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Ewings sarcoma of the pelvis

Grace Egan, Joanna Pierro, Pillai Pallavi Madhusoodhan, Ghulam Ilyas, Benjamin Cohen, Teena Bhatla
Ewing sarcoma (EWS) is a primitive neuroectodermal tumor arising in bone or soft tissue. It is the second most common primary bone malignancy of children and adolescents, with a peak incidence in the second decade of life. It most often arises in the long bones of the extremities and pelvis. Here, we present a novel case of EWS arising from the mastoid bone in a 5-year-old African American male who presented with symptoms of acute mastoiditis. This unique presentation highlights the importance of considering EWS in a patient who presents with atypical mastoiditis or a rapidly growing mass in the postauricular region...
November 23, 2017: Journal of Pediatric Hematology/oncology
C Liu, L G Cui, H L Wang
Ewing's sarcoma/primitive neuroectodermal tumor (EWS/PNET) in the kidney is a rare but high-grade malignant tumor that affects predominantly elder children and adolescents. Patients mostly present with nonspecific symptoms such as abdominal pain and gross hematuria. Since EWS/PNET has a rapid clinical progression with early metastasis and death, it is essential to make an accurate and early diagnosis. Once diagnosed, multimodality treatment, including radical surgery combined with adjuvant chemotherapy, and radiotherapy if necessary, is recommended...
October 18, 2017: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
Chong Zhu, Kristofor A Olson, Michael Roth, David S Geller, Richard G Gorlick, Jonathan Gill, Nadia N Laack, R L Randall
BACKGROUND: Curative therapy for ES requires both chemotherapy and local control of primary tumor. There is no universally accepted standard approach to local control modalities. This survey was conducted to determine practice patterns and factors influencing the choice to offer various local control modalities to patients with ES of the spine and pelvis. METHODS: The survey consisted of four scenarios involving a 15-year-old girl who presented with Ewing sarcoma of thoracic vertebra, sacrum, iliac wing, and acetabulum with or without neurologic compromise...
October 16, 2017: Journal of Surgical Oncology
Safia K Ahmed, R Lor Randall, Steven G DuBois, William S Harmsen, Mark Krailo, Karen J Marcus, Katherine A Janeway, David S Geller, Joel I Sorger, Richard B Womer, Linda Granowetter, Holcombe E Grier, Richard G Gorlick, Nadia N I Laack
PURPOSE: To identify clinical and treatment variables associated with a higher risk of local failure in Ewing sarcoma patients treated on recent Children's Oncology Group protocols. METHODS AND MATERIALS: Data for 956 patients treated with ifosfamide and etoposide-based chemotherapy on INT-0091, INT-0154, and AEWS0031 were analyzed. Local treatment modalities were defined as surgery, definitive radiation therapy (RT), or surgery plus radiation (S+RT). Five-year cumulative incidence of local failure was determined...
December 1, 2017: International Journal of Radiation Oncology, Biology, Physics
Hongbin Fan, Zheng Guo, Jun Fu, Xiangdong Li, Jing Li, Zhen Wang
The present study describes a novel surgical strategy used to treat immature pelvic Ewing's sarcoma (ES), one made possible owing to the intrinsic structure of the skeletally immature pelvis. A total of 12 children and adolescents with open triradiate cartilage received limb-salvage surgeries following a diagnosis of pelvic ES. In total, 3 patients with iliac lesions (2 lesions with extension into the sacrum) received surgical tumor excisions and allograft reconstructions. Another 8 patients with periacetabular lesions received trans-acetabular osteotomies and allograft reconstructions...
October 2017: Oncology Letters
Vikul Kumar, Anshu Singh, Vivek Sharma, Mohan Kumar
Ewing sarcoma/peripheral primitive neuroectodermal tumor (ES/pPNET) is a malignant small, round cell tumor arising from bone and soft tissue in children and young adults. It can occur at osseous and extraosseous sites. Its usual locations are diaphysis of long bones followed by pelvis, ribs, vertebrae, and rarely skull. We reviewed the literature and PubMed advanced search on ES/pPNET occurring at extraosseous sites, mainly involving the central nervous system (CNS). We reported a case of a 22-year-old male presenting with seizure finally diagnosed as a case of ES/pPNET...
July 2017: Asian Journal of Neurosurgery
Avani Dholakia Rao, Qinyu Chen, Ralph P Ermoian, Sara R Alcorn, Maria Luisa S Figueiredo, Michael J Chen, Karin Dieckmann, Shannon M MacDonald, Matthew M Ladra, Daria Kobyzeva, Alexey V Nechesnyuk, Kristina Nilsson, Eric C Ford, Brian A Winey, Rosangela C Villar, Stephanie A Terezakis
BACKGROUND/OBJECTIVES: The practice of palliative radiation therapy (RT) is based on extrapolation from adult literature. We evaluated patterns of pediatric palliative RT to describe regimens used to identify opportunity for future pediatric-specific clinical trials. DESIGN/METHODS: Six international institutions with pediatric expertise completed a 122-item survey evaluating patterns of palliative RT for patients ≤21 years old from 2010 to 2015. Two institutions use proton RT...
November 2017: Pediatric Blood & Cancer
S Frisch, B Timmermann
As an alternative to conventional photon-based radiotherapy, radiation with protons is recognised to offer considerable advantages. Today, central nervous system tumours, various sarcomatous tumours, childhood cancer and head and neck tumours are commonly treated with proton therapy. This review evaluates current data from clinical and dosimetric trials on the treatment of selected sarcomatous tumours like rhabdomyosarcoma, osteosarcoma, chordoma, chondrosarcoma and Ewing sarcoma. Special considerations for paediatric tumours and future prospects of proton therapy are outlined...
August 2017: Clinical Oncology: a Journal of the Royal College of Radiologists
Ankush Golhar, Samrat Ray, Beate Haugk, Suresh Kumar Singhvi
Ewing's sarcoma is a highly aggressive malignant tumour most commonly affecting long bones in children and adolescents. It is part of the Ewing's sarcoma family of tumours (ESFTs) that also include peripheral primitive neuroectodermal tumour and Askin's tumours. ESFTs share common cytogenetic aberrations, antigenic profiles and proto-oncogene expression with an overall similar clinical course. In 99% of ESFTs, genetic translocation with molecular fusion involves the EWSR1 gene on 22q12. Approximately 30% of ESFTs are extraosseous, most commonly occurring in the soft tissues of extremities, pelvis, retroperitoneum and chest wall...
May 4, 2017: BMJ Case Reports
Safia K Ahmed, Steven I Robinson, Carola A S Arndt, Ivy A Petersen, Michael G Haddock, Peter S Rose, Nadia N Issa Laack
PURPOSE: Local control for Ewing sarcoma (ES) has improved in modern studies. However, it is unclear if these gains have also been achieved for pelvis tumors. The purpose of this study is to evaluate local control and survival in pelvis ES patients treated in the modern era. METHODS: All pelvis ES patients diagnosed from 1990 to 2012 and seen at Mayo Clinic were identified. Factors relevant to survival and local control were analyzed. RESULTS: The cohort consisted of 48 patients...
September 2017: Pediatric Blood & Cancer
Julio Rama-López, Rafael Ramos Asensio, Cesar García-Garza, Pablo Luna Fra, Maria Del Carmen Gassent Balaguer, José Fuster Salva
A broad spectrum of diseases can be included in the differential diagnosis of neck masses. We report a case of extraosseous Ewing sarcoma that presented as a neck mass in a 70-year-old man. To the best of our knowledge, this is the first reported case of extraosseous Ewing sarcoma of the supraclavicular fossa. Published cases of extraosseous Ewing sarcoma in the neck have been described in other age groups, but those tumors were confined to the parapharyngeal space. Also, there have been reported cases in patients older than 70 years in which Ewing sarcoma affected other structures such as the larynx and the pelvis, but none in the soft tissues of the neck...
January 2017: Ear, Nose, & Throat Journal
Miguel Flores, Anthony Caram, Edward Derrick, John D Reith, Laura Bancroft, Kurt Scherer
Ewing sarcoma (ES) is a primary malignant bone tumor which most commonly arises in children and young adults. The common clinical presentation with ES includes nighttime pain or pain related to activity, though patients may also present with a combination of localized swelling, a palpable mass, pathologic fracture, and constitutional symptoms. Clinical diagnosis may be delayed when a patient presents with clinical or imaging findings that overlap with non-malignant etiologies, such as fibrous dysplasia (FD) or osteomyelitis...
September 18, 2016: Curēus
Michelle Mertens, Filip W N Haenen, Vasiliki Siozopoulou, Marc Van Cleemput
INTRODUCTION: Extraskeletal Ewing's sarcoma (EES) is a rare finding in comparison with Ewing's sarcoma of bone and usually manifests in young patients. However, even in older patients, one must consider the diagnosis. PATIENTS AND METHODS: In this case, we describe a 52-year-old woman diagnosed with EES, mimicking as adenocarcinoma of the sigmoid. RESULTS: The tumor was not visualized by a multi-slice spiral computed tomography of the abdomen and pelvis with intravenous contrast, and eventually the diagnosis was made by positive immunohistochemical staining for CD99 and by molecular testing for EWSR1 translocation...
October 4, 2016: Acta Chirurgica Belgica
Minna Laitinen, Michael Parry, Jose I Albergo, Lee Jeys, Vaiyapuri Sumathi, Robert Grimer
BACKGROUND: Malignant bone tumors of the pelvis in children are rare and knowledge of their behavior is limited. METHODS: A total of 113 skeletally immature patients under 16 years of age, comprising 58 females and 55 males were treated between 1983 and 2014. Tumors comprised Ewing's sarcoma (ES) in 88 (77.9%) or osteosarcoma (OS) in 25 (22.1%). Metastases at diagnosis were present in 36 (31.9%). The mean follow-up was 5.2 years (2 to 16). RESULTS: For patients with ES, the overall survival was 37...
September 3, 2016: Journal of Pediatric Orthopedics
J Liu, Y Chen, X L Ling, Y Gong, J P Ding, Z K Zhang, Y J Wang
OBJECTIVE: To explore the clinical, pathological and imaging features of Ewing's sarcoma in pelvis and to improve knowledge and diagnosis of the disease. METHODS: A retrospective analysis of the clinical, pathological and imaging data of pathologically confirmed 13 cases of Ewing's sarcoma in pelvis was carried out between May 2008 and March 2016 in the Affiliated Hospital of Hangzhou Normal University, the Third Hospital of Hebei Medical University and the Second Hospital of Hebei Medical University...
July 19, 2016: Zhonghua Yi Xue za Zhi [Chinese medical journal]
Wan-Shan Li, I-Chuang Liao, Mei-Chin Wen, Howard Haw-Chang Lan, Shih-Chen Yu, Hsuan-Ying Huang
AIMS: BCOR-CCNB3 sarcoma is a genetically defined undifferentiated malignancy with Ewing sarcoma (ES)-like round cells, and preferentially affects the bones of male adolescents. Sarcomas harbouring BCOR-CCNB3 rarely arise from soft tissues; therefore, we aimed to report four cases to expand the clinicopathological spectrum. METHODS AND RESULTS: By reverse transcription polymerase chain reaction and confirmatory sequencing, we detected a BCOR-CCNB3 transcript in primary undifferentiated sarcomas of the deep musculature of four male patients, comprising two teenagers (aged 14 and 17 years) and two adults (aged 34 and 44 years)...
November 2016: Histopathology
Anita P Javalgi, Mahesh H Karigoudar, Katyayani Palur
Ewing's sarcoma is a highly malignant tumour of osseous or non-osseous origin, tremed as extra-skeletal Ewings sarcoma if arising from soft tissue. It is rare occurrence tumor most commonly occurring in paravertebral area, chest wall, head & neck and retroperitoneum. Reporting an interesting case of retroperitoneal Ewing's sarcoma in 39 years old female. Patient had complains of abdominal discomfort & vague pain since 2 months, following weakness in lower limb and loss of weight. On detail history and examination she was further referred to detail pathological and radiological investigations...
April 2016: Journal of Clinical and Diagnostic Research: JCDR
Ozlem O Akagunduz, Serra A Kamer, Burcin Kececi, Bengu Demirag, Haldun Oniz, Mehmet Kantar, Nazan Cetingul, Dundar Sabah, Yavuz Anacak
PURPOSE: To evaluate the results of radiotherapy and the prognostic factors affecting local control in nonextremity Ewing sarcomas. METHODS: Between 1995 and 2011, 44 patients with nonextremity Ewing sarcomas were treated with radiotherapy. Tumor localizations were pelvis in 23, spine in 13, thoracic region in 5, and cranium in 3 patients. Tumor size was ≥8 cm in 56.8% of patients. Distant metastases were present in 19 of the patients at the time of diagnosis (43...
March 2016: Tumori
Asle Charles Hesla, Panagiotis Tsagozis, Nina Jebsen, Olga Zaikova, Henrik Bauer, Otte Brosjö
BACKGROUND: Treatment of Ewing sarcoma of the pelvic bones remains one of the most difficult tasks in the treatment of bone sarcomas. Whether surgery or radiation therapy is the best local treatment is still a matter of debate. The aim of the present study was to compare sacral and nonsacral sites with regard to the treatment and outcome of pelvic Ewing sarcomas. METHODS: Patients with Ewing sarcoma of the osseous pelvis diagnosed between 1986 and 2011 were identified through the Scandinavian Sarcoma Group registry...
February 3, 2016: Journal of Bone and Joint Surgery. American Volume
Partha Ray, Julekha Girach, Anish Pradip Sanghrajka
A 15-year-old girl presented with a 2-month history of non-specific right hip pain associated with pain in the back, right flank and foot. Her symptoms deteriorated, interfering with weight-bearing. Following admission, she was found to be febrile with a flexion deformity of her right-hip, and tenderness over the iliac crest and posterior pelvis. A markedly elevated C reactive protein and erythrocyte sedimentation rate, with an ultrasound-proven scan effusion within the right hip were all suggestive of septic arthritis...
January 21, 2016: BMJ Case Reports
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