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1.
Am J Case Rep ; 22: e930384, 2021 Jun 05.
Artigo em Inglês | MEDLINE | ID: mdl-34089579

RESUMO

BACKGROUND Ewing sarcoma and primitive neuroectodermal tumor are rare tumors grouped under the spectrum of the Ewing sarcoma family of tumors. These highly malignant tumors involve the bones and commonly occur in children. Ewing sarcoma of the skull bone accounts for only 1% of all Ewing sarcomas, with primary skull base Ewing sarcoma occurring in less than 1% of cases. We present a case of skull base Ewing sarcoma with complete symptom recovery and near-total radiological resolution. CASE REPORT A 4-year-old girl initially presented with a 2-month history of vomiting, poor oral intake, weight loss, and gradual visual deterioration followed by acute symptoms of fever, breathing difficulties, and seizure. Initial computed tomography and magnetic resonance imaging of the brain displayed a large sinonasal mass with extensive regional infiltration and bony destruction and no evidence of distant metastasis. A transnasal biopsy was taken. The histopathology result revealed features of skull base Ewing sarcoma. The child was given a combination of radiotherapy and chemotherapy, to which she responded well, with a minimal residual tumor. CONCLUSIONS Skull base Ewing sarcoma is a rare entity, presenting a challenge to the reporting radiologists. Differential diagnoses of esthesioneuroblastoma, olfactory neuroepithelioma, and, more commonly, sinonasal carcinoma can be misleading since they have similar radiological appearances to skull base Ewing sarcoma, which differs in treatment regimen and prognosis. Therefore, a combination of histopathological appearance, radiographic findings, and clinical correlation is important to determine the correct diagnosis, establish the appropriate treatment regime, and improve the patient's survival.


Assuntos
Neoplasias Nasais , Sarcoma de Ewing , Criança , Pré-Escolar , Feminino , Humanos , Cavidade Nasal , Doenças Raras , Sarcoma de Ewing/diagnóstico por imagem , Sarcoma de Ewing/terapia , Base do Crânio
2.
Clin Nucl Med ; 46(5): 422-423, 2021 May 01.
Artigo em Inglês | MEDLINE | ID: mdl-33630793

RESUMO

ABSTRACT: Ewing sarcoma is an aggressive malignancy that most often presents as an undifferentiated primary bone tumor; less commonly, it arises in extraosseous soft tissues of mesenchymal cell origin. Primary extraosseous involvement of seminal vesicle is rarely reported. Herein, we describe FDG PET/CT and MRI findings of a 55-year-old man who presented with a pelvic mass, which was confirmed to be primary Ewing sarcoma/primitive neuroectodermal tumor arising from seminal vesicle by histopathologic findings.


Assuntos
Neoplasias Ósseas/diagnóstico por imagem , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Sarcoma de Ewing/diagnóstico por imagem , Glândulas Seminais/diagnóstico por imagem , Neoplasias Ósseas/patologia , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Sarcoma de Ewing/patologia
3.
J Int Med Res ; 49(2): 300060520986681, 2021 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-33530793

RESUMO

A rare and highly malignant small round cell tumor, Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET) usually occurs in the pelvis, long-axis bones, and femur. In contrast, extraosseous ES is more often found in the paraspinal region, limbs, and retroperitoneum, but is extremely rare in the stomach. We report a case of a 55-year-old woman who presented with fatigue, fever, and black stool. Preoperative computed tomography (CT) imaging showed a large ulcerative lesion of approximately 5.5 × 5.0 cm in the stomach and irregular thickening of the ulcer wall. Upper endoscopy revealed a large, irregular ulcer in the posterior wall of the stomach. Histopathological examination suggested that the mass with the largest diameter (7.5 cm) was ES. Immunohistochemistry indicated positivity for CD99. Enhanced CT of the whole body was performed but no definite masses were found in other organs, and the patient was diagnosed with primary gastric ES. The patient underwent radical distal gastrectomy with Roux-en-Y gastrojejunostomy, but refused chemoradiotherapy.


Assuntos
Tumores Neuroectodérmicos Primitivos Periféricos , Tumores Neuroectodérmicos Primitivos , Sarcoma de Ewing , Feminino , Humanos , Imuno-Histoquímica , Pessoa de Meia-Idade , Sarcoma de Ewing/diagnóstico por imagem , Sarcoma de Ewing/cirurgia , Estômago
4.
Sichuan Da Xue Xue Bao Yi Xue Ban ; 52(1): 149-152, 2021 Jan.
Artigo em Chinês | MEDLINE | ID: mdl-33474905

RESUMO

Objective: This study investigated the clinicopathological traits and ultrasound features of female reproductive system extraosseous Ewing's sarcoma (EES) and explored the diagnostic value of ultrasonography for this condition. Methods: Cases of female pelvic EES diagnosed and treated at our hospital between June 2009 and June 2019 were included in this study. Pathology data and ultrasound manifestations were assessed retrospectively to summarize the clinical traits and ultrasound features of female reproductive system EES. Based on the results, recommendations for the ultrasonography-based diagnosis of this disease were proposed. Results: During the 10-year study period, 13 female patients were diagnosed with EES in the pelvic cavity based on the results of postoperative pathology tests. The age of the patients ranged from 8 mouth to 40 years, and no patients demonstrated specific clinical symptoms. However, an examination of tumor biomarkers revealed that certain patients had elevated levels of CA125. In the 13 patients, 19 lesions were identified, including 16 that involved the reproductive system. The primary ultrasound manifestation was uneven, low-echo solidity or cystic solidity, exhibiting large size, irregular shape, and unclear boundary. A few patients had concurrent ascites. Although some lesions lacked blood supply, the blood supply of most lesions was medium to abundant, and the blood flow was mostly characterized by low resistant. Almost none of the lesions were definitively diagnosed preoperatively. Conclusions: Preoperative definitive diagnosis of EES in the female reproductive system remains a great clinical challenge. Although certain clinical traits and ultrasound features are associated with this disease, and color Doppler ultrasonography might provide vital information indicating the presence of EES, the final diagnosis still depends on the pathological test results of the patients.


Assuntos
Sarcoma de Ewing , Criança , Feminino , Genitália Feminina , Humanos , Estudos Retrospectivos , Sarcoma de Ewing/diagnóstico por imagem , Ultrassonografia , Ultrassonografia Doppler em Cores
5.
Clin Imaging ; 73: 108-110, 2021 May.
Artigo em Inglês | MEDLINE | ID: mdl-33360836

RESUMO

Extraskeletal Ewing sarcoma (EES) is a rare soft tissue tumor, and EES of the head and neck is particularly rare. Radiographic imaging of these lesions is crucial given their anatomical complexity and infrequent incidence. Conventional EES imaging features include a large, hyperintense, heterogeneously enhancing lesion, with frequent invasion of local structures. In this case report, a 19-year old male presented with left facial swelling and pain. He underwent sclerotherapy and bleomycin treatment for a presumed lymphatic malformation. Initial imaging demonstrated a rim-enhancing lesion within the left buccal space with no muscle invasion or bony erosion present. Two years later, imaging identified an enlarging buccal mass with destruction of the zygomatic arch and inferolateral orbital wall. The patient underwent surgical resection of the mass. Pathology confirmed the mass to be a small round blue cell tumor and FISH testing confirmed the presence of the EWSR1 gene arrangement that is consistent with EES. The patient tolerated the procedure well and underwent chemoradiation therapy. At three years postoperatively, the patient remains disease free. The presented case demonstrates an unusual presentation of a buccal space EES as a rim-enhancing, centrally hypointense mass with no bony erosion or muscular invasion. The location and atypical imaging appearance of this case offer insight for future diagnosis of EES.


Assuntos
Sarcoma de Ewing , Neoplasias de Tecidos Moles , Adulto , Diagnóstico por Imagem , Humanos , Masculino , Boca , Sarcoma de Ewing/diagnóstico por imagem , Sarcoma de Ewing/terapia , Adulto Jovem
6.
Bull Cancer ; 107(11): 1186-1198, 2020 Nov.
Artigo em Francês | MEDLINE | ID: mdl-33059872

RESUMO

Benign and malign tumors can affect the temporomandibular joint (TMJ) as any other articulation. Nevertheless, TMJ tumors are rare and mostly benign. Their clinical expression is varied including symptomatology similar to TMJ dysfunctional disorders, otologic or neurologic pathologies. In some cases, they remain totally asymptomatic. Hence, diagnosis is difficult since the symptomatology can be misleading with TMJ dysfunctional disorders or otologic disorders wrongly diagnosed. There is thus frequently a long delay between symptoms onset and diagnosis. The great variety of TMJ lesions explains the wide range of possible treatment modalities, mostly based on surgery. We provide here a review of the lesions originating from the TMJ. Tumoral or cystic mandibular lesion affecting the TMJ through local extension will not be discussed. Osteoma, osteoid osteoma, osteoblastoma, chondroma, osteochondroma, chondroblastoma, tenosynovial giant cell tumors, giant cell lesions, non-ossifying fibroma, hemangioma, lipoma or Langerhans cell histiocytosis are all possible diagnosis among the benign tumors found in the TMJ. Pseudotumors include synovial chondromatosis and aneurysmal bone cyst. Finally, malign tumors of the TMJ include mainly sarcomas (osteosarcoma, chondrosarcoma, synovial sarcoma, Ewing sarcoma, and fibrosarcoma), but also multiple myeloma and secondary metastases. We will review the clinical, radiological and histological aspects of each of these lesions. The treatment and the recurrence risk will also be discussed.


Assuntos
Neoplasias Ósseas , Articulação Temporomandibular , Cistos Ósseos Aneurismáticos/etiologia , Cistos Ósseos Aneurismáticos/patologia , Cistos Ósseos Aneurismáticos/terapia , Neoplasias Ósseas/complicações , Neoplasias Ósseas/diagnóstico por imagem , Neoplasias Ósseas/patologia , Neoplasias Ósseas/cirurgia , Condroblastoma/complicações , Condroblastoma/diagnóstico por imagem , Condroblastoma/cirurgia , Condroma/diagnóstico por imagem , Condroma/patologia , Condroma/cirurgia , Condrossarcoma/patologia , Condrossarcoma/terapia , Diagnóstico Diferencial , Fibrossarcoma/diagnóstico por imagem , Fibrossarcoma/patologia , Fibrossarcoma/terapia , Tumor de Células Gigantes do Osso/diagnóstico por imagem , Tumor de Células Gigantes do Osso/terapia , Tumor de Células Gigantes de Bainha Tendinosa/complicações , Tumor de Células Gigantes de Bainha Tendinosa/diagnóstico por imagem , Tumor de Células Gigantes de Bainha Tendinosa/cirurgia , Hemangioma/diagnóstico por imagem , Hemangioma/terapia , Histiocitose de Células de Langerhans/patologia , Histiocitose de Células de Langerhans/cirurgia , Humanos , Lipoma/diagnóstico por imagem , Lipoma/patologia , Lipoma/cirurgia , Mieloma Múltiplo/patologia , Osteoblastoma/diagnóstico por imagem , Osteoblastoma/patologia , Osteoblastoma/cirurgia , Osteocondroma/diagnóstico por imagem , Osteocondroma/patologia , Osteocondroma/cirurgia , Osteoma/diagnóstico por imagem , Osteoma/patologia , Osteoma Osteoide/complicações , Osteoma Osteoide/diagnóstico por imagem , Osteoma Osteoide/patologia , Osteossarcoma/diagnóstico por imagem , Osteossarcoma/patologia , Osteossarcoma/terapia , Sarcoma de Ewing/diagnóstico por imagem , Sarcoma de Ewing/patologia , Sarcoma Sinovial/diagnóstico por imagem
7.
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
8.
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
9.
Arch Bronconeumol ; 56(10): 674-676, 2020 10.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-32586699

Assuntos
Adenocarcinoma/diagnóstico por imagem , Betacoronavirus , Neoplasias Ósseas/diagnóstico por imagem , Técnicas de Laboratório Clínico , Infecções por Coronavirus/diagnóstico , Biópsia Guiada por Imagem/métodos , Neoplasias Pulmonares/diagnóstico por imagem , Pulmão/diagnóstico por imagem , Pandemias , Pneumonia Viral/diagnóstico , Sarcoma de Ewing/diagnóstico por imagem , Adenocarcinoma/complicações , Adenocarcinoma/patologia , Idoso , Betacoronavirus/genética , Betacoronavirus/isolamento & purificação , Neoplasias Ósseas/complicações , Neoplasias Ósseas/patologia , Infecções por Coronavirus/complicações , Infecções por Coronavirus/diagnóstico por imagem , Infecções por Coronavirus/epidemiologia , Gerenciamento Clínico , Feminino , Humanos , Achados Incidentais , Transmissão de Doença Infecciosa do Paciente para o Profissional/prevenção & controle , Neoplasias Pulmonares/complicações , Neoplasias Pulmonares/patologia , Masculino , Invasividade Neoplásica , Equipamento de Proteção Individual , Neoplasias Pleurais/diagnóstico por imagem , Neoplasias Pleurais/secundário , Pneumonia Viral/complicações , Pneumonia Viral/diagnóstico por imagem , Pneumonia Viral/epidemiologia , RNA Viral/sangue , Reação em Cadeia da Polimerase em Tempo Real , Caixa Torácica/diagnóstico por imagem , Sarcoma de Ewing/complicações , Sarcoma de Ewing/patologia , Canal Medular/diagnóstico por imagem , Canal Medular/patologia , Telecomunicações , Adulto Jovem
10.
Brain Tumor Pathol ; 37(3): 111-117, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32449046

RESUMO

The CIC-DUX4 translocation is the most common genetic alteration of small round cell sarcomas without EWSR1 rearrangement. These "Ewing-like sarcomas" usually occur in peripheral soft tissues, and rare primary central nervous system (CNS) tumors have been described. We report a rare case of primary spinal intramedullary Ewing-like sarcoma harboring CIC-DUX4 translocation. A 23-year-old man presented with weakness in the extremities. Magnetic resonance imaging revealed a large intramedullary tumor spanning C3-C5 with heterogeneous enhancement following gadolinium administration. Histologically, most of the tumor displayed dense myeloid proliferation composed of medium- to slightly small-sized primitive cells. Postoperatively, he received local adjuvant radiation therapy without tumor progression for 10 months. Target RNA sequencing analysis revealed the CIC-DUX4 fusion gene. Methylation array analysis resulted in a diagnosis of "methylation class CNS Ewing sarcoma family tumor with CIC alteration". Although this tumor lacked characteristic histological features such as lobular structures in association with desmoplastic stroma, relatively uniform nuclei with prominent nucleoli and eosinophilic cytoplasm, which are often found in CIC-rearranged sarcomas of soft tissue, were identified. Recently, many CNS and soft tissue tumors require genetic analysis for precise diagnosis. To consider certain molecular testing, careful histological examination is essential.


Assuntos
Proteínas de Fusão Oncogênica/genética , Sarcoma de Ewing/genética , Sarcoma de Ewing/patologia , Neoplasias da Coluna Vertebral/genética , Neoplasias da Coluna Vertebral/patologia , Translocação Genética , Diagnóstico Diferencial , Humanos , Imageamento por Ressonância Magnética , Masculino , Sarcoma de Ewing/diagnóstico por imagem , Neoplasias de Tecidos Moles , Neoplasias da Coluna Vertebral/diagnóstico por imagem , Adulto Jovem
11.
Braz J Med Biol Res ; 53(2): e8962, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32022102

RESUMO

The aims of this study were to evaluate the intra- and interobserver reproducibility of manual segmentation of bone sarcomas in magnetic resonance imaging (MRI) studies and to compare manual and semiautomatic segmentation methods. This retrospective study included twelve osteosarcoma and eight Ewing sarcoma MRI studies performed prior to any therapeutic intervention. All cases were histopathologically confirmed. Three radiologists used 3D-Slicer software to perform manual segmentation of bone sarcomas in a blinded and independent manner. One radiologist segmented manually and also performed semiautomatic segmentation with the GrowCut tool. Segmentation exercises were timed for comparison. The dice similarity coefficient (DSC) and Hausdorff distance (HD) were used to evaluate similarity between the segmentation results and further statistical analyses were performed to compare DSC, HD, and volumetric results. Manual segmentation was reproducible with intraobserver DSC varying from 0.83 to 0.97 and HD from 3.37 to 28.73 mm. Interobserver DSC of manual segmentation showed variation from 0.73 to 0.97 and HD from 3.93 to 33.40 mm. Semiautomatic segmentation compared to manual segmentation resulted in DSCs of 0.71-0.96 and HDs of 5.38-31.54 mm. Semiautomatic segmentation required significantly less time compared to manual segmentation (P value ≤0.05). Among all situations compared, tumor volumetry did not show significant statistical differences (P value >0.05). We found excellent intra- and interobserver agreement for manual segmentation of osteosarcoma and Ewing sarcoma. There was high similarity between manual and semiautomatic segmentation, with a significant reduction of segmentation time using the semiautomatic method.


Assuntos
Neoplasias Ósseas/diagnóstico por imagem , Osteossarcoma/diagnóstico por imagem , Sarcoma de Ewing/diagnóstico por imagem , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Humanos , Processamento de Imagem Assistida por Computador , Imageamento por Ressonância Magnética , Masculino , Variações Dependentes do Observador , Reprodutibilidade dos Testes , Estudos Retrospectivos , Adulto Jovem
12.
Eur J Radiol ; 124: 108855, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-32018075

RESUMO

PURPOSE: To assess the value of using Apparent Diffusion Coefficient (ADC) mapping in Diffusion Weighted Imaging (DWI) when monitoring treatment response in pediatric Osteogenic and Ewing sarcomas and to correlate with level of necrosis on post-surgical excision pathology. METHOD: This retrospective study includes 7 Osteosarcoma and 8 Ewing sarcoma patients. Pre-treatment and post-treatment focal MRIs were evaluated for ADC values, tumor volumes and variability of both measurements. We also compared the measurement between Ewing and Osteosarcoma groups, as well as between good (=/>90 % necrosis post-excision) and poor (<90 % necrosis post-excision) responders. RESULTS: All measurements except Maximum ADC (p = 0.20) showed a statistically significant difference when comparing them before and after treatment. When we segregated our population according to pathologic complete response, there was no difference in ADC measurements, volumetric measurements or either variability between good (8 Patients) and poor responders (7 Patients). When comparing the before-after changes in our measurement between the Ewing sarcoma and Osteosarcoma cases, there was no significant difference in the change between pre and post treatment (Δ) Mean or Maximum ADC, or in Δtumor-volume when measured on STIR or SPIR T1 post-contrast sequences. Only the ΔMinimum-ADC showed a statistically significant difference (p < 0.02) in this group. CONCLUSIONS: ADC can potentially reflect cellular changes associated with chemotherapy use, reflecting a response to treatment. However, quantitative use of those parameters to dictate a change in management, treatment regimen or chemotherapy dose in order to target a good response (>/ = 90 % necrosis post-excision) needs further investigation.


Assuntos
Neoplasias Ósseas/diagnóstico por imagem , Imagem de Difusão por Ressonância Magnética/métodos , Osteossarcoma/diagnóstico por imagem , Sarcoma de Ewing/diagnóstico por imagem , Adolescente , Neoplasias Ósseas/tratamento farmacológico , Criança , Feminino , Humanos , Masculino , Osteossarcoma/tratamento farmacológico , Estudos Retrospectivos , Sarcoma de Ewing/tratamento farmacológico , Resultado do Tratamento
14.
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
15.
Clin Nucl Med ; 45(2): e103-e105, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31876802

RESUMO

Primary Ewing sarcoma/primitive neuroectodermal tumor is a group of rare aggressive tumors in adults derived from neuroectoderm, and primary renal involvement is extremely rare. We describe an F-FDG PET/CT findings of a 28-year-old man who presented with left renal mass with inferior vena cava thrombus, which turned out to be primary Ewing sarcoma on histopathology specimen post left nephrectomy.


Assuntos
Neoplasias Renais/diagnóstico por imagem , Tumores Neuroectodérmicos/diagnóstico por imagem , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Sarcoma de Ewing/diagnóstico por imagem , Trombose Venosa/diagnóstico por imagem , Adulto , Fluordesoxiglucose F18 , Humanos , Neoplasias Renais/complicações , Masculino , Tumores Neuroectodérmicos/complicações , Compostos Radiofarmacêuticos , Sarcoma de Ewing/complicações , Veia Cava Inferior/diagnóstico por imagem , Veia Cava Inferior/patologia , Trombose Venosa/complicações
16.
Clin Nucl Med ; 45(2): e112-e114, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31876813

RESUMO

Secondary involvement of inferior vena cava is a common presentation in aggressive solid malignancies, especially arising from kidneys, adrenals, and hepatobiliary system, mostly resulting from local infiltration. Rarely, primary tumors are seen arising from vascular tissue. The Ewing family of tumors commonly arises from bone, but there is a high propensity of these tumors to originate from nonosseous sites of mesenchymal cell origin. We would herewith demonstrate a rare presentation of Ewing sarcoma, seen originating from inferior vena cava, presenting as extensive intravascular tumor thrombosis, evaluated on FDG PET/CT imaging in a 12-year-old girl.


Assuntos
Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Sarcoma de Ewing/diagnóstico por imagem , Veia Cava Inferior/diagnóstico por imagem , Criança , Feminino , Fluordesoxiglucose F18 , Humanos , Compostos Radiofarmacêuticos , Veia Cava Inferior/patologia
17.
Urology ; 136: 241-244, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31605682

RESUMO

Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET) of the kidney in children younger than 10 years of age is extremely rare. We describe here the case of a 7-year-old female patient who was diagnosed with ES/PNET. The timeframe for this case spanned from 8 months prior to diagnosis until 8 months postsurgical removal of the tumor. In addition, we summarized the cases of PNET in children younger than 10 years of age in the last decade.


Assuntos
Neoplasias Renais/diagnóstico por imagem , Neoplasias Renais/patologia , Imageamento por Ressonância Magnética , Neoplasias Primárias Múltiplas/diagnóstico por imagem , Neoplasias Primárias Múltiplas/patologia , Tumores Neuroectodérmicos Primitivos Periféricos/diagnóstico por imagem , Tumores Neuroectodérmicos Primitivos Periféricos/patologia , Sarcoma de Ewing/diagnóstico por imagem , Sarcoma de Ewing/patologia , Tomografia Computadorizada por Raios X , Criança , Feminino , Humanos , Estadiamento de Neoplasias
18.
Clin Orthop Relat Res ; 478(2): 290-302, 2020 02.
Artigo em Inglês | MEDLINE | ID: mdl-31580267

RESUMO

BACKGROUND: Local treatment of pelvic Ewing's sarcoma may be challenging, and intergroup studies have focused on improving systemic treatments rather than prospectively evaluating aspects of local tumor control. The Euro-EWING99 trial provided a substantial number of patients with localized pelvic tumors treated with the same chemotherapy protocol. Because local control included surgical resection, radiation therapy, or a combination of both, we wanted to investigate local control and survival with respect to the local modality in this study cohort. QUESTIONS/PURPOSES: (1) Do patients with localized sacral tumors have a lower risk of local recurrence and higher survival compared with patients with localized tumors of the innominate bones? (2) Is the local treatment modality associated with local control and survival in patients with sacral and nonsacral tumors? (3) Which local tumor- and treatment-related factors, such as response to neoadjuvant chemotherapy, institution where the biopsy was performed, and surgical complications, are associated with local recurrence and patient survival in nonsacral tumors? (4) Which factors, such as persistent extraosseous tumor growth after chemotherapy or extent of bony resection, are independently associated with overall survival in patients with bone tumors undergoing surgical treatment? METHODS: Between 1998 and 2009, 1411 patients with previously untreated, histologically confirmed Ewing's sarcoma were registered in the German Society for Pediatric Oncology and Hematology Ewing's sarcoma database and treated in the Euro-EWING99 trial. In all, 24% (339 of 1411) of these patients presented with a pelvic primary sarcoma, 47% (159 of 339) of which had macroscopic metastases at diagnosis and were excluded from this analysis. The data from the remaining 180 patients were reviewed retrospectively, based on follow-up data as of July 2016. The median (range) follow-up was 54 months (5 to 191) for all patients and 84 months (11 to 191) for surviving patients. The study endpoints were overall survival, local recurrence and event-free survival probability, which were calculated with the Kaplan-Meier method and compared using the log-rank test. Hazard ratios (HRs) with their respective 95% CIs were estimated in a multivariate Cox regression model. RESULTS: Sacral tumors were associated with a reduced probability of local recurrence (12% [95% CI 1 to 22] versus 28% [95% CI 20 to 36] at 5 years, p = 0.032), a higher event-free survival probability (66% [95% CI 51 to 81] versus 50% [95% CI 41 to 58] at 5 years, p = 0.026) and a higher overall survival probability (72% [95% CI 57 to 87] versus 56% [95% CI 47 to 64] at 5 years, p = 0.025) compared with nonsacral tumors. With the numbers available, we found no differences between patients with sacral tumors who underwent definitive radiotherapy and those who underwent combined surgery and radiotherapy in terms of local recurrence (17% [95% CI 0 to 34] versus 0% [95% CI 0 to 20] at 5 years, p = 0.125) and overall survival probability (73% [95% CI 52 to 94] versus 78% [95% CI 56 to 99] at 5 years, p = 0.764). In nonsacral tumors, combined local treatment was associated with a lower local recurrence probability (14% [95% CI 5 to 23] versus 33% [95% CI 19 to 47] at 5 years, p = 0.015) and a higher overall survival probability (72% [95% CI 61 to 83] versus 47% [95% CI 33 to 62] at 5 years, p = 0.024) compared with surgery alone. Even in a subgroup of patients with wide surgical margins and a good histologic response to induction treatment, the combined local treatment was associated with a higher overall survival probability (87% [95% CI 74 to 100] versus 51% [95% CI 33 to 69] at 5 years, p = 0.009), compared with surgery alone.A poor histologic response to induction chemotherapy in nonsacral tumors (39% [95% CI 19 to 59] versus 64% [95% CI 52 to 76] at 5 years, p = 0.014) and the development of surgical complications after tumor resection (35% [95% CI 11 to 59] versus 68% [95% CI 58 to 78] at 5 years, p = 0.004) were associated with a lower overall survival probability in nonsacral tumors, while a tumor biopsy performed at the same institution where the tumor resection was performed was associated with lower local recurrence probability (14% [95% CI 4 to 24] versus 32% [95% CI 16 to 48] at 5 years, p = 0.035), respectively.In patients with bone tumors who underwent surgical treatment, we found that after controlling for tumor localization in the pelvis, tumor volume, and surgical margin status, patients who did not undergo complete (defined as a Type I/II resection for iliac bone tumors, a Type II/III resection for pubic bone and ischium tumors and a Type I/II/III resection for tumors involving the acetabulum, according to the Enneking classification) removal of the affected bone (HR 5.04 [95% CI 2.07 to 12.24]; p < 0.001), patients with a poor histologic response to induction chemotherapy (HR 3.72 [95% CI 1.51 to 9.21]; p = 0.004), and patients who did not receive additional radiotherapy (HR 4.34 [95% CI 1.71 to 11.05]; p = 0.002) had a higher risk of death. The analysis suggested that the same might be the case in patients with a persistent extraosseous tumor extension after induction chemotherapy (HR 4.61 [95% CI 1.03 to 20.67]; p = 0.046), although the wide CIs pointing at a possible sparse-data bias precluded any definitive conclusions. CONCLUSION: Patients with sacral Ewing's sarcoma appear to have a lower probability for local recurrence and a higher overall survival probability compared with patients with tumors of the innominate bones. Our results seem to support a recent recommendation of the Scandinavian Sarcoma Group to locally treat most sacral Ewing's sarcomas with definitive radiotherapy. Combined surgical resection and radiotherapy appear to be associated with a higher overall survival probability in nonsacral tumors compared with surgery alone, even in patients with a wide resection and a good histologic response to neoadjuvant chemotherapy. Complete removal of the involved bone, as defined above, in patients with nonsacral tumors may be associated with a decreased likelihood of local recurrence and improved overall survival. Persistent extraosseous tumor growth after induction treatment in patients with nonsacral bone tumors undergoing surgical treatment might be an important indicator of poorer overall survival probability, but the possibility of sparse-data bias in our cohort means that this factor should first be validated in future studies. LEVEL OF EVIDENCE: Level III, therapeutic study.


Assuntos
Neoplasias Ósseas/terapia , Osteotomia , Neoplasias Pélvicas/terapia , Sarcoma de Ewing/terapia , Adolescente , Adulto , Neoplasias Ósseas/diagnóstico por imagem , Neoplasias Ósseas/mortalidade , Neoplasias Ósseas/patologia , Quimioterapia Adjuvante , Criança , Pré-Escolar , Europa (Continente) , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Pessoa de Meia-Idade , Terapia Neoadjuvante , Recidiva Local de Neoplasia , Osteotomia/efeitos adversos , Osteotomia/mortalidade , Neoplasias Pélvicas/diagnóstico por imagem , Neoplasias Pélvicas/mortalidade , Neoplasias Pélvicas/patologia , Intervalo Livre de Progressão , Radioterapia Adjuvante , Ensaios Clínicos Controlados Aleatórios como Assunto , Estudos Retrospectivos , Fatores de Risco , Sarcoma de Ewing/diagnóstico por imagem , Sarcoma de Ewing/mortalidade , Sarcoma de Ewing/patologia , Fatores de Tempo , Adulto Jovem
19.
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
Imageamento 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
20.
Clin Nucl Med ; 45(1): e57-e58, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31524682

RESUMO

We present a case of a 19-year-old woman with Ewing sarcoma of the iliac bone in whom Ga-PSMA-HBED-CC PET/CT showed high radiotracer activity in the primary tumor. The present case documents the in vivo expression of PSMA in Ewing sarcoma family of tumors and adds on to the list of nonprostatic malignancies showing PSMA expression.


Assuntos
Neoplasias Ósseas/diagnóstico por imagem , Ácido Edético/análogos & derivados , Fluordesoxiglucose F18 , Radioisótopos de Gálio , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Sarcoma de Ewing/diagnóstico por imagem , Humanos , Masculino , Adulto Jovem
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