176 results on '"Ewing family of tumors"'
Search Results
2. A Trial For Participants With Ewing's Sarcoma Treated With Vigil in Combination With Irinotecan and Temozolomide (VITA)
- Published
- 2023
3. Pretreatment Anti-Therapeutic Antibodies (PATA) in Patients Treated With hu14.18K322A Antibody
- Author
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University of Wisconsin, Madison
- Published
- 2020
4. Editorial: DNA damage response pathways meet endocrine systems
- Author
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Haim Werner and Leonard Girnita
- Subjects
DNA damage (DDR) ,DNA repair ,DNA methylation ,Ewing family of tumors ,mutation ,Diseases of the endocrine glands. Clinical endocrinology ,RC648-665 - Published
- 2023
- Full Text
- View/download PDF
5. Primary spinal multifocal intradural-extramedullary Ewing sarcoma in children: presentation of a case and review of the literature.
- Author
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Müngen, Eren, Kurucu, Nilgün, Kutluk, Tezer, Oğuz, Kader K., Söylemezoğlu, Figen, and Yalçın, Bilgehan
- Abstract
Background. Primary spinal, intradural, extramedullary Ewing sarcoma (PSIEES) is exceptionally uncommon. Here, we present an interesting pediatric case with a PSIEES diagnosis confirmed by the presence of a specific fusion protein in the tumor tissue and who then developed a cerebellar recurrence. We also reviewed the PSIEES cases in childhood reported in the literature. Case. An 8.5-year-old boy was admitted to a local hospital with a one-month history of severe back and limb pain, and inability to move his lower limbs. Physical examination revealed paraparesis in the lower extremities. Spinal MRI revealed multiple intradural extramedullary masses at the L2-L3, L4-5 and L5-S1 levels. He underwent surgery and near total excision of all three masses were performed. Histopathological diagnosis of Ewing Sarcoma was confirmed with EWS-ERG gene rearrangement. The patient was treated according to EuroEwing chemotherapy protocol. A total dose of 4500 cGy radiotherapy was applied to the tumor location at L2-S1 paravertebral region. Eighteen months after the end of treatment, a mass in the left cerebellar hemisphere was determined. Gross total excision was performed. Histopathological examination of the tumor showed Ewing sarcoma. Radiological screening revealed isolated central nervous system recurrence. A total of 4500 cGy radiotherapy was applied. He is on a second-line treatment consisting of gemcitabine and docetaxel without any evidence of disease. Conclusions. Ewing Sarcoma with spinal intradural region in childhood is very rare. We could only find 17 pediatric cases reported in the literature. Neurological findings occur earlier in tumors of this region. The prognosis is worse than other extraosseous Ewing sarcoma. [ABSTRACT FROM AUTHOR]
- Published
- 2021
- Full Text
- View/download PDF
6. Primary uterine Ewing sarcoma – A case report
- Author
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Yen-Chen Wu, Yu-Chien Kao, and Ching-Wen Chang
- Subjects
Ewing family of tumors ,Peripheral primitive neuroectodermal tumors ,Primary Ewing sarcoma ,Uterus ,Gynecology and obstetrics ,RG1-991 - Abstract
Objective: Ewing sarcoma is a type of neuroectodermal tumors (Ewing family of tumors-EFT) that mostly affect the bone or soft tissue. Primary uterine Ewing sarcoma is extremely rare. Case report: We report a case of a primary uterine Ewing sarcoma in a 46-year-old patient, treated with total abdominal hysterectomy, and bilateral salpingo-oophorectomy and following adjuvant chemotherapy with 6 cycles of vincristine, doxorubicin, and cyclophosphamide, achieving complete remission for one year. Conclusion: Complete resection for EFT is the first choice of treatment, regardless of their origins. Adjuvant chemotherapy or radiotherapy is mandatory if needed. Due to rarity of the disease, this report re-emphasizes the accurate diagnosis and appropriate treatment for these unusual tumor types occurred in female genital organs.
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- 2021
- Full Text
- View/download PDF
7. Cross contamination meets misclassification: Awakening of CHP-100 from sleeping beauty sleep-A reviewed model for Ewing's sarcoma.
- Author
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Dirks, Wilhelm Gerhard, Capes-Davis, Amanda, Eberth, Sonja, Fähnrich, Silke, Wilting, Jörg, Nagel, Stefan, Steenpass, Laura, and Becker, Jürgen
- Subjects
EWING'S sarcoma ,MICROSATELLITE repeats ,CELL lines - Abstract
A human cell line of neuroblastic tissue, which was believed to have been lost to science due to its unavailability in public repositories, is revived and reclassified. In the 1970s, a triple set of neuroblastoma (NB) cell lines became available for research as MYCNamplified vs nonamplified models (CHP-126/-134 and CHP-100, respectively). Confusingly, CHP-100 was used in subsequent years as a model for NB and, since the 1990s, as a model for neuroepithelioma and later as a model for Ewing's sarcoma (ES), which inevitably led to non-reproducible results. A deposit at a bioresource center revealed that globally available stocks of CHP-100 were identical to the prominent NB cell line IMR-32 and CHP-100 was included into the list of misidentified cell lines. Now we report on the rediscovery of an authentic CHP-100 cell line and provide evidence of incorrect classification during establishment. We show that CHP-100 cells carry a t(11;22)(q24;q12) type II EWSR1-FLI1 fusion and identify it as a classic ES. Although the question of whether CHP-100 was a virtual and never existing cell line from the beginning is now clarified, the results of all relevant publications should be considered questionable. Neither the time of the cross-contamination event with IMR-32 is known nor was the final classification as a model for Ewing family of tumors available with an associated short tandem repeat profile. After a long road of errors and confusion, authentic CHP-100 is now characterized as a type II EWSR1-FLI1 fusion model 44 years after its establishment. [ABSTRACT FROM AUTHOR]
- Published
- 2021
- Full Text
- View/download PDF
8. Editorial: DNA damage response pathways meet endocrine systems.
- Author
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Werner, Haim and Girnita, Leonard
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DNA repair ,ENDOCRINE system ,DNA methylation ,DNA damage - Published
- 2023
- Full Text
- View/download PDF
9. Primary Cutaneous Ewing Sarcoma/Primitive Neuroectodermal Tumor
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Gardner, Jerad M., Smoller, Bruce R., Rongioletti, Franco, Margaritescu, Irina, and Smoller, Bruce R
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- 2015
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10. Soft Tissue 'Small Round Blue Cell Tumors' of Childhood
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Walters, Matthew P., Zambrano, Eduardo V., and Mackinnon Jr, Alexander Craig, editor
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- 2012
- Full Text
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11. Extraosseous Ewing′s tumor of larynx: A rare presentation
- Author
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Vinod Shinde, Swapnil Gosavi, Rashmi Prashant, Devendra Jain, Gundappa D Mahajan, and Nayanna Karodpati
- Subjects
Ewing family of tumors ,larynx ,primitive neural crest ,primitive neuroectodermal tumor ,Medicine - Abstract
Primitive neuroectodermal tumors (PNETs) are a group of highly malignant tumors composed of small round cells of neuroectodermal origin that affect soft tissue and bone. PNET of the larynx is extremely rare. We report a case of a 41-year-old male who presented with the complaints of progressively increasing stridor of 3 months duration, which was diagnosed as a case of neuroectodermal tumor in the subglottis. Patient was subjected to microlaryngeal surgery and the tumor was excised. Postoperatively, patient was given three cycles of chemotherapy comprising of ifosfamide, etoposide, and mesna, along with granulocyte colony stimulating factor, with 21 days interval. After chemotherapy repeat computed tomography scan showed no evidence of the tumor and no lymphadenopathy. Patient is symptom free for 18 months following completion of treatment. He is under regular follow-up and is undergoing monthly serial endoscopic evaluation.
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- 2015
- Full Text
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12. Extra-osseous Ewing sarcoma of the pancreas: case report with radiologic, pathologic, and molecular correlation, and brief review of the literature.
- Author
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Komforti, Miglena K., Thomas, Rebecca M., Sokolovskaya, Evgeniya, D'Agostino, Catherine A., and Benayed, Ryma
- Abstract
In 2002, due to extensive histomorphologic, immunohistochemical, and cytogenetic similarities, the World Health Organization unified undifferentiated small round blue cell neoplasms of soft tissue and bone (previously segregated as Ewing sarcoma or Primitive Neuroectodermal tumor) into one category: Ewing family of tumors (EFT). Osseous EFT are more common, and while extra-osseous EFT can occur anywhere in the body, those of the pancreas are rare and likely to be seen in the second decade of life in the head of the pancreas. We report the case of a 39-year-old Caucasian male with a large heterogeneously enhancing mass in the pancreatic body. Pathologic examination showed a malignant round blue cell tumor diffusely positive for CD99, chromogranin, and synaptophysin; Ki-67 proliferation index was greater than 80%. FISH showed EWSR1 gene rearrangement in 90% of cells and Archer FusionPlexTM-targeted RNA sequencing analysis identified the EWSR1-FLI1 fusion transcript. The diagnosis of EFT of the pancreas was rendered. Unfortunately, the patient had minimal improvement and was transitioned to oral pain medications to continue care at a different institution. [ABSTRACT FROM AUTHOR]
- Published
- 2018
- Full Text
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13. Multiscale-omic assessment of EWSR1-NFATc2 fusion positive sarcomas identifies the mTOR pathway as a potential therapeutic target
- Author
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Colin M Stets, Richard D Maradiaga, Alan Rogers, Sherri Z. Millis, John L. Hays, Howard M. Katzenstein, Nathan D. Seligson, and James L. Chen
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0301 basic medicine ,Cancer Research ,Genomics ,Disease ,Predictive markers ,Article ,Tumour biomarkers ,03 medical and health sciences ,0302 clinical medicine ,Downregulation and upregulation ,Ewing family of tumors ,Medicine ,PI3K/AKT/mTOR pathway ,RC254-282 ,business.industry ,Cancer ,Neoplasms. Tumors. Oncology. Including cancer and carcinogens ,Sarcoma ,medicine.disease ,Precision medicine ,030104 developmental biology ,Oncology ,030220 oncology & carcinogenesis ,Cancer research ,business - Abstract
Sarcomas harboring EWSR1-NFATc2 fusions have historically been categorized and treated as Ewing sarcoma. Emerging evidence suggests unique molecular characteristics and chemotherapy sensitivities in EWSR1-NFATc2 fusion positive sarcomas. Comprehensive genomic profiles of 1024 EWSR1 fusion positive sarcomas, including 14 EWSR1-NFATc2 fusions, were identified in the FoundationCore® database. Additional data from the Gene Expression Omnibus, the Genomics of Drug Sensitivity in Cancer and The Cancer Genome Atlas datasets were included for analysis. EWSR1-NFATc2 fusion positive sarcomas were genomically distinct from traditional Ewing sarcoma and demonstrated upregulation of the mTOR pathway. We also present a case of a 58-year-old male patient with metastatic EWSR1-NFATc2 fusion positive sarcoma who achieved 47 months of disease stabilization when treated with combination mTOR and VEGF inhibition. EWSR1-NFATc2 fusion positive sarcomas are molecularly distinct entities with overactive mTOR signaling; which may be therapeutically targetable. These findings support the use of precision medicine in the Ewing family of tumors.
- Published
- 2021
14. AnEWSR1-CREB3L1Fusion Gene in Extraskeletal Undifferentiated Round Cell Sarcoma Expands the Spectrum of Genetic Landscape in the 'Ewing-Like' Undifferentiated Round Cell Sarcomas
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Alan Rogers, Konstantin Shilo, David A. Liebner, Caroline Bissonnette, Raphael E. Pollock, and O. Hans Iwenofu
- Subjects
Pathology ,medicine.medical_specialty ,CD99 ,Biology ,medicine.disease ,Undifferentiated Round Cell Sarcoma ,Small Cell Osteosarcoma ,Pathology and Forensic Medicine ,Fusion gene ,Ewing family of tumors ,FLI1 ,medicine ,Immunohistochemistry ,Surgery ,Sarcoma ,Anatomy - Abstract
The molecular findings in Ewing sarcoma have greatly expanded in recent years. Furthermore, this is particularly true for the subset termed “Ewing-like” undifferentiated round cell sarcomas in which new translocations have been reported since the fourth edition of the WHO Classification of Tumours of Soft Tissue and Bone. Amid this expanding genetic landscape, we report a case of extraskeletal undifferentiated round cell “Ewing-like” sarcoma in a 27-year-old female. The patient presented with a large lung mass accompanied on staging imaging by deposits suspicious for metastatic disease in the humerus, calvarium, and lymph nodes of the neck and chest. Biopsy of the lung mass revealed a densely packed monotonous proliferation of round, uniform neoplastic cells with scant cytoplasm. By immunohistochemistry, the tumor cells were diffusely positive for CD99, synaptophysin, TLE1, EMA, and MUC4 and negative for FLI1, PAX7, AE1/3, S100, SOX10, WT1, p63, desmin, and HMB45. Fluorescence in situ hybridization demonstrated rearrangement of the EWSR1 gene. Next-generation sequencing based assay revealed an EWSR1-CREB3L1 fusion. Taken together, the histomorphologic and molecular findings were considered consistent with an undifferentiated round cell sarcoma with an EWSR1-CREB3L1 fusion. Although described in entities such as sclerosing epithelioid fibrosarcoma, low-grade fibromyxoid sarcoma, and small cell osteosarcoma, this has not been previously described in undifferentiated round cell (“Ewing-like”) sarcoma. This finding adds to the growing list of undifferentiated round cell sarcomas with Ewing-like morphologic phenotype–associated fusion genes and may contribute to further defining and characterizing the different subset of tumors in the Ewing family of tumors.
- Published
- 2020
15. Cytopathological findings of primary pulmonary Ewing family of tumors with EWSR1 translocation: A case report.
- Author
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Mizuguchi, Keishi, Minato, Hiroshi, Onishi, Hitomi, Mitani, Yuki, and Kawai, Jun
- Abstract
Primary pulmonary neoplasms of the Ewing family of tumors ( EFT) are extremely rare and usually occur in adolescents or young adults. Only about 40 cases of pulmonary EFT have been reported in English literature, and no cytological studies have been documented. In this report, we describe the cytopathological findings of a primary pulmonary EFT in an elderly patient. A 70-year-old man sought care because of a progressing cough and dyspnea. Chest computed tomography revealed a circumscribed mass of 6 cm in the left upper lobe. Fine needle aspiration cytology and core needle biopsy revealed uniform round cell proliferation. The predominant population consisted of cells with thickened nuclear membranes, finely dispersed chromatin, single distinct nucleoli, and indistinct cytoplasm. The other population consisted of smaller cells with darker chromatin. The cytoplasm stained positive for periodic acid-Schiff stain and was digested by diastase. Immunohistochemistry showed positivity for MIC2 ( CD99), and focal positivity for neuron specific enolase, synaptophysin, and chromogranin A. Fluorescence in situ hybridization ( FISH) revealed EWSR1 translocation. Although rare, pulmonary EFT cannot be disregarded, regardless of age. When two populations of uniform, round cells are observed, immunohistochemistry with MIC2 (CD99) and cytogenetic analysis by reverse transcription polymerase chain reaction or FISH should be considered. Cytological diagnosis may play an important role in the early diagnosis and treatment of pulmonary EFT. [ABSTRACT FROM AUTHOR]
- Published
- 2016
- Full Text
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16. Successful Radical Pneumonectomy for a Primitive Neuroendodermal Tumor in the Lung: A Case Report and Review of the Literature
- Author
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Jiankai Wang, Fanying Liu, Pan Yin, Hua Jiang, Duo Zhao, Peng Chen, Bingqing Yue, and Jingyu Chen
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medicine.medical_specialty ,RD1-811 ,diagnosis ,medicine.medical_treatment ,CD99 ,Case Report ,Malignancy ,03 medical and health sciences ,Pneumonectomy ,0302 clinical medicine ,Ewing family of tumors ,Medicine ,primitive neuroendodermal tumors ,Lymph node ,pneumonectomy ,small round cell malignant tumors ,Lung ,business.industry ,030224 pathology ,medicine.disease ,Dissection ,medicine.anatomical_structure ,030220 oncology & carcinogenesis ,Surgery ,Sarcoma ,Radiology ,prognosis ,business - Abstract
Peripheral primitive neuroendodermal tumors (PNETs) and Ewing's sarcoma belong to the Ewing family of tumors and are small round-cell malignancies originating from spinal cord cells. These tumors account for 5% of all small round-cell malignant neoplasms. PNETs that arise from the lung parenchyma without pleural or chest wall involvement are very rare. We report a case of an adult female with a large pulmonary PNET who had given birth just 1 month prior to the diagnosis. She had cough and expectoration for 6 months, and the preoperative examination showed no metastases. Thus, we performed radical pneumonectomy and lymph node dissection. The patient recovered well without surgical complications and was discharged 7 days after the surgery. Postoperative pathology confirmed that the tumor was a small round-cell malignancy, and the tumor cells were positive for CD99, Friend leukemia virus integration 1 (FLI-1), and neuron-specific enolase (NSE), which was consistent with the diagnosis of a PNET. For primary large pulmonary PNETs, radical pneumonectomy may be a safe surgical method, worthy of further application in clinical practice.
- Published
- 2021
17. Gastric Ewing Sarcoma identified on a Meckel's scan
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Lavi Nissim and Gerald A. Mandell
- Subjects
lcsh:Medical physics. Medical radiology. Nuclear medicine ,Pathology ,medicine.medical_specialty ,PNET ,Axial skeleton ,lcsh:R895-920 ,Ewing Sarcoma ,Meckel's scan ,Malignancy ,030218 nuclear medicine & medical imaging ,03 medical and health sciences ,0302 clinical medicine ,Ewing family of tumors ,medicine ,Radiology, Nuclear Medicine and imaging ,Primitive Neuroectodermal tumor ,business.industry ,Stomach ,medicine.disease ,Primary bone ,medicine.anatomical_structure ,Primitive neuroectodermal tumor ,Osteosarcoma ,Sarcoma ,Nuclear Medicine ,business ,030217 neurology & neurosurgery - Abstract
Ewing Sarcoma is a malignant small round blue cell tumor most commonly found in bones and soft tissues of the axial skeleton and extremities. The Ewing family of tumors, including peripheral neuroectodermal tumor, represent the second most common malignancy in the pediatric population and second most common primary bone tumor after osteosarcoma. In a majority of Ewing Sarcoma cases, there is a translocation between chromosomes 11 and 22. Extraskeletal Ewing Sarcoma of the stomach is exceptionally rare, with only a handful of case reports. Here we report a case of primary Ewing Sarcoma of the stomach found initially as a filling defect in the stomach on technetium-99m pertechnetate scintigraphy to evaluate for gastrointestinal bleeding.
- Published
- 2020
18. Primary Ewing Family of Tumor Arising in the Ovary
- Author
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Chi-An Chen, Tom Wei-Wu Chen, Yi-Ping Li, Koping Chang, Shu-Ping Lee, and Wen-Fang Cheng
- Subjects
Adult ,0301 basic medicine ,Pathology ,medicine.medical_specialty ,Ovary ,Sarcoma, Ewing ,Pathology and Forensic Medicine ,Metastasis ,03 medical and health sciences ,Ovarian tumor ,0302 clinical medicine ,Ewing family of tumors ,medicine ,Humans ,Neuroectodermal Tumors, Primitive, Peripheral ,Etoposide ,Ovarian Neoplasms ,Ifosfamide ,Peripheral Primitive Neuroectodermal Tumor ,business.industry ,Obstetrics and Gynecology ,medicine.disease ,030104 developmental biology ,medicine.anatomical_structure ,030220 oncology & carcinogenesis ,Female ,Sarcoma ,business ,medicine.drug - Abstract
Ewing sarcoma and peripheral primitive neuroectodermal tumor constitute the Ewing family of tumors (EFT). EFTs primarily arising in the ovary are extremely rare. We report the case of a 22-yr-old nulliparous woman with a primary EFT in the ovary that initially presented as a 3-cm teratoma-like ovarian tumor, with rapid progression to a 15-cm-sized tumor with liver metastasis in 3 mo. The patient underwent suboptimal debulking surgery and salvage chemotherapy with vincristine, doxorubicin, and cyclophosphamide alternating with ifosfamide and etoposide. In conclusion, primary EFT in the ovary is extremely rare with highly aggressive behavior and poor outcome for metastatic disease. Demonstration of EWSR1 rearrangement, observed in a variety of soft tissue tumors, is very helpful in the diagnosis of EFT when interpreted on the basis morphology and immunohistochemistry.
- Published
- 2019
19. The Ewing Family of Tumors Relies on BCL-2 and BCL-XL to Escape PARP Inhibitor Toxicity
- Author
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Sheeba Jacob, Colin M. Coon, Steven C. Smith, Joel D. Leverson, Maninderjit S. Ghotra, Yuki Kato Maves, Giovanna T. Stein, Andrew J. Souers, Daniel A. R. Heisey, Krista M. Powell, Konstantinos V. Floros, Marissa L. Calbert, Timothy L. Lochmann, Sosipatros A. Boikos, Cyril H. Benes, and Anthony C. Faber
- Subjects
0301 basic medicine ,Cancer Research ,Navitoclax ,Combination therapy ,biology ,business.industry ,Venetoclax ,Bcl-xL ,medicine.disease ,Olaparib ,03 medical and health sciences ,chemistry.chemical_compound ,030104 developmental biology ,0302 clinical medicine ,Oncology ,chemistry ,Ewing family of tumors ,030220 oncology & carcinogenesis ,PARP inhibitor ,biology.protein ,Cancer research ,Medicine ,Sarcoma ,business - Abstract
Purpose: It was recently demonstrated that the EWSR1-FLI1 t(11;22)(q24;12) translocation contributes to the hypersensitivity of Ewing sarcoma to PARP inhibitors, prompting clinical evaluation of olaparib in a cohort of heavily pretreated Ewing sarcoma tumors. Unfortunately, olaparib activity was disappointing, suggesting an underappreciated resistance mechanism to PARP inhibition in patients with Ewing sarcoma. We sought to elucidate the resistance factors to PARP inhibitor therapy in Ewing sarcoma and identify a rational drug combination capable of rescuing PARP inhibitor activity. Experimental Design: We employed a pair of cell lines derived from the same patient with Ewing sarcoma prior to and following chemotherapy, a panel of Ewing sarcoma cell lines, and several patient-derived xenograft (PDX) and cell line xenograft models. Results: We found olaparib sensitivity was diminished following chemotherapy. The matched cell line pair revealed increased expression of the antiapoptotic protein BCL-2 in the chemotherapy-resistant cells, conferring apoptotic resistance to olaparib. Resistance to olaparib was maintained in this chemotherapy-resistant model in vivo, whereas the addition of the BCL-2/XL inhibitor navitoclax led to tumor growth inhibition. In 2 PDXs, olaparib and navitoclax were minimally effective as monotherapy, yet induced dramatic tumor growth inhibition when dosed in combination. We found that EWS-FLI1 increases BCL-2 expression; however, inhibition of BCL-2 alone by venetoclax is insufficient to sensitize Ewing sarcoma cells to olaparib, revealing a dual necessity for BCL-2 and BCL-XL in Ewing sarcoma survival. Conclusions: These data reveal BCL-2 and BCL-XL act together to drive olaparib resistance in Ewing sarcoma and reveal a novel, rational combination therapy that may be put forward for clinical trial testing.
- Published
- 2019
20. Risk factors for detectable metastatic disease at presentation in Ewing sarcoma – An analysis of the SEER registry
- Author
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Benjamin J. Miller, Niveditta Ramkumar, Eric Henderson, and Dipak B. Ramkumar
- Subjects
Adult ,Male ,0301 basic medicine ,Oncology ,Cancer Research ,medicine.medical_specialty ,Adolescent ,Epidemiology ,Bone Neoplasms ,Sarcoma, Ewing ,Disease ,Malignancy ,Logistic regression ,Young Adult ,03 medical and health sciences ,0302 clinical medicine ,Risk Factors ,Ewing family of tumors ,Internal medicine ,medicine ,Humans ,Registries ,Neoplasm Metastasis ,Child ,Univariate analysis ,business.industry ,Prognosis ,medicine.disease ,Primary tumor ,Logistic Models ,030104 developmental biology ,Child, Preschool ,030220 oncology & carcinogenesis ,Female ,Sarcoma ,business ,SEER Program - Abstract
Ewing family of tumors (EFT) represents the second-most common primary bone malignancy in children and adolescents. Approximately 25% of patients have radiographically detectable metastatic disease at presentation and experience poorer five-year survival, yet risk factors for metastatic disease at presentation are poorly characterized. We sought to study patient characteristics associated with metastatic disease upon presentation for patients with EFT.We identified EFT cases in the Surveillance, Epidemiology, and End Results Program (SEER) registry from 2004 to 2012. Using univariate analyses and multivariable logistic regression, we explored the relationship between demographic and clinical factors and the presence of detectable metastatic disease at presentation.Among 870 EFT cases, 35% (n = 304) presented with detectable metastatic disease. These patients were commonly older (24 years: 28% vs 19%, p = 0.002) and had a primary tumor site in the axial skeleton (56% vs 44%, p 0.001). After adjusting for all covariates, compared to patients11 years, those24 years old faced a two-fold increase in the odds of metastatic disease (OR = 1.99, 95% CI: 1.17-3.38). Axial (OR = 2.31, 95% CI: 1.58-3.37) and "other" (OR = 2.35, 95% CI: 1.15-4.81) tumor locations had more than twice the likelihood of presenting with metastatic disease, compared to extremity tumor sites. Increasing tumor size conferred up to a three-fold increase in odds of metastatic disease (pTrend0.001).Advanced age, axial tumor location, and increasing tumor size are associated with increased odds of detectable metastatic disease upon presentation with EFT. Although these characteristics are not modifiable, they provide objective factors that may inform patient counseling of metastatic risk.
- Published
- 2018
21. Extraosseous Ewing's tumor of larynx: A rare presentation.
- Author
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Shinde, Vinod, Gosavi, Swapnil, Prashant, Rashmi, Jain, Devendra, Mahajan, Gundappa D., and Karodpati, Nayanna
- Subjects
- *
NEUROECTODERMAL tumors , *CANCER , *EMBRYONAL tumors , *LARYNX ,CENTRAL nervous system tumors - Abstract
Primitive neuroectodermal tumors (PNETs) are a group of highly malignant tumors composed of small round cells of neuroectodermal origin that affect soft tissue and bone. PNET of the larynx is extremely rare. We report a case of a 41-yearold male who presented with the complaints of progressively increasing stridor of 3 months duration, which was diagnosed as a case of neuroectodermal tumor in the subglottis. Patient was subjected to microlaryngeal surgery and the tumor was excised. Postoperatively, patient was given three cycles of chemotherapy comprising of ifosfamide, etoposide, and mesna, along with granulocyte colony stimulating factor, with 21 days interval. After chemotherapy repeat computed tomography scan showed no evidence of the tumor and no lymphadenopathy. Patient is symptom free for 18 months following completion of treatment. He is under regular follow-up and is undergoing monthly serial endoscopic evaluation. [ABSTRACT FROM AUTHOR]
- Published
- 2015
- Full Text
- View/download PDF
22. Ewing-Like Sarcomas
- Author
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Simone Mocellin
- Subjects
business.industry ,Ewing family of tumors ,Cancer research ,Medicine ,Sarcoma ,business ,medicine.disease - Abstract
Ewing-like sarcomas are also known as Ewing-like sarcoma family, Ewing-like tumor family, atypical Ewing sarcomas, and Ewing-like tumors. The terms Ewing sarcoma family of tumors and Ewing family of tumors include both Ewing sarcoma (osseous and extraosseous) and Ewing-like sarcomas.
- Published
- 2020
23. The clinical heterogeneity of round cell sarcomas with EWSR1/FUS gene fusions: Impact of gene fusion type on clinical features and outcome
- Author
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Paul A. Meyers, Lei Zhang, Cristina R. Antonescu, William D. Tap, John H. Healey, and Yusuke Tsuda
- Subjects
Oncology ,Adult ,Male ,Cancer Research ,medicine.medical_specialty ,NFATC2 ,Adolescent ,Oncogene Proteins, Fusion ,Bone Neoplasms ,Sarcoma, Ewing ,Biology ,Article ,Fusion gene ,03 medical and health sciences ,0302 clinical medicine ,Ewing family of tumors ,Internal medicine ,Genetics ,medicine ,Round cell ,Biomarkers, Tumor ,Humans ,Oncogene Fusion ,Child ,Gene ,Cells, Cultured ,Aged ,ETS transcription factor family ,Infant ,Middle Aged ,medicine.disease ,Primary tumor ,Survival Analysis ,030220 oncology & carcinogenesis ,Child, Preschool ,RNA-Binding Protein FUS ,Female ,Sarcoma ,RNA-Binding Protein EWS - Abstract
The genetic hallmark of classic Ewing sarcoma is a recurrent fusion between EWSR1 and FUS gene with a member of the ETS transcription factor family. In contrast, tumors with non-ETS gene partners have been designated until recently "Ewing-like sarcoma," as a provisional molecular entity, as their clinical and pathologic features were still evolving. However, this group was reclassified as "round cell sarcoma with EWSR1-non-ETS fusions" in the latest 2020 WHO classification. Moreover, round cell sarcomas with either CIC or BCOR gene abnormalities, initially classified under Ewing family of tumors, are now regarded as stand-alone pathologic entities based on their distinct features. In this study we investigated the clinical characteristics of 226 confirmed Ewing sarcoma patients (EWSR1-FLI1 [n = 176], EWSR1/FUS-ERG [n = 35], EWSR1/FUS-FEV [n = 12], and EWSR1-ETV1/4 [n = 3]) and 14 round cell sarcoma patients with EWSR1-non-ETS fusion (EWSR1/FUS-NFATC2 [n = 10], EWSR1-PATZ1 [n = 3], and EWSR1-VEZF1 [n = 1]). The impact on overall survival (OS) was assessed in 90 patients with available follow-up, treated between 2011 and 2018. Patients with fusions involving FEV and NFATC2 genes showed an older median age at diagnosis, compared to those with EWSR1-FLI1 (P = .005), while extraskeletal location was more common in tumors with noncanonical EWSR1-FLI1 fusions (P = .001). Axial and pelvic primary sites were more common in patients with EWSR1-FLI1 (72%), while tumors with NFATC2 fusions were more frequent in the limb (78%, P = .006). The 3-year OS in patients with EWSR1-FLI1 was 91%, compared to only 60% in patients with alternative fusions (P = .037); the latter group showing a higher rate of metastases at presentation. However, this OS difference was not significant in patients with localized tumor (P = .585). Our study demonstrates significant correlations between fusion subtype and age at presentation, primary tumor sites, and OS, in both conventional Ewing sarcoma and round cell sarcoma with EWSR1-non ETS fusions patients. Larger studies are needed to determine survival differences in localized tumors.
- Published
- 2020
24. Delayed Diagnosis of Metastatic Ewing Sarcoma Masked by Charcot-Marie-Tooth Disease.
- Author
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Menjak, Ines B., Gupta, Abha, and Grinman, Michelle N.
- Subjects
- *
CANCER diagnosis , *LUNG cancer -- Case studies , *CHARCOT-Marie-Tooth disease - Abstract
An 18-year-old male with a history of Charcot-Marie-Tooth disease (CMT) presented with metastatic Ewing sarcoma to the lungs. He had been followed by several healthcare professionals who ascribed his enlarging 23 cm gluteal mass to his CMT. The patient experienced a significant delay in diagnosis, not uncommon in sarcoma. This case explores the various system and cognitive errors that contributed to this delay. [ABSTRACT FROM AUTHOR]
- Published
- 2013
- Full Text
- View/download PDF
25. FDG PET/CT of Primary Ewing Sarcoma of IVC
- Author
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Ritesh R Suthar, Archi Agrawal, Sneha Shah, Venkatesh Rangarajan, Ameya D Puranik, Akshay Sharad Bedmutha, and Nilendu Purandare
- Subjects
medicine.medical_specialty ,Vena Cava, Inferior ,Sarcoma, Ewing ,Inferior vena cava ,030218 nuclear medicine & medical imaging ,03 medical and health sciences ,0302 clinical medicine ,Ewing family of tumors ,Fluorodeoxyglucose F18 ,Positron Emission Tomography Computed Tomography ,medicine ,Humans ,Radiology, Nuclear Medicine and imaging ,Child ,business.industry ,Mesenchymal stem cell ,General Medicine ,medicine.disease ,Thrombosis ,medicine.vein ,030220 oncology & carcinogenesis ,cardiovascular system ,Local infiltration ,Fdg pet ct ,Female ,Radiology ,Sarcoma ,Presentation (obstetrics) ,Radiopharmaceuticals ,business - Abstract
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.
- Published
- 2019
26. Diagnostic utility of dual-color EWS break-apart fluorescence in-situ hybridization probe in the diagnosis and differential diagnosis of pediatric Ewing family of tumors
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Nehal Kamal, Naglaa Elkinaai, Enas Mohsen, and Manal Zamzam
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Pathology ,medicine.medical_specialty ,medicine.diagnostic_test ,Ewing family of tumors ,business.industry ,medicine ,Differential diagnosis ,business ,Dual color ,Fluorescence in situ hybridization - Published
- 2018
27. Prostatic carcinoma with neuroendocrine differentiation harboring the EWSR1-FEV fusion transcript in a man with the WRN G327X germline mutation: A new variant of prostatic carcinoma or a member of the Ewing sarcoma family of tumors?
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Jyotsna Kochiyil, Robert J. Poppiti, Christopher A. Febres-Aldana, Kritika Krishnamurthy, Ana Maria Medina, and Ruben Delgado
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0301 basic medicine ,Male ,Werner Syndrome Helicase ,Oncogene Proteins, Fusion ,Sarcoma, Ewing ,Biology ,Neuroendocrine differentiation ,Pathology and Forensic Medicine ,Loss of heterozygosity ,03 medical and health sciences ,Prostate cancer ,0302 clinical medicine ,Germline mutation ,Ewing family of tumors ,medicine ,Humans ,Germ-Line Mutation ,In Situ Hybridization, Fluorescence ,High-Throughput Nucleotide Sequencing ,Prostatic Neoplasms ,Cell Differentiation ,Cell Biology ,medicine.disease ,DNA-Binding Proteins ,030104 developmental biology ,Fusion transcript ,Amino Acid Substitution ,030220 oncology & carcinogenesis ,Primitive neuroectodermal tumor ,Cancer research ,Sarcoma ,RNA-Binding Protein EWS ,Transcription Factors - Abstract
Since the discovery of the TMPRSS2-ERG fusion transcript in prostatic carcinoma (PCa) more than ten years ago, a long list of recurrent genomic rearrangements involving other transcription factors of the ETS family has been described. Fusions of ETS with the EWSR1 partner gene define many members of the Ewing family of tumors, including primitive neuroectodermal tumor (PNET). Although the expression of EWSR1 appears to be necessary for the oncogenic effects of ETS factors, the EWSR1-ETS rearrangement has never been reported in PCa. Herein, we discuss the pathologic diagnosis of a prostatic tumor in a 44 year-old man, recently treated with finasteride, with the EWSR1-FEV fusion (exon 7: exon 2, join in-frame) discovered by RNA-sequencing and fluorescence in situ hybridization. The tumor was morphologically and immunophenotypically equivocal for a Ewing sarcoma/PNET, and most consistent with a PCa with neuroendocrine differentiation. The patient's family history of PCa led to germline mutation testing by next-generation sequencing showing heterozygosity for the WRNG327X mutation. The WRN protein along with ATM, BRCA1, BRCA2, and RAD51 among others, comprise a DNA repair system by homologous recombination, and its alterations are associated with forms of hereditary PCa. We dispute whether the detection of EWSR1-FEV mandates one to diagnose the patient's tumor as a member of the Ewing sarcoma family.
- Published
- 2019
28. Extra-osseous Ewing sarcoma of the pancreas: case report with radiologic, pathologic, and molecular correlation, and brief review of the literature
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Ryma Benayed, Miglena K. Komforti, Evgeniya Sokolovskaya, Rebecca M. Thomas, and Catherine A. D’Agostino
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Adult ,Male ,Pathology ,medicine.medical_specialty ,Oncogene Proteins, Fusion ,Proliferation index ,CD99 ,Sarcoma, Ewing ,Pathology and Forensic Medicine ,03 medical and health sciences ,0302 clinical medicine ,Ewing family of tumors ,medicine ,Humans ,Molecular Biology ,biology ,business.industry ,Chromogranin A ,Cell Biology ,General Medicine ,medicine.disease ,Pancreatic Neoplasms ,medicine.anatomical_structure ,Fusion transcript ,030220 oncology & carcinogenesis ,Primitive neuroectodermal tumor ,biology.protein ,030211 gastroenterology & hepatology ,Sarcoma ,Pancreas ,business - Abstract
In 2002, due to extensive histomorphologic, immunohistochemical, and cytogenetic similarities, the World Health Organization unified undifferentiated small round blue cell neoplasms of soft tissue and bone (previously segregated as Ewing sarcoma or Primitive Neuroectodermal tumor) into one category: Ewing family of tumors (EFT). Osseous EFT are more common, and while extra-osseous EFT can occur anywhere in the body, those of the pancreas are rare and likely to be seen in the second decade of life in the head of the pancreas. We report the case of a 39-year-old Caucasian male with a large heterogeneously enhancing mass in the pancreatic body. Pathologic examination showed a malignant round blue cell tumor diffusely positive for CD99, chromogranin, and synaptophysin; Ki-67 proliferation index was greater than 80%. FISH showed EWSR1 gene rearrangement in 90% of cells and Archer FusionPlexTM-targeted RNA sequencing analysis identified the EWSR1-FLI1 fusion transcript. The diagnosis of EFT of the pancreas was rendered. Unfortunately, the patient had minimal improvement and was transitioned to oral pain medications to continue care at a different institution.
- Published
- 2018
29. Metastatic Primitive Neuroectodermal Tumor of the Prostate: A Case Report and Review of the Literature
- Author
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Nour Dababo, Ejaz Shamim, Ali Kord Valeshabad, Paul Choi, Karen L. Xie, and Alaa Alsadi
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Adult ,Male ,Pathology ,medicine.medical_specialty ,Urology ,030232 urology & nephrology ,03 medical and health sciences ,Fatal Outcome ,0302 clinical medicine ,Drug Therapy ,Ewing family of tumors ,Prostate ,Positron Emission Tomography Computed Tomography ,medicine ,Humans ,Neuroectodermal Tumors, Primitive ,Prostatectomy ,medicine.diagnostic_test ,business.industry ,Prostatic Neoplasms ,Magnetic resonance imaging ,Prognosis ,medicine.disease ,Treatment Outcome ,medicine.anatomical_structure ,Oncology ,030220 oncology & carcinogenesis ,Primitive neuroectodermal tumor ,business - Published
- 2018
30. Chest Wall Ewing Sarcoma Family of Tumors: Long-Term Outcomes
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Indelicato, Daniel J., Keole, Sameer R., Lagmay, Joanne P., Morris, Christopher G., Gibbs, C. Parker, Scarborough, Mark T., Islam, Saleem, and Marcus, Robert B.
- Subjects
- *
EWING'S sarcoma , *HEALTH outcome assessment , *CANCER radiotherapy , *CANCER of unknown primary origin , *FOLLOW-up studies (Medicine) , *PHYSIOLOGICAL effects of radiation , *THERAPEUTICS - Abstract
Purpose: To review the 40-year University of Florida experience treating Ewing sarcoma family of tumors of the chest wall. Methods and Materials: Thirty-nine patients were treated from 1966 to 2006. Of the patients, 22 were treated with radiotherapy (RT) alone, and 17 patients were treated with surgery with or without RT. Of 9 patients with metastatic disease, 8 were treated with RT alone. The risk profiles of each group were otherwise similar. The median age was 16.6 years, and the most frequent primary site was the rib (n = 17). The median potential follow-up was 19.2 years. Results: The 5-year actuarial overall survival (OS), cause-specific survival (CSS), and local control (LC) rates were 34%, 34%, and 72%, respectively. For the nonmetastatic subset (n = 30), the 5-year OS, CSS, and LC rates were 44%, 44%, and 79%, respectively. LC was not statistically significantly different between patients treated with RT alone (61%) vs. surgery + RT (75%). None of the 4 patients treated with surgery alone experienced local failure. No patient or treatment variable was significantly associated with local failure. Of the patients, 26% experienced Common Toxicity Criteria (CTC) Grade 3+ toxicity, including 2 pulmonary deaths. Modern intensive systemic therapy helped increase the 5-year CSS from 7% to 49% in patients treated after 1984 (p = 0.03). Conclusions: This is the largest single-institution series describing the treatment of chest wall Ewing tumors. Despite improvements in survival, obtaining local control is challenging and often accompanied by morbidity. Effort should be focused on identifying tumors amenable to combined-modality local therapy and to improving RT techniques. [ABSTRACT FROM AUTHOR]
- Published
- 2011
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31. Understanding Micrometastatic Disease and Anoikis Resistance in Ewing Family of Tumors and Osteosarcoma.
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Strauss, Sandra J., Ng, Tony, Mendoza-Naranjo, Ariadna, Whelan, Jeremy, and Sorensen, Poul H. B.
- Subjects
CANCER cells ,CLINICAL medicine ,DRUG therapy ,IMMUNE system ,CANCER invasiveness ,OSTEOSARCOMA - Abstract
Detection of micrometastatic tumor cells in the bone marrow or peripheral blood of patients with Ewing family of tumors (EFTs) and osteosarcoma has been shown to correlate with poor outcome. Although one of the aims of chemotherapy is eradication of micrometastatic disease, these cells vary phenotypically from primary tumor cells and appear to be more resistant to chemotherapy. As a barrier to metastasis, cells normally undergo a form of cell death termed anoikis after they lose contact with the extracellular matrix or neighboring cells. Tumor cells that acquire malignant potential have developed mechanisms to resist anoikis and thereby survive after detachment from their primary site and while traveling through the circulation. Investigating mechanisms of resistance to anoikis, therefore, provides a valuable model to investigate regulation of micrometastatic disease. This review focuses on the current understanding of the mechanisms involved in mediating cell survival and resistance to anoikis in EFTs and osteosarcoma and discusses future studies that may help to identify novel therapeutics targeted at micrometastatic disease. [ABSTRACT FROM AUTHOR]
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- 2010
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32. Spinal and Paraspinal Ewing Tumors
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Indelicato, Daniel J., Keole, Sameer R., Shahlaee, Amir H., Morris, Christopher G., Gibbs, C. Parker, Scarborough, Mark T., Pincus, David W., and Marcus, Robert B.
- Subjects
- *
EWING'S sarcoma , *OSTEOSARCOMA , *CANCER radiotherapy , *FOLLOW-up studies (Medicine) , *HEALTH outcome assessment , *SPINAL cord tumors , *PATIENTS , *TUMOR treatment , *THERAPEUTICS - Abstract
Purpose: To perform a review of the 40-year University of Florida experience treating spinal and paraspinal Ewing tumors. Patients and Methods: A total of 27 patients were treated between 1965 and 2007. For local management, 21 patients were treated with radiotherapy (RT) alone and 6 with surgery plus RT. All patients with metastatic disease were treated with RT alone. The risk profiles of each group were otherwise similar. The median age was 17 years, and the most frequent subsite was the sacral spine (n = 9). The median potential follow-up was 16 years. Results: The 5-year actuarial overall survival, cause-specific survival, and local control rate was 62%, 62%, and 90%, respectively. For the nonmetastatic subset (n = 22), the 5-year overall survival, cause-specific survival, and local control rate was 71%, 71%, and 89%, respectively. The local control rate was 84% for patients treated with RT alone vs. 100% for those treated with surgery plus RT. Patients who were >14 years old and those who were treated with intensive therapy demonstrated superior local control. Of 9 patients in our series with Frankel C or greater neurologic deficits at presentation, 7 experienced a full recovery with treatment. Of the 27 patients, 37% experienced Common Toxicity Criteria Grade 3 or greater toxicity, including 2 deaths from sepsis. Conclusion: Aggressive management of spinal and paraspinal Ewing tumors with RT with or without surgery results in high toxicity but excellent local control and neurologic outcomes. Efforts should be focused on identifying disease amenable to combined modality local therapy and improving RT techniques. [Copyright &y& Elsevier]
- Published
- 2010
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33. Radiation Treatment for Ewing Family of Tumors in Adults: The University of Florida Experience
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Shi, Wenyin, Indelicato, Daniel J., Keole, Sameer R., Morris, Christopher G., Scarborough, Mark T., Gibbs, Parker C., and Zlotecki, Robert A.
- Subjects
- *
RADIOTHERAPY - Abstract
Purpose: To review the clinical characteristics and outcomes of adult patients with Ewing family of tumors treated with radiation at the University of Florida. Methods and Materials: Clinical features, treatment, and outcomes of 47 patients older than 18 years with Ewing family of tumors treated with combined radiation therapy and chemotherapy from 1970 to 2005 were retrospectively reviewed. Analysis was stratified by age older or younger than 30 years. Patients with metastatic disease at the time of diagnosis were excluded from the study. Results: The 29 men and 18 women had a median age of 24 years. Thirty-three patients were 18–30 years old and 14 patients were older than 30 years. Median follow-up of living patients was 8.2 years. The 5-year overall survival rate for all patients was 43% (p = 0.8523). The 5-year local control rate for all patients was 75% (p = 0.9326). The 5-year rate of freedom from distant metastasis for all patients was 45% (p = 0.5471). There were no significant differences in 5-year overall survival, local control, and freedom from distant metastasis rates; patterns of distant failure; or toxicity profiles between older adult patients and younger adult patients. Conclusions: We found that the natural history and treatment outcomes of the Ewing family of tumors were consistently similar in adults (young and old) and children. Thus, aggressive combined modality approaches should be considered for adult patients. [Copyright &y& Elsevier]
- Published
- 2008
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34. Prediction of response and prognostic factors for Ewing family of tumors in a low incidence population.
- Author
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Yonemori, Kan, Yamaguchi, Umio, Kaneko, Masayuki, Uno, Hajime, Takeuchi, Masahiro, Ando, Masashi, Fujiwara, Yasuhiro, Hosono, Ako, Makimoto, Atsushi, Hasegawa, Tadashi, Yokoyama, Ryouhei, Nakatani, Fumihiko, Kawai, Akira, Beppu, Yasuo, and Chuman, Hirokazu
- Subjects
- *
CANCER invasiveness , *EWING'S sarcoma , *TUMORS , *PROGNOSIS , *DRUG therapy , *METASTASIS - Abstract
There is some unknown reason Ewing family of tumors (EFTs) is much less common on Asia and Africa than in the Western Caucasian population. This study analyzed the prediction of response and prognostic factors for Ewing family of tumors (EFTs) in an Asian population with a low incidence. We retrospectively reviewed 94 patients with EFTs between 1978 and 2006. Fifteen patients received local therapy only. Statistical analyses were performed for 79 patients, including those who received systemic chemotherapy, to identify factors related to chemotherapy responsiveness, event-free survival, and overall survival. Of the 79 patients whose records were analyzed, the 5-year event-free rate and overall survival (OS) rate were 41 and 54%, respectively. The response rate to first-line chemotherapy was 61% in 70 patients with assessable lesions. A significant predictor of response was existence of a non-pelvic primary tumor ( P = 0.04). Significant prognostic factors for OS were age, performance status, and metastases at the time of diagnosis ( P < 0.01, respectively). Fifty-four patients had disease progression or recurrence after first-line treatment. The time to progression was 3.4 months after salvage treatment. Progression during first-line treatment was significantly associated with time to progression after salvage treatment ( P = 0.01). All patients treated without chemotherapy in first-line treatment were recurred with poor prognosis. A non-pelvic primary tumor was a favorable predictor of responsiveness to chemotherapy. Chemo-resistant patients might less benefit from second line chemotherapy. Chemotherapy in first-line treatment should not be omitted, even if primary tumor was extirpated completely. [ABSTRACT FROM AUTHOR]
- Published
- 2008
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35. Ewing Sarcoma tumor cells express CD34: implications for autologous stem cell transplantation.
- Author
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Yaniv, I., Stein, J., Luria, D., Cohen, I. J., Liberzon, E., Manor, S., Grunshpan, A., Sverdlov, Y., Kodman, Y., Issakov, J., Feinmesser, M., Zaizov, R., and Avigad, S.
- Subjects
- *
EWING'S sarcoma , *BONE cancer , *CD antigens , *GENETIC transcription , *GENE expression , *CELL transplantation , *STEM cells , *CANCER cells - Abstract
The significance of tumor cell contamination in marrow and peripheral blood stem cell (PBSC) collections of patients with solid tumors remains controversial. Various methods have been developed to purge tumor cells from autologous stem cell products, including CD34+ selection. PBSC harvests from patients with Ewing family of tumors (EFT) were analyzed for contaminating tumor cells prior and after CD34+ selection using reverse transcription-polymerase chain reaction (RT-PCR) and flow cytometry (FC) analyzes. The expression of CD34 was studied by RT-PCR and FC in 14 primary tumors and 13 PBSC harvests, respectively. Tumor cells were identified in the harvests by both methods. In two patients, contaminating tumor cells were evident by RT-PCR only after positive selection. FC analysis confirmed a higher level of tumor cells in the CD34+ fraction. In an attempt to explore this finding, expression of CD34 was detected in 93% of primary tumors and 67% of contaminated harvests. As CD34 is expressed on EFT cells, these cells may be enriched following CD34+ selection of harvests, although the total number of tumor cells is reduced. Other methods of purging, rather than CD34+ selection, should be explored in patients with EFT undergoing autologous stem cell transplantation.Bone Marrow Transplantation (2007) 39, 589–594. doi:10.1038/sj.bmt.1705640; published online 19 March 2007 [ABSTRACT FROM AUTHOR]
- Published
- 2007
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36. Atypical Presentation of Primary Non-metastatic Ewings Sarcoma of the Lumbosacral Spine-'Cauda Equina Syndrome' Clinically and a 'Tuberculosis mimicking challenge' on MRI-A Case Report
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Mohit Kukreja, Chetan Anchan, and Vidyadhar G. Telang
- Subjects
Pathology ,medicine.medical_specialty ,business.industry ,Cauda equina ,Cauda equina syndrome ,medicine.disease ,Malignancy ,Sacrum ,medicine.anatomical_structure ,Ewing family of tumors ,Primitive neuroectodermal tumor ,medicine ,General Earth and Planetary Sciences ,Sarcoma ,business ,Lumbosacral joint ,General Environmental Science - Abstract
Introduction: The Ewing family of tumors comprises Ewing's sarcoma (EWS), extraskeletal EWS, primitive neuroectodermal tumor (PNET) of bone and soft tissue, and chest wall tumor (Askin tumor). The translocation t(11; 22) (q24; q12) is identified in more than 90% of cases.EWS is the second most common primary bone malignancy in childhood. In contrast to long bone involvement, delays in spinal EWS diagnosis may occur because symptoms may not be present until neurological deficits occur. To date, although there have been reported cases of EWS in the lumbosacral region, the reported cases are very less and the presentations might drastically differ. Case Presentation: A 14 year old boy comes with a 12 weeks h/o urinary incontinence and constipation. Over the next 4 months, the patient had new-onset intermittent abdominal pain with worsening urinary symptoms.MRI confirmed a diagnosis of Koch's spine. However, the histopathology reported it as an “Ewings sarcoma/Primitive Neuroectodermal Tumor”. Discussion: Ewing’s tumor of sacrum is rare, but should be suspected in low backache in children. Cauda equina syndrome can be a valid presentation for EWS spine. MRI can identify cases early and enables early treatment though it is not specific. Histopathological diagnosis is a must before any definitive management.EWS spine can mimick Tuberculosis both clinico-radiologically, only to be confirmed with a tissue biopsy. Conclusion: Ewing’s sarcoma of the lumbosacral spine can have an atypical presentation and there should be a high degree of suspicion to diagnose it early.
- Published
- 2017
37. What is the impact of local control in Ewing sarcoma: analysis of the first Brazilian collaborative study group – EWING1
- Author
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Reynaldo Jesus Garcia Filho, Carlos Roberto Galia, Alexandre David, Julie Francine Cerutti Santos, Eduardo Areas Toller, Gerardo Badell, Suely Akiko Nakagawa, Ricardo Gehrke Becker, Algemir Lunardi Brunetto, André Mathias Baptista, Lauro José Gregianin, Osvaldo André Serafini, Valter Penna, and Eduardo S. Yonamime
- Subjects
0301 basic medicine ,Male ,Cancer Research ,medicine.medical_treatment ,Radiation oncology ,0302 clinical medicine ,Ewing family of tumors ,Surgical oncology ,Medicine ,Cumulative incidence ,Neoplasm Metastasis ,Child ,lcsh:Neoplasms. Tumors. Oncology. Including cancer and carcinogens ,Combined Modality Therapy ,Magnetic Resonance Imaging ,Tumor Burden ,Treatment Outcome ,Oncology ,030220 oncology & carcinogenesis ,Child, Preschool ,Female ,Sarcoma ,Brazil ,Research Article ,Adult ,medicine.medical_specialty ,Adolescent ,Bone Neoplasms ,Sarcoma, Ewing ,lcsh:RC254-282 ,03 medical and health sciences ,Young Adult ,Genetics ,Bone tumors ,Humans ,In patient ,Neoplasm Staging ,Proportional Hazards Models ,business.industry ,Local failure ,medicine.disease ,Surgery ,Radiation therapy ,030104 developmental biology ,Orthopedics ,Local control ,Orthopedic surgery ,business ,Tomography, X-Ray Computed ,Ewing sarcoma - Abstract
Children's Cancer Institute Rafael Accordi Foundation, Porto Alegre, RS, Brazil Background: Relapse in localized Ewing sarcoma patients has been a matter of concern regarding poor prognosis. Therefore, we investigated the impact of local control modality (surgery, surgery plus radiotherapy, and radiotherapy) on clinical outcomes such as survival and recurrence in patients with non-metastatic Ewing sarcoma treated on the first Brazilian Collaborative Group Trial of the Ewing Family of Tumors (EWING1). Methods: Seventy-three patients with localized Ewing sarcoma of bone aged < 30 years were included. The treating physicians defined the modality of local control based on the recommendations of the coordinating center and the patient and tumor characteristics. Possible associations of local control modality with local failure (LF), disease-free survival (DFS), event-free survival (EFS), overall survival (OS), and clinical characteristics were analyzed. Results: Mean patient age was 12.8 years (range, 2 to 25 years) and median follow-up time was 4.5 years (range, 2. 3 to 6.7 years). Forty-seven patients underwent surgery, 13 received radiotherapy, and 13 received both. The 5-year EFS, OS, and DFS for all patients was 62.1%, 63.3%, and 73.1%, respectively. The 5-year cumulative incidence (CI) of LF was 7.6% for surgery, 11.1% for radiotherapy, and 0% for postoperative radiotherapy (PORT) (p = 0.61). The 5-year EFS was 71.7% for surgery, 30.8% for radiotherapy, and 64.1% for PORT (p = 0.009). Conclusions: There was a significant effect of local control modality on EFS and OS in the study. Surgery and PORT modalities yielded very close results. The group treated with radiotherapy alone had considerably worse outcomes. This may be confounded by greater risk factors in these patients. There was no significant effect of local control modality on the CI of LF and DFS. HCPA, Serv Orthoped & Traumatol, Rua Ramiro Barcelos 2350, BR-90035903 Porto Alegre, RS, Brazil Univ Fed Rio Grande do Sul, HCPA, Dept Pediat, Porto Alegre, RS, Brazil Pontificia Univ Catolica Rio Grande Sul PUCRS, Dept Pediat, Hosp Sao Lucas, Porto Alegre, RS, Brazil HCPA, Serv Orthoped & Traumatol, Porto Alegre, RS, Brazil Univ Fed Sao Paulo UNIFESP, Support Grp Children & Adolescents Canc GRAACC, Sao Paulo, SP, Brazil Hosp Canc Infantojuvenil, Fundacao Pio 12, Barretos, SP, Brazil Ctr Hosp Pereira Rossell, Montevideo, Uruguay Hosp AC Camargo Canc Ctr, Orthoped Serv, Sao Paulo, SP, Brazil Santa Casa Misericordia Porto Alegre, Serv Orthoped & Traumatol, Porto Alegre, RS, Brazil Univ Sao Paulo, Orthoped Trauma Inst, Hosp Clin Sao Paulo, Sch Med, Sao Paulo, SP, Brazil Santa Casa Misericordia Sao Paulo HSCSP, Dept Orthoped & Traumatol, Sao Paulo, SP, Brazil Pontificia Univ Catolica Rio Grande Sul PUCRS, Hosp Sao Lucas, Serv Orthoped & Traumatol, Porto Alegre, RS, Brazil Univ Estadual Paulista UNESP, Hosp Clin Botucatu, Sch Med, Botucatu, SP, Brazil Inst Canc Infantil, Porto Alegre, RS, Brazil Univ Fed Sao Paulo UNIFESP, Support Grp Children & Adolescents Canc GRAACC, Sao Paulo, SP, Brazil Children's Cancer Institute Rafael Accordi Foundation, Porto Alegre, RS, Brazil Web of Science
- Published
- 2017
38. <atl>High-dose chemoradiotherapy (HDC) in the Ewing family of tumors (EFT)
- Author
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Burdach, S. and Jürgens, H.
- Subjects
- *
EWING'S sarcoma , *DRUG therapy , *STEM cells , *THERAPEUTICS - Abstract
EFT is defined by the expression of ews/ets fusion genes. The type of the fusion transcript impacts on the clinical biology. EFT requires risk adapted treatment. A risk-adapted treatment is determined by tumor localisation, tumor stage and volume. For metastatic and relapsed disease the pattern of spread and the time of relapse are the determinants of risk stratification. Staging of Ewing tumors has been considerably improved by magnetic resonance imaging and modern isotope scanning techniques. However, the determination of the extent of the metastatic spread in particular number of involved bones remains an unresolved issue. The prognosis for high-risk Ewing tumors has been improved by multimodal and high-dose radio/chemotherapy (HDC). The concepts for high-dose therapy in Ewing tumors are based on dose response and dose intensity relationships. In single agent HDC most experience exists with Melphalan. Several chemotherapeutic agents have been used in combination HDC with or without TBI such as Adriamycin, BCNU, Busulphan, Carboplatin, Cyclophosphamide, Etoposide, Melphalan, Thiotepa Procarbazin and Vincristine. Todate, superiority of any high-dose chemotherapy regimen has not been established. However, the clinical biology, the pattern of spread and the time of relapse determine the prognosis of patient who are eligible for HDC. In particular, patients with multifocal bone or bone marrow metastases have a poorer prognosis than patients with lung metastases. In addition, patients with a relapse within 24 months have a poorer prognosis than patients with a relapse later than 24 months after diagnosis. This review will analyze the results of single- and multi-agent chemotherapy with respect to agent combination, dose and risk stratum of patient population. Future therapeutic modalities for the treatment of EFT might encompass immunotherapeutic and genetic strategies including allogeneic stem cell transplantation. [Copyright &y& Elsevier]
- Published
- 2002
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39. Highly aggressive undifferentiated small round blue cell tumor of foot with unique SMARCA1, KAT6A and NAV3 mutations
- Author
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Robert Orlando, Michael Marcus, Ravin Rupani, Cyrus Parsa, and Jin Guo
- Subjects
Mutation ,business.industry ,Ewing's sarcoma ,Cancer ,Case Report ,medicine.disease_cause ,medicine.disease ,Ewing family of tumors ,Tumor progression ,FLI1 ,medicine ,Cancer research ,Surgery ,business ,Chromosome 22 ,Gene - Abstract
Malignancies characterized histologically by high-grade monotonous small round blue cells (SRBCs) belong to a heterogeneous group of neoplasms often referred to as Ewing family of tumors. The most common molecular confirmation of these neoplasms is by fusions between EWSR1 gene on chromosome 22 and the ETS family of transcription factors, including FLI1 gene (11q24) and the ERG (21q22), that are implicated in the development of different tissues as well as cancer progression. In this article, we present a case of highly aggressive extraskeletal SRBC tumor involving the foot of a 24-year-old male with sole molecular findings of mutations in KAT6A, NAV3 and SMARCA1 genes with high expression of soft tissue markers (COL1A1, COL1A2, COL3A1) and MYC mRNA. To our knowledge, this unique mutational pattern has not previously been described in SRBCs.
- Published
- 2019
40. Management of recurrent or refractory Ewing sarcoma: A systematic review of phase II clinical trials in the last 15 years
- Author
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Sen Dong, Xin Sun, Xiaodong Tang, Lu Xie, Jie Xu, and Wei Guo
- Subjects
0301 basic medicine ,Oncology ,Cancer Research ,medicine.medical_specialty ,medicine.medical_treatment ,Phases of clinical research ,Targeted therapy ,03 medical and health sciences ,chemistry.chemical_compound ,0302 clinical medicine ,Ewing family of tumors ,Regorafenib ,Internal medicine ,medicine ,Chemotherapy ,business.industry ,Articles ,medicine.disease ,Clinical trial ,Irinotecan ,030104 developmental biology ,chemistry ,030220 oncology & carcinogenesis ,Sarcoma ,business ,medicine.drug - Abstract
The aim of the present study was to evaluate the antitumor activity of drugs in phase II clinical trials for recurrent or refractory EWS. A systematic review was performed using clinical trials from four data sources: i) ClinicalTrials.gov; ii) PubMed; iii) Clinicaltrialsregister.eu; and iv) American Society of Clinical Oncology. The search terms included: '(Ewing sarcoma OR Ewing family of tumors) AND (phase II OR phase I/II)'. Overall, 465 trials were identified and 64 were included in the present study, of which, 37 had published results. The highest objective response rate came from irinotecan-based chemotherapy. Currently, the majority of targeted therapy has failed to demonstrate any activity except for regorafenib. Trials using anti-angiogenesis small molecular tyrosine kinase inhibitors (aaTKIs) are currently ongoing with promising early results. For immunotherapy, anti-insulin like growth factor 1 receptor antibody demonstrated disappointing activity. The best outcome came from irinotecan-based regimens. Targeted therapy with aaTKIs is worthy of further investigation, with immunotherapy is not recommended for off-label use.
- Published
- 2019
41. Cytopathological findings of primary pulmonary Ewing family of tumors with EWSR1 translocation: A case report
- Author
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Keishi Mizuguchi, Hitomi Onishi, Yuki Mitani, Hiroshi Minato, and Jun Kawai
- Subjects
Pulmonary and Respiratory Medicine ,Pathology ,medicine.medical_specialty ,Ewing family of tumors ,CD99 ,Population ,Case Report ,Case Reports ,lung ,03 medical and health sciences ,0302 clinical medicine ,Biopsy ,medicine ,030212 general & internal medicine ,education ,education.field_of_study ,elderly man ,Lung ,medicine.diagnostic_test ,biology ,business.industry ,Chromogranin A ,General Medicine ,medicine.anatomical_structure ,Oncology ,EWSR1 ,030220 oncology & carcinogenesis ,biology.protein ,Aspiration cytology ,Immunohistochemistry ,business ,Fluorescence in situ hybridization - Abstract
Primary pulmonary neoplasms of the Ewing family of tumors (EFT) are extremely rare and usually occur in adolescents or young adults. Only about 40 cases of pulmonary EFT have been reported in English literature, and no cytological studies have been documented. In this report, we describe the cytopathological findings of a primary pulmonary EFT in an elderly patient. A 70-year-old man sought care because of a progressing cough and dyspnea. Chest computed tomography revealed a circumscribed mass of 6 cm in the left upper lobe. Fine needle aspiration cytology and core needle biopsy revealed uniform round cell proliferation. The predominant population consisted of cells with thickened nuclear membranes, finely dispersed chromatin, single distinct nucleoli, and indistinct cytoplasm. The other population consisted of smaller cells with darker chromatin. The cytoplasm stained positive for periodic acid–Schiff stain and was digested by diastase. Immunohistochemistry showed positivity for MIC2 (CD99), and focal positivity for neuron specific enolase, synaptophysin, and chromogranin A. Fluorescence in situ hybridization (FISH) revealed EWSR1 translocation. Although rare, pulmonary EFT cannot be disregarded, regardless of age. When two populations of uniform, round cells are observed, immunohistochemistry with MIC2 (CD99) and cytogenetic analysis by reverse transcription polymerase chain reaction or FISH should be considered. Cytological diagnosis may play an important role in the early diagnosis and treatment of pulmonary EFT.
- Published
- 2016
42. The Differential Diagnosis of Sinonasal/Nasopharyngeal Neuroendocrine/Neuroectodermally Derived Tumors
- Author
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Kathleen T. Montone
- Subjects
Adenoma ,Pathology ,medicine.medical_specialty ,Nose Neoplasms ,Esthesioneuroblastoma, Olfactory ,Neuroendocrine tumors ,Pituitary neoplasm ,Neuroendocrine differentiation ,Nose neoplasm ,Pathology and Forensic Medicine ,Diagnosis, Differential ,Paraganglioma ,Ewing family of tumors ,medicine ,Humans ,Pituitary Neoplasms ,Melanoma ,business.industry ,General Medicine ,Sinonasal Tract ,medicine.disease ,Neuroendocrine Tumors ,Medical Laboratory Technology ,Nasal Cavity ,Differential diagnosis ,business ,Paranasal Sinus Neoplasms - Abstract
ContextThe differential diagnosis of neuroendocrine neoplasms arising in the sinonasal tract is broad and includes lesions of epithelial, mesenchymal, and neuroectodermal origin.ObjectiveTo review the differential diagnosis of sinonasal neuroendocrine and neuroectodermally derived tumors.Data SourcesThe current literature was reviewed to provide updated information regarding the differential diagnosis and means for diagnosing neuroendocrine tumors including sinonasal neuroendocrine carcinoma, olfactory neuroblastoma, malignant melanoma, paraganglioma, pituitary adenoma, and Ewing family of tumors.ConclusionsThe differential diagnosis of neoplasms with neuroendocrine differentiation in the sinonasal tract is broad, and diagnosis often includes not only histologic review but also immunohistochemical or molecular analysis.
- Published
- 2015
43. AGGRESSIVE PRIMITIVE TUMOR IN A PEDIATRIC PATIENT WITH LETHAL EVOLUTION
- Author
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Hendri Kerole Silva De Oliveira, Lioney Nobre Cabral, Naelka Sarmento, Tiago Novaes Pinheiro, Milena Gomes Melo Leite, and André Luiz Tannus Dutra
- Subjects
Pathology ,medicine.medical_specialty ,biology ,business.industry ,Calponin ,Mucous membrane ,medicine.disease ,Pathology and Forensic Medicine ,Lesion ,Pediatric patient ,medicine.anatomical_structure ,Ewing family of tumors ,Mucoepidermoid carcinoma ,medicine ,biology.protein ,Immunohistochemistry ,Radiology, Nuclear Medicine and imaging ,Dentistry (miscellaneous) ,Surgery ,Oral Surgery ,medicine.symptom ,business ,Telangiectasia - Abstract
Primitive tumors are considered rare, malignant, small and round-cell tumors that represent a disease of childhood and categorized into the Ewing family of tumors due its similarities. Herein we report a case of primitive tumor in a pediatric patient with lethal evolution. A 1-year-old black male patient was showing an expansive lesion in the left hemimandible, 8 cm in diameter, exhibiting superficial telangiectasia, crackling consistency, similar to a mucous membrane. The radiographic findings revealed irregular lytic radiolucency. An incisional biopsy was performed and the histopathologic report suggested a mucoepidermoid carcinoma. A histochemical and immunohistochemical panel was performed with the following markers: PAS-stain (+), DAK-p63 (+), AE1 ⁄AE3 (+), S-100 (+), and calponin (focaly +). The patient died 2 months after the biopsy procedure, before the immunohistochemical report was finished. We discuss the aggressive nature of such a rare tumor.
- Published
- 2020
44. Analysis of bone and soft-tissue sarcomas registered during the year 2012 at Tata Memorial Hospital, Mumbai, with clinical outcomes
- Author
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Jaya Ghosh, Ajay Puri, Prakash Nayak, Mukta Ramadwar, Nehal Khanna, Jyoti Bajpai, Shashikant Juvekar, Bharat Rekhi, Bhanupriya Sanduptla, Tushar Vora, Ashish Gulia, Amit Janu, Venkatesh Rangarajan, Subhash Desai, Nilendu Purandare, Siddhartha Laskar, and Girish Chinnaswamy
- Subjects
Adult ,Male ,medicine.medical_specialty ,Adolescent ,medicine.medical_treatment ,Chondrosarcoma ,Bone Neoplasms ,Sarcoma, Ewing ,Disease-Free Survival ,03 medical and health sciences ,Young Adult ,0302 clinical medicine ,Ewing family of tumors ,Positron Emission Tomography Computed Tomography ,medicine ,Humans ,030212 general & internal medicine ,Ifosfamide ,Child ,Aged ,Aged, 80 and over ,Chemotherapy ,Osteosarcoma ,business.industry ,Soft tissue sarcoma ,Soft tissue ,Sarcoma ,Middle Aged ,medicine.disease ,Synovial sarcoma ,Methotrexate ,Treatment Outcome ,Oncology ,Amputation ,Doxorubicin ,030220 oncology & carcinogenesis ,Cohort ,Female ,Radiotherapy, Adjuvant ,Radiology ,business - Abstract
Introduction: Primary bone and soft tissue sarcomas are rare, but diagnostically and therapeutically challenging group of tumors, requiring multidisciplinary management. There are limited documented studies from multidisciplinary teams , in the form of comprehensive analysis of these tumors, from our country. This study is an analysis of cases of osteosarcomas, Ewing sarcomas (ESs), chondrosarcomas (CSs), and soft-tissue sarcomas (STSs), registered at our institution during 2012. Methods: Clinical details, including outcomes of cases of bone and STSs, during the year 2012, were retrieved from the medical records of our institution and were further analyzed. Results: Ninety-five high-grade, extremity-based, treatment-naive cases of osteosarcomas were treated with a novel, dose-dense, nonhigh-dose methotrexate-based OGS-12 protocol. Good histopathologic response (necrosis ≥90%) was achieved in 59% nonmetastatic and 56% metastatic patients. At a median follow-up of 48 months, the estimated 5-year event-free survival and overall survival (OS) were 67% and 78%, respectively. In the metastatic cohort at a median follow-up of 51 months, the 5-year estimated progression-free survival was 24% and OS was 26%. Among 87 (73.2%) nonmetastatic and 32 (26.8%) metastatic, analyzable cases of ES, at a median follow-up of 40 months, the disease-free survival (DFS) and OS in the nonmetastatic group were 62% and 83%; in the metastatic group, they were 37.5% and 65.6%, respectively. Among 40 cases of CSs (33 nonmetastatic and 7 metastatic), 21 had limb salvage surgery while 5 had amputation. Microscopically, 90.4% were Grade II CSs. Five-year OS and DFS were 84.6% and 71%, respectively. Among 189 high-grade, extremity-based STSs (89% nonmetastatic), synovial sarcoma was the most common subtype (31%). Eighty-five percent had limb preservation surgery; a majority were offered adjuvant radiation with or without chemotherapy. At a median follow-up of 51 (1–63) months, 3-year local control, DFS, and OS were 81%, 48%, and 64%, respectively. Conclusions: The novel OGS 12 and Ewing Family of Tumors 2001 protocols have shown comparable outcomes to international standards in cases of osteosarcoma and ES, respectively, and merit wider applications, especially in low- and middle-income countries (LMICs). Outcomes in STS and CSs were also comparable and underscore the importance of a multidisciplinary approach for the management of sarcomas in LMICS.
- Published
- 2018
45. Ewing Sarcoma Presenting as a Congenital Scalp Mass
- Author
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Jacqueline A Haas, Dahlia M Rice, and Clinton S. Morrison
- Subjects
Pathology ,medicine.medical_specialty ,Scalp ,Extraosseous Ewing Sarcoma ,business.industry ,Soft tissue ,030206 dentistry ,Sarcoma, Ewing ,medicine.disease ,Scalp mass ,03 medical and health sciences ,0302 clinical medicine ,medicine.anatomical_structure ,Otorhinolaryngology ,Ewing family of tumors ,Medicine ,Humans ,Sarcoma ,Oral Surgery ,Age of onset ,Presentation (obstetrics) ,030223 otorhinolaryngology ,business - Abstract
Ewing sarcoma is a locally aggressive, highly malignant tumor most commonly seen in the skeletal system. The "Ewing family of tumors" also includes other tissue types that are not common, such as soft tissue origin classified as extraosseous Ewing sarcoma (EES) or primitive neuroendocrine origin. Age of onset most often occurs within the first 2 decades of life. Congenital presentation of EES is exceedingly rare. We report the first described case to our knowledge of congenital EES originating from the scalp.
- Published
- 2018
46. Ewing Sarcoma and the History of Similar and Possibly Related Small Round Cell Tumors: From Whence Have We Come and Where are We Going?
- Author
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Scott E. Kilpatrick, John D. Reith, and Brian P. Rubin
- Subjects
0301 basic medicine ,Oncology ,medicine.medical_specialty ,Desmoplastic small-round-cell tumor ,Biopsy ,Context (language use) ,Bone Neoplasms ,Sarcoma, Ewing ,History, 21st Century ,Small Cell Osteosarcoma ,Pathology and Forensic Medicine ,Diagnosis, Differential ,03 medical and health sciences ,0302 clinical medicine ,Ewing family of tumors ,Predictive Value of Tests ,Internal medicine ,medicine ,Biomarkers, Tumor ,Pathology ,Humans ,Neuroectodermal tumor ,business.industry ,History, 20th Century ,medicine.disease ,Immunohistochemistry ,Mesenchymal chondrosarcoma ,030104 developmental biology ,Molecular Diagnostic Techniques ,030220 oncology & carcinogenesis ,Primitive neuroectodermal tumor ,Sarcoma ,Anatomy ,Diffusion of Innovation ,business ,Forecasting - Abstract
The diagnosis of small round cell tumors always has been extremely difficult, and our current classification systems continue to evolve. Since its initial discovery by Dr James Ewing, the historical context of what is acceptably included under the designation "Ewing sarcoma" has changed. Although Ewing sarcoma and primitive neuroectodermal tumor were both initially described in the early 20th century, these tumors were considered likely distinct entities until the end of that same century, almost 75 years later. With modern immunohistochemistry and more recent advances in molecular techniques, the understanding of Ewing sarcoma and Ewing-like tumors has improved dramatically but also raises new questions and challenges. We now know that this category of tumors is remarkably more heterogenous than initially thought, especially in regards to its cytogenetics and molecular properties, and some of these differences likely have prognostic relevance. Whether we are now expanding the spectrum of Ewing sarcoma or simply recognizing new entities is controversial. Therapeutic approaches to address these new categories and/or entities need further focus and attention. Herein, we provide a comprehensive historical perspective on Ewing sarcoma, Ewing-like tumors (CIC and BCOR-rearranged sarcomas), and related and/or similar small round cell tumors, often included in the differential diagnosis, including mesenchymal chondrosarcoma, desmoplastic small round cell tumor, and small cell osteosarcoma. We also seek to provide updates and insights into the evolving classification and clinical relevance of the Ewing family of tumors.
- Published
- 2018
47. The Prognostic Value of Initial Tumor Size in Patients with Ewing Family of Tumors
- Author
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Sushil Ranamagar, Amit Joshi, Rajeeb Kumar Deo, and Prakash Chitalkar
- Subjects
Oncology ,medicine.medical_specialty ,Tumor size ,business.industry ,Patient characteristics ,medicine.disease ,Malignancy ,Metastasis ,Surgery ,medicine.anatomical_structure ,Ewing family of tumors ,Internal medicine ,medicine ,In patient ,Bone marrow ,Progression-free survival ,business - Abstract
Introduction: Ewing Family of Tumors (EFT) is a high grade embryonic malignancy, common in children andyoung adults (CYAs). The prognostic factors include initial tumor volume, site, presence of metastasis, and theEWS/FLI 1 mutation. Low volume disease is known to result in higher response rates and longer survival times.Methods: Twelve patients with non-metastatic EFT were managed with the intensive Ewing Family Tumor(EFT 2001) protocol. The patient characteristics studied were - age, sex, site, number of sites involved,bone marrow involvement and size (volume). Tumor volume was measured in non-metastatic tumors by tridimensionalvolume in MRI scans.Results: Progression free survival (PFS) of patients with initial tumor volume ????100 cc ranged from 30 monthsto 74 months (mean 56.4 months) while that for > 100 cc ranged from 19 months to 79 months (mean 40months) (p value: 0.701). However there was trend towards better survival in patients with initial tumor volume????100 cc.Conclusions: There is a trend towards better PFS in patients with smaller tumor volume at presentation. Thusinitial tumor volume is a prognostic factor in Ewing family of tumors.doi:http://dx.doi.org/10.3126/mjsbh.v12i2.12925
- Published
- 2015
48. Do Long Term Survivors of Ewing Family of Tumors Experience Low Bone Mineral Density and Increased Fracture Risk?
- Author
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Gerhard M. Hobusch, Gerold Holzer, Christoph Krall, and Iris M. Noebauer-Huhmann
- Subjects
Adult ,Male ,Oncology ,medicine.medical_specialty ,Adolescent ,medicine.medical_treatment ,Osteoporosis ,Neuroectodermal Tumors ,Comorbidity ,Sarcoma, Ewing ,Fractures, Bone ,Young Adult ,Absorptiometry, Photon ,Clinical Research ,Ewing family of tumors ,Internal medicine ,Prevalence ,medicine ,Humans ,Orthopedics and Sports Medicine ,Survivors ,Vitamin D ,Child ,Survival rate ,Bone mineral ,Chemotherapy ,Evidence-Based Medicine ,business.industry ,General Medicine ,Middle Aged ,medicine.disease ,Primary tumor ,Surgery ,Causality ,Survival Rate ,Bone Diseases, Metabolic ,Cross-Sectional Studies ,Child, Preschool ,Population Surveillance ,Orthopedic surgery ,Linear Models ,Female ,Sarcoma ,business ,Follow-Up Studies - Abstract
Multimodal treatment regimens for Ewing's sarcoma have led to survival rates approaching 70% of patients with no metastases at diagnosis. However, these treatments have long-term side effects. Low bone mineral density (BMD) and risk of fractures can occur owing in part to chemotherapy and limited mobility from local control of the primary tumor.We performed this study to answer the following questions: (1) Do long-term survivors of the Ewing family of tumors sustain low BMD? (2) Which factors are associated with BMD in these patients? (3) Do they experience fractures? (4) Are BMD and fractures associated with each other?We queried our institutional registry to identify all known survivors of Ewing tumors who were treated before 2005. Of 100 such patients, 67 (67%) responded to a postal survey to participate in this study, and an additional 11 (11%) patients were excluded according to prespecified criteria. In the remaining 56 long-term survivors (27 females, 29 males; mean±SD age at followup, 32±10 years; mean followup, 15±7 years), BMD was measured by dual-energy x-ray absorptiometry and history of fractures was assessed using a questionnaire. Associations were tested using univariate and multivariate models by stepwise variable selection procedure, including Bonferroni correction.Thirty-one of 56 (56%) patients had a pathologic BMD. Seven (13%) had osteoporosis and 24 (43%) had osteopenia. Factors related to low BMD after Bonferroni correction were the length of time between surgery and followup and the BMI at followup. Twenty-one patients reported 29 fractures. With the numbers available, BMD levels were not associated with fractures.We could not confirm some potentially important predictors for fractures to be associated with clinical events of interest. However, the data are valuable as hypothesis-generating pilot data for future, multicenter prospective studies. If BMD changes cannot explain the propensity of fractures, there may be other bone characteristics like microarchitectural changes of bone to more accurately explain the effect.Level IV, prognostic study. See the Instructions for Authors for a complete description of levels of evidence.
- Published
- 2014
49. Adamantinoma-Like Ewing Sarcoma Mimicking Basal Cell Adenocarcinoma of the Parotid Gland: A Case Report and Review of the Literature
- Author
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Martha R. Clarke, Lei Zhang, Cristina R. Antonescu, Raja R. Seethala, and Cecilia Lezcano
- Subjects
Pathology ,medicine.medical_specialty ,Adenoid cystic carcinoma ,CD99 ,Adamantinoma ,Case Report ,Sarcoma, Ewing ,12E7 Antigen ,Adenocarcinoma ,Biology ,Pathology and Forensic Medicine ,Diagnosis, Differential ,Antigens, CD ,Ewing family of tumors ,Biomarkers, Tumor ,medicine ,Humans ,Parotid Gland ,Myoepithelial cell ,Middle Aged ,medicine.disease ,Parotid Neoplasms ,Parotid gland ,Treatment Outcome ,medicine.anatomical_structure ,Oncology ,Otorhinolaryngology ,Female ,Keratin-3 ,Sarcoma ,Tomography, X-Ray Computed ,Cell Adhesion Molecules - Abstract
Adamantinoma-like Ewing sarcoma (AES) is a rare variant of the Ewing family of tumors that resembles classic adamantinoma of bone. AES shows epithelial differentiation and a more complex immunohistochemical expression profile with keratin and basal marker immunoreactivity and can resemble a variety of carcinomas. We report an unusual case of an AES of the parotid gland that mimicked a basal cell adenocarcinoma. Like basal cell adenocarcinoma, this AES showed a nested basaloid proliferation with peripheral palisading in tumor nests with ‘basaloid’ epithelial differentiation as highlighted by cytokeratin AE1/3 and p40 positivity. However, unlike most basal cell adenocarcinomas, this tumor demonstrated high grade morphology, showed no true ductal or myoepithelial component, and also showed a tendency towards neuroectodermal phenotype with focal rosette formation, CD99 and weak synaptophysin immunoreactivity. EWSR1 and FLI1 fluorescence in situ hybridization confirmed the presence of a translocation supporting the diagnosis of AES. This is the first case of AES presenting as a primary parotid mass highlighting the potential to be mistaken for primary salivary gland carcinomas, which in addition to basal cell adenocarcinoma include other basaloid tumors such as adenoid cystic carcinoma.
- Published
- 2014
50. Ewing'S Sarcoma/PNET of the Labia Majora an Unusual Presentation in 2 Year Old Girl
- Author
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Irena M, Suhih Dj, Majic, and Bajmak A
- Subjects
medicine.medical_specialty ,business.industry ,media_common.quotation_subject ,Labia ,Ewing's sarcoma ,Labia majora ,medicine.disease ,Surgery ,Vulva ,Inguinal hernia ,medicine.anatomical_structure ,Ewing family of tumors ,medicine ,Girl ,Differential diagnosis ,business ,media_common - Abstract
ES/PNET is a high-grade malignant neoplasm that often develops in the skeletal system. Primary extra skeletal ES/PNET is uncommon condition that rarely affects the female genital tract. Reports on the Ewing family of tumors involving the vulva/labia majora are extremely rare in the relevant literature. A 2 year old girl presented to the clinic with 6 months history of a right vulvar mass. She was admitted to our hospital for the vulvar mass, originally thought to be an inguinal hernia. The mass was excised under general anesthesia. It was subsequently found to consist of small round cells positive for anti-CD99 antibody, thus suggesting a diagnosis of ES/PNET. Re-resection was performed five months later to obtain negative microscopic margins. Our findings underline the crucial role of molecular biology techniques in the differential diagnosis of small round cell tumors in this unusual location. In summary, we describe a rare case of extra skeletal ES/PNET with distinct rosette-like structures in a 2 year old girl with a good prognosis.
- Published
- 2016
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