Extraskeletal Ewing's Sarcoma Family of Tumours in Adults: Analysis of 57 Patients from a Single Institution

Weshi, Amr El; Allam, Ayman; Ajarim, Dahish; Dayel, Fouad Al; Pant, Rajive; Bazarbashi, Shouki; Memon, Muhammad Ali

doi:10.1016/j.clon.2010.02.010

Cited by 97 publications

(135 citation statements)

References 24 publications

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“…Oko 80% ESFT se javlja u toku prve dvije dekade života i to najčešće u skeletu (80% slučajeva). Kod pacijenata starijih od 18 godina česta je ekstraskeletalna forma oboljenja [2][3][4][5][6]. Ek-straskeletalni Ewing-ov sarkom (EES) je veoma rijedak tumor.…”

Section: Uvodunclassified

“…Pacijenti se najčešće žale na nejasnu bol, kao što je bio slučaj i kod našeg bolesnika, i palpabilnu, brzo rastuću masu. S obzirom da se radi o rijetkom tumoru kod odraslih, dijagnoza i tretman pacijenata sa ovim oboljenjem predstavlja veliki izazov [1,5,13,14]. Pacijenti sa metastatskom bolešću mogu imati povišenu temperaturu, malaksalost, biohumoralne znakove inflamacije te povišen LDH [1][2][3][4][5]15].…”

Section: Diskusijaunclassified

“…S obzirom da se radi o rijetkom tumoru kod odraslih, dijagnoza i tretman pacijenata sa ovim oboljenjem predstavlja veliki izazov [1,5,13,14]. Pacijenti sa metastatskom bolešću mogu imati povišenu temperaturu, malaksalost, biohumoralne znakove inflamacije te povišen LDH [1][2][3][4][5]15]. Masivan primarni tumor prilikom otkrivanja najčešće korelira sa visokom stopom mortaliteta [2][3][4][5].…”

Section: Diskusijaunclassified

“…Pacijenti sa metastatskom bolešću mogu imati povišenu temperaturu, malaksalost, biohumoralne znakove inflamacije te povišen LDH [1][2][3][4][5]15]. Masivan primarni tumor prilikom otkrivanja najčešće korelira sa visokom stopom mortaliteta [2][3][4][5]. Uprkos masivnom primarnom tumoru, regionalne i udaljene metastaze su neuobičajene.…”

Section: Diskusijaunclassified

“…Oko 20% pacijenata razvije metastaze u mozgu, kičmenoj moždini i peritoneumu. Prisustvo metastaza smanjuje petogodišnju stopu preživljavanja sa 35-71% na svega 0-34% [3,5,16]. Neoadjuvantna hemoterapija je standard prije definitivne primjene radioterapije ili hirurške resekcije za lokalizovane forme ovog oboljenja [1,6,8].…”

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Primarni ekstraskeletalni Ewing-ov sarkom torakalnog zida – prikaz bolesnika

Stajčić¹,

Vujnović²,

Radosavljević³

et al. 2014

Biomedicinska istraž.

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Kratak sadržajUvod. Ektraskeletalni Ewing-ov sarkom (EES) je neuroektodermalni tumor koštanog porijekla, koji se najčešće javlja u drugoj deceniji života. EES je izuzetno rijedak tumor. Cilj rada je prikazati RTG i CT nalaze kod rijetkog oblika torakalne lokalizacije EES kao što je slučaj kod našeg pacijenta.Prikaz bolesnika. U radu predstavljamo EES porijekla lijeve visceralne pleure, koji je dijagnostikovan kod dvadesetjednogodišnjeg muškarca, sa neodređenim bolom u ramenu. Inicijalno je načinjena radiografija grudnog koša, na kojoj je uočena pleuralna efuzija i jasno ograničena mekotkivna masa duž lijevog lateralnog torakalnog zida. Neposredno urađena je kompjuterizovana tomografija grudnog koša, koja jasnije prikazuje difuznu distribuciju multiplih nodularnih mekotkivnih zadebljanja pleure lijevog hemitoraksa. Histološki je dokazan EES visceralne pleure.Zaključak. EES -primitivni neuroektodermalni tumor je rijedak tumor koji treba razlikovati od drugih tumora malih okruglih ćelija na osnovu njegove morfologije i laboratorijskih nalaza. Iako je ekstremno rijedak , EES treba da bude uzet u obzir u diferencijalnoj dijagnozi primarnih tumora zida grudnog koša.Ključne riječi: ektraskeletalni Ewing-ov sarkom, tumori torakalnog zida

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Section: Uvodunclassified

Section: Diskusijaunclassified

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Primarni ekstraskeletalni Ewing-ov sarkom torakalnog zida – prikaz bolesnika

Stajčić¹,

Vujnović²,

Radosavljević³

et al. 2014

Biomedicinska istraž.

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Primary Ewing sarcoma of the liver: Diagnosis, management, and prognosis: A case report and literature review

Hajri

Ismail

Zenaidi

et al. 2022

Clinical Case Reports

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Ewing's sarcoma family of tumors (ESFT) is a rare entity of mesenchymal tumors deriving from neural crest tissue with a variable degree of neuroectodermal differentiation and sharing common morphological and cytogenetic aberrations. 1 It includes extraosseous Ewing sarcoma (ES), primitive neuroectodermal tumor (PNET), Askin tumor, and atypical ES. PNETs were recognized for the first time by Arthur Purdy Stout in 1918 2 and constitutes approximately 1% of all sarcomas. ES was first described by James Ewing in 1921. 3 These tumors commonly occur in the young population as the majority of patients are younger than 30 years of age. In adults, ESFT arises in more than 50% of cases in soft tissues (trunk, retroperitoneum, intra-abdominal tissues, and viscera).The primary involvement of the liver is extremely rare, and only 11 cases have been reported. [4][5][6][7][8][9][10][11][12][13][14] These tumors are aggressive, with a high tendency to relapse and metastasize especially in the lungs, bone marrow, brain, and lymph nodes. 15 The present work aims to report a new case on primitive hepatic ES and review all cases of primitive hepatic ES/PNET reported in the literature and describe clinical, radiological, histological, cytogenetical, therapeutic, and prognosis features of this singular tumor site in the different cases. | CASE REPORTA 26-year-old man presented with paroxysmal right upper quadrant pain and progressive distension of his upper

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Clinical features and outcomes in patients with extraskeletal ewing sarcoma

Applebaum

Worch

Matthay

et al. 2011

Cancer

202

186

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BACKGROUND: Ewing sarcoma can arise in either bone or soft tissue. The purpose of this study was to investigate whether patient characteristics, treatment strategies, and outcomes differ between skeletal Ewing sarcoma and extraskeletal Ewing sarcoma (EES). METHODS: Patients <40 years of age with Ewing sarcoma or peripheral primitive neuroectodermal tumor reported to the United States Surveillance, Epidemiology, and End Results Program database from 1973 to 2007 were evaluated based on skeletal (n ¼ 1519) versus extraskeletal (n ¼ 683) site of origin. Patient characteristics were compared using Fisher exact tests. Overall survival was estimated via the Kaplan-Meier method and compared using log-rank tests and Cox proportional hazard models. RESULTS: Patients with EES had a higher mean age (19.5 vs 16.3 years; P < .001) and were less likely to be male (53.4% vs 63.3%; P < .001) or white (84.8% vs 92.5%; P < .001) compared with patients with skeletal tumors. Extraskeletal tumors were more likely to arise in axial locations (72.9% vs 54.2%; P ¼ .001) but were less likely to arise specifically in the pelvis (19.8% vs 26.6%; P < .001). Metastatic status or tumor size did not differ by group. Five-year overall survival was superior for localized EES compared with localized skeletal tumors (69.7% vs 62.6%; P ¼ .02). The hazard ratio for death in patients with localized skeletal tumors compared with localized EES was 2.36 (95% confidence interval, 1.61-3.44) beyond 24 months from initial diagnosis. CONCLUSIONS: Patient characteristics and outcomes differ among patients with EES compared with patients with skeletal Ewing sarcoma. These findings may have important implications for patient care. Cancer 2011;117:3027-

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Extraskeletal Ewing's Sarcoma Family of Tumours in Adults: Analysis of 57 Patients from a Single Institution

Cited by 97 publications

References 24 publications

Primarni ekstraskeletalni Ewing-ov sarkom torakalnog zida – prikaz bolesnika

Primarni ekstraskeletalni Ewing-ov sarkom torakalnog zida – prikaz bolesnika

Primary Ewing sarcoma of the liver: Diagnosis, management, and prognosis: A case report and literature review

Clinical features and outcomes in patients with extraskeletal ewing sarcoma

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