adult Ewing sarcoma (ES) is a rare disease, the optimal treatment model is unknown. This study aimed to retrospectively analyze treatment-related prognostic factors of nonspinal ES in Chinese adults. METHODS: Eighty-one patients treated between January 2005 and December 2017 were included in the present study. Thirty-three (40.7%) presented with metastatic disease at diagnosis. Eight patients were submitted to primary surgery followed by chemotherapy, while 73 patients received chemotherapy before and after surgery and/or local radiotherapy. The chemotherapy regimen included 8-17 cycles of vincristine, doxorubicin, and cyclophosphamide (VDC) alternating with ifosfamide and etoposide (IE) every 3 weeks. Clinical outcomes and safety were analyzed. RESULTS: VDC/IE chemotherapy was well tolerated in adult patients with ES. Multivariate Cox regression analyses revealed that chemotherapy of at least 12 cycles was a favorable independent prognostic factor of event-free survival (hazard ratio, 0.558; 95% confidence interval, 0.323-0.965; P = 0.037) and overall survival (hazard ratio, 0.424; 95% confidence interval, 0.240-0.748; P = 0.003). Similarly, a low frequency of chemotherapy delays was an independent prognostic factor of improved OS (hazard ratio, 0.438; 95% confidence interval, 0.217-0.887; P = 0.022). CONCLUSION: Our study suggests that adults with ES should be treated with an aggressive multidisciplinary approach, intensive chemotherapy with adequate cycles and appropriate intervals can be recommended in this group.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Neoplasias Ósseas/tratamento farmacológico , Sarcoma de Ewing/tratamento farmacológico , Adolescente , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Neoplasias Ósseas/patologia , Neoplasias Ósseas/radioterapia , Neoplasias Ósseas/cirurgia , Quimioterapia Adjuvante , Ciclofosfamida/administração & dosagem , Ciclofosfamida/efeitos adversos , Doxorrubicina/administração & dosagem , Doxorrubicina/efeitos adversos , Esquema de Medicação , Etoposídeo/administração & dosagem , Etoposídeo/efeitos adversos , Feminino , Humanos , Ifosfamida/administração & dosagem , Ifosfamida/efeitos adversos , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Sarcoma de Ewing/patologia , Sarcoma de Ewing/radioterapia , Sarcoma de Ewing/cirurgia , Análise de Sobrevida , Resultado do Tratamento , Vincristina/administração & dosagem , Vincristina/efeitos adversos , Adulto Jovem
14.
Bull Hosp Jt Dis (2013) ; 77(4): 269-274, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31785141

RESUMO

The terminal overgrowth of the tibia following pediatric transtibial amputation is a common problem leading to pain, disability, and repeat surgical procedures. We present three patients who underwent transtibial amputation due to sarcoma of the lower extremity followed by compress osseointegration prosthesis fixation. The minimum follow-up was 1 year. The average age of patients was 10.8 years. There were no complications that required surgical revision. To date, there has been no evidence of terminal appositional overgrowth in this series. The application of an end-cap implant utilizing compressive osseointegration fixation can prevent terminal bone overgrowth in pediatric transtibial amputations.


Assuntos
Amputação , Neoplasias Ósseas/cirurgia , Complicações Pós-Operatórias/prevenção & controle , Implantação de Prótese , Sarcoma de Ewing/cirurgia , Tíbia/cirurgia , Amputação/efeitos adversos , Neoplasias Ósseas/diagnóstico por imagem , Neoplasias Ósseas/patologia , Criança , Feminino , Humanos , Masculino , Osseointegração , Implantação de Prótese/efeitos adversos , Implantação de Prótese/instrumentação , Sarcoma de Ewing/diagnóstico por imagem , Sarcoma de Ewing/patologia , Tíbia/diagnóstico por imagem , Tíbia/crescimento & desenvolvimento , Tíbia/patologia , Fatores de Tempo , Resultado do Tratamento
15.
J Plast Reconstr Aesthet Surg ; 72(12): 1887-1899, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31563475

RESUMO

BACKGROUND: Limb salvage is important in pediatric patients with bone sarcomas. The vascularized fibula flap is a versatile option, combined or not with a bone allograft. The authors evaluated the functional long-term outcomes, complications, and survival of using this technique in pediatric patients. METHODS: A retrospective review of 27 pediatric patients reconstructed between 2011 and 2018 with the fibula flap after bone sarcoma resection was conducted. Long-term functional outcomes, complications, and survival were assessed. Variables analyzed were age, sex, Capanna technique, follow-up, complications, additional surgeries, time to weight bearing, length discrepancy, and sport practice. RESULTS: Twenty-seven patients with a mean age of 9.3 years were included. The mean follow-up was 44.33 months. The Capanna technique was performed in 15 patients. All extremities but one were salvaged. The overall complication rate was 74.07%. Fibula fracture and nonunion rates were 34.04% and 11.11%, respectively. Partial weight bearing was resumed at a mean of 9.07 months. About 79.17% of patients with a 12-month follow-up achieved full weight bearing. An age below 8 years was significantly associated with a lower major complication rate and a shorter time to weight bearing and full weight bearing. Major complications and additional surgeries were significantly associated with longer periods until weight bearing and full weight bearing. CONCLUSIONS: The fibula flap allows the majority of extremities to be reconstructed. However, a high rate of complications and additional surgeries should be anticipated. Full weight bearing is usually achieved within the first year, with modest functional increase afterward. Less complications and a faster functional recovery are expected in patients below the age of 8 years.


Assuntos
Neoplasias Ósseas/cirurgia , Transplante Ósseo/métodos , Fíbula/transplante , Osteossarcoma/cirurgia , Sarcoma de Ewing/cirurgia , Retalhos Cirúrgicos , Adolescente , Aloenxertos/irrigação sanguínea , Calcâneo/cirurgia , Criança , Pré-Escolar , Feminino , Neoplasias Femorais/cirurgia , Fixação Interna de Fraturas/métodos , Fraturas Espontâneas/cirurgia , Humanos , Úmero/cirurgia , Masculino , Complicações Pós-Operatórias/etiologia , Rádio (Anatomia)/cirurgia , Estudos Retrospectivos , Terapia de Salvação/métodos , Transplante Homólogo/métodos , Resultado do Tratamento
16.
J Surg Oncol ; 120(6): 985-993, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31381161

RESUMO

PURPOSE: The purpose of this study was to clarify which local treatment is oncologically and functionally effective in pelvic Ewing sarcoma (ES). METHODS: A consecutive series of patients who underwent pelvic resections and acetabular reconstructions after chemotherapy between 1986 and 2016 at a supra-regional center were evaluated. RESULTS: The cohort consisted of 35 patients. The 5-year overall survival (OS) and local recurrence-free survival (LRFS) was 61% and 72%, respectively. Preoperative radiotherapy (RT) and surgery provided an excellent/good histological response in 92% and achieved significantly better OS (5 years, 64%) and LRFS (5 years, 100%) than surgery alone or surgery with postoperative RT. The Musculoskeletal Tumor Society functional scores were significantly better in patients with hip transposition than those with structural reconstructions (74% vs 57%; P = .031) using custom-made prostheses, irradiated autografts, and ice-cream cone prostheses. These scores were significantly lower if patients had deep infection (P = .035), which was the most common complication (28%) in structural reconstructions but did not occur in hip transposition even when performed after preoperative RT. CONCLUSION: Acetabular reconstruction with hip transposition resulted in no deep infection and superior function in patients with pelvic ES even when combined with preoperative RT, which improved tumor necrosis and rate of local control and survival.


Assuntos
Acetábulo/cirurgia , Neoplasias Ósseas/cirurgia , Recidiva Local de Neoplasia/cirurgia , Ossos Pélvicos/cirurgia , Procedimentos Cirúrgicos Reconstrutivos/métodos , Terapia de Salvação , Sarcoma de Ewing/cirurgia , Acetábulo/patologia , Adolescente , Adulto , Neoplasias Ósseas/patologia , Neoplasias Ósseas/terapia , Criança , Terapia Combinada , Feminino , Seguimentos , Humanos , Masculino , Recidiva Local de Neoplasia/patologia , Recidiva Local de Neoplasia/terapia , Ossos Pélvicos/patologia , Prognóstico , Estudos Retrospectivos , Sarcoma de Ewing/patologia , Sarcoma de Ewing/terapia , Taxa de Sobrevida , Adulto Jovem
17.
World Neurosurg ; 132: 303-308, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31394361

RESUMO

BACKGROUND: Primary Ewing sarcoma of the mobile spine is a rare disease. Its management requires careful surgical planning, because radical, margin-free excision is directly correlated with prognosis. Extensive bone removal in the cervical spine can lead to instability and cause postoperative iatrogenic cervical deformity. Thus, spinal instrumentation plays an important role in restoring postresection spinal stability and improving quality of life. METHODS: We present a novel technique that allows successful removal of a large Ewing sarcoma of the subaxial cervical spine, infiltrating and traversing the posterior bone elements, and extending into the paraspinal muscles. This technique involves radical en bloc resection of posteriorly located cervical tumors via multilevel pediculotomy, with terminal vertebrae pedicle screw reconstruction. RESULTS: Terminal vertebrae cervical pedicle screw reconstruction allowed wide surgical excision with satisfactory oncologic and mechanical results. CONCLUSIONS: This technique enables 1-stage total tumor resection and stabilization and may be a viable alternative to radical en bloc resection of posteriorly located epidural malignant lesions of the cervical spine in selected cases.


Assuntos
Vértebras Cervicais/cirurgia , Procedimentos Neurocirúrgicos/métodos , Parafusos Pediculares , Procedimentos Cirúrgicos Reconstrutivos/métodos , Sarcoma de Ewing/cirurgia , Neoplasias da Coluna Vertebral/cirurgia , Adolescente , Vértebras Cervicais/diagnóstico por imagem , Humanos , Masculino , Osteotomia/métodos , Sarcoma de Ewing/complicações , Sarcoma de Ewing/diagnóstico por imagem , Compressão da Medula Espinal/diagnóstico por imagem , Compressão da Medula Espinal/etiologia , Neoplasias da Coluna Vertebral/complicações , Neoplasias da Coluna Vertebral/diagnóstico por imagem
18.
Arch. esp. urol. (Ed. impr.) ; 72(6): 570-580, jul.-ago. 2019. ilus
Artigo em Espanhol | IBECS | ID: ibc-187661

RESUMO

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


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


Assuntos
Humanos , Feminino , Adolescente , Neoplasias Renais/cirurgia , Sarcoma de Ewing/cirurgia , Sarcoma de Ewing/tratamento farmacológico , Sarcoma de Ewing/radioterapia , Neoplasias Renais/tratamento farmacológico , Neoplasias Renais/radioterapia , Resultado do Tratamento , Nefrectomia
19.
Sci Rep ; 9(1): 11000, 2019 07 29.
Artigo em Inglês | MEDLINE | ID: mdl-31358784

RESUMO

Accurate survival estimations in Ewing sarcoma are necessary to develop risk- and response adaptive treatment strategies allowing for early decision-making. We aim to develop an easy-to-use survival estimation tool from diagnosis and surgery. A retrospective study of 1314 Ewing sarcoma patients was performed. Associations between prognostic variables at diagnosis/surgery and overall survival (OS), were investigated using Kaplan-Meier and multivariate Cox models. Predictive accuracy was evaluated by cross-validation and Harrell C-statistics. Median follow-up was 7.9 years (95%CI 7.6-8.3). Independent prognostic factors at diagnosis were age, volume, primary tumor localization and disease extent. 5 risk categories (A-E) were identified with 5-year OS of 88% (86-94), 69% (64-74), 57% (50-64), 51% (42-60) and 28% (22-34) respectively. Harrell C-statistic was 0.70. Independent prognostic factors from surgery were age, volume, disease extent and histological response. In categories A-B, 5y OS increased to 92% (87-97) and 79% (71-87) respectively for 100% necrosis and decreased to 76% (67-85) and 62% (55-69) respectively for <100% necrosis. In categories C-E, 5y OS increased to 65% (55-75), 65% (52-78) and 52% (38-66) respectively for ≥90% necrosis and decreased to 38% (22-54), 11% (0-26) and 7% (0-19) respectively for <90% necrosis. We present an easy-to-use survival estimation tool from diagnosis in Ewing sarcoma based on age, volume, primary tumor localization and disease extent. Histological response is a strong additional prognostic factor for OS.


Assuntos
Neoplasias Ósseas/diagnóstico , Neoplasias Ósseas/cirurgia , Sarcoma de Ewing/diagnóstico , Sarcoma de Ewing/cirurgia , Adolescente , Adulto , Neoplasias Ósseas/epidemiologia , Criança , Feminino , Humanos , Masculino , Prognóstico , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Sarcoma de Ewing/epidemiologia , Análise de Sobrevida , Adulto Jovem
20.
Pediatr Blood Cancer ; 66(11): e27938, 2019 11.
Artigo em Inglês | MEDLINE | ID: mdl-31347793

RESUMO

The therapies used to treat Ewing sarcoma are associated with a risk of second malignant neoplasm (SMN). We conducted a systematic review to pool available evidence on the risks, types, and outcomes after SMN. We obtained 52 articles that met inclusion criteria. Cumulative incidence rates of SMN ranged from 0.9 to 8.4% and 10.1 to 20.5% at 5 and 30 years after initial diagnosis. Of the 327 reported SMNs, 63.6% were solid tumors, although acute myeloid leukemia /myelodysplastic syndrome was the single most commonly diagnosed SMN, with generally poor outcomes. Patients treated for Ewing sarcoma are at substantial risk of SMN, with a broad range of reported secondary cancers.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Neoplasias Ósseas , Neoplasias Induzidas por Radiação/etiologia , Segunda Neoplasia Primária/etiologia , Radioterapia/efeitos adversos , Sarcoma de Ewing , Fatores Etários , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias Ósseas/tratamento farmacológico , Neoplasias Ósseas/radioterapia , Neoplasias Ósseas/cirurgia , Carcinoma/epidemiologia , Carcinoma/etiologia , Carcinoma/terapia , Humanos , Incidência , Leucemia Mieloide/epidemiologia , Leucemia Mieloide/etiologia , Leucemia Mieloide/terapia , Linfoma/epidemiologia , Linfoma/etiologia , Linfoma/terapia , Melanoma/epidemiologia , Melanoma/etiologia , Melanoma/terapia , Neoplasias Induzidas por Radiação/epidemiologia , Neoplasias Induzidas por Radiação/terapia , Segunda Neoplasia Primária/epidemiologia , Segunda Neoplasia Primária/terapia , Risco , Sarcoma/epidemiologia , Sarcoma/etiologia , Sarcoma/terapia , Sarcoma de Ewing/tratamento farmacológico , Sarcoma de Ewing/radioterapia , Sarcoma de Ewing/cirurgia , Fatores de Tempo , Resultado do Tratamento
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