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Childhood extraosseous osteosarcoma

MedGen UID:
232060
•Concept ID:
C1332968
•
Neoplastic Process
Synonyms: Childhood Extraosseous Osteosarcoma; childhood extraosseous osteosarcoma; Childhood Extraskeletal Osteosarcoma; childhood extraskeletal osteosarcoma; extraosseous osteosarcoma of childhood; paediatric extraosseous osteosarcoma; paediatric extraskeletal osteosarcoma; Pediatric Extraosseous Osteosarcoma; pediatric extraosseous osteosarcoma; Pediatric Extraskeletal Osteosarcoma; pediatric extraskeletal osteosarcoma
 
Monarch Initiative: MONDO:0004176

Definition

An osteosarcoma that arises from the soft tissue and occurs during childhood. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVChildhood extraosseous osteosarcoma

Recent clinical studies

Etiology

Ewing Sarcoma of the Cervical Epidural Space Presenting with Tetraplegia: Case Report and Review of Literature.
Kutty RK, Peethambaran A, Sunilkumar BS, Balachandran Nair KG, Korde P, Jain SK
World Neurosurg 2017 Nov;107:1046.e9-1046.e15. Epub 2017 Aug 7 doi: 10.1016/j.wneu.2017.07.182. PMID: 28797979
Comparison of clinical features and outcomes in patients with extraskeletal versus skeletal localized Ewing sarcoma: A report from the Children's Oncology Group.
Cash T, McIlvaine E, Krailo MD, Lessnick SL, Lawlor ER, Laack N, Sorger J, Marina N, Grier HE, Granowetter L, Womer RB, DuBois SG
Pediatr Blood Cancer 2016 Oct;63(10):1771-9. Epub 2016 Jun 14 doi: 10.1002/pbc.26096. PMID: 27297500Free PMC Article
Cooperative trial CWS-91 for localized soft tissue sarcoma in children, adolescents, and young adults.
Dantonello TM, Int-Veen C, Harms D, Leuschner I, Schmidt BF, Herbst M, Juergens H, Scheel-Walter HG, Bielack SS, Klingebiel T, Dickerhoff R, Kirsch S, Brecht I, Schmelzle R, Greulich M, Gadner H, Greiner J, Marky I, Treuner J, Koscielniak E
J Clin Oncol 2009 Mar 20;27(9):1446-55. Epub 2009 Feb 17 doi: 10.1200/JCO.2007.15.0466. PMID: 19224858
Ewing's sarcoma family of tumors: an overview from diagnosis to survivorship.
Lahl M, Fisher VL, Laschinger K
Clin J Oncol Nurs 2008 Feb;12(1):89-97. doi: 10.1188/08.CJON.89-97. PMID: 18258578
Histopathology of childhood sarcomas, Intergroup Rhabdomyosarcoma Studies I and II: clinicopathologic correlation.
Newton WA Jr, Soule EH, Hamoudi AB, Reiman HM, Shimada H, Beltangady M, Maurer H
J Clin Oncol 1988 Jan;6(1):67-75. doi: 10.1200/JCO.1988.6.1.67. PMID: 3275751

Diagnosis

Primary spinal multifocal intradural-extramedullary Ewing sarcoma in children: presentation of a case and review of the literature.
Müngen E, Kurucu N, Kutluk T, Oğuz KK, Söylemezoğlu F, Yalçın B
Turk J Pediatr 2021;63(6):1084-1090. doi: 10.24953/turkjped.2021.06.018. PMID: 35023660
Ewing Sarcoma of the Cervical Epidural Space Presenting with Tetraplegia: Case Report and Review of Literature.
Kutty RK, Peethambaran A, Sunilkumar BS, Balachandran Nair KG, Korde P, Jain SK
World Neurosurg 2017 Nov;107:1046.e9-1046.e15. Epub 2017 Aug 7 doi: 10.1016/j.wneu.2017.07.182. PMID: 28797979
Extraosseous Ewing sarcoma of the vagina: a rare entity.
Machado L, Al-Hamdani A, Sankhla DK, Al-Moundhri MS
Ann Saudi Med 2013 Mar-Apr;33(2):182-6. doi: 10.5144/0256-4947.2013.182. PMID: 23563009Free PMC Article
Ewing's sarcoma family of tumors: an overview from diagnosis to survivorship.
Lahl M, Fisher VL, Laschinger K
Clin J Oncol Nurs 2008 Feb;12(1):89-97. doi: 10.1188/08.CJON.89-97. PMID: 18258578
Primitive neuroectodermal tumor and Ewing's sarcoma.
Dehner LP
Am J Surg Pathol 1993 Jan;17(1):1-13. doi: 10.1097/00000478-199301000-00001. PMID: 8383465

Therapy

Primary spinal multifocal intradural-extramedullary Ewing sarcoma in children: presentation of a case and review of the literature.
Müngen E, Kurucu N, Kutluk T, Oğuz KK, Söylemezoğlu F, Yalçın B
Turk J Pediatr 2021;63(6):1084-1090. doi: 10.24953/turkjped.2021.06.018. PMID: 35023660
Cooperative trial CWS-91 for localized soft tissue sarcoma in children, adolescents, and young adults.
Dantonello TM, Int-Veen C, Harms D, Leuschner I, Schmidt BF, Herbst M, Juergens H, Scheel-Walter HG, Bielack SS, Klingebiel T, Dickerhoff R, Kirsch S, Brecht I, Schmelzle R, Greulich M, Gadner H, Greiner J, Marky I, Treuner J, Koscielniak E
J Clin Oncol 2009 Mar 20;27(9):1446-55. Epub 2009 Feb 17 doi: 10.1200/JCO.2007.15.0466. PMID: 19224858
Ewing's sarcoma family of tumors: an overview from diagnosis to survivorship.
Lahl M, Fisher VL, Laschinger K
Clin J Oncol Nurs 2008 Feb;12(1):89-97. doi: 10.1188/08.CJON.89-97. PMID: 18258578
Ewing's sarcoma of soft tissues in childhood: a report from the Intergroup Rhabdomyosarcoma Study, 1972 to 1991.
Raney RB, Asmar L, Newton WA Jr, Bagwell C, Breneman JC, Crist W, Gehan EA, Webber B, Wharam M, Wiener ES, Anderson JR, Maurer HM
J Clin Oncol 1997 Feb;15(2):574-82. doi: 10.1200/JCO.1997.15.2.574. PMID: 9053479
Histopathology of childhood sarcomas, Intergroup Rhabdomyosarcoma Studies I and II: clinicopathologic correlation.
Newton WA Jr, Soule EH, Hamoudi AB, Reiman HM, Shimada H, Beltangady M, Maurer H
J Clin Oncol 1988 Jan;6(1):67-75. doi: 10.1200/JCO.1988.6.1.67. PMID: 3275751

Prognosis

Primary spinal multifocal intradural-extramedullary Ewing sarcoma in children: presentation of a case and review of the literature.
Müngen E, Kurucu N, Kutluk T, Oğuz KK, Söylemezoğlu F, Yalçın B
Turk J Pediatr 2021;63(6):1084-1090. doi: 10.24953/turkjped.2021.06.018. PMID: 35023660
Comparison of clinical features and outcomes in patients with extraskeletal versus skeletal localized Ewing sarcoma: A report from the Children's Oncology Group.
Cash T, McIlvaine E, Krailo MD, Lessnick SL, Lawlor ER, Laack N, Sorger J, Marina N, Grier HE, Granowetter L, Womer RB, DuBois SG
Pediatr Blood Cancer 2016 Oct;63(10):1771-9. Epub 2016 Jun 14 doi: 10.1002/pbc.26096. PMID: 27297500Free PMC Article
Extraosseous Ewing sarcoma of the vagina: a rare entity.
Machado L, Al-Hamdani A, Sankhla DK, Al-Moundhri MS
Ann Saudi Med 2013 Mar-Apr;33(2):182-6. doi: 10.5144/0256-4947.2013.182. PMID: 23563009Free PMC Article
Ewing's sarcoma family of tumors: an overview from diagnosis to survivorship.
Lahl M, Fisher VL, Laschinger K
Clin J Oncol Nurs 2008 Feb;12(1):89-97. doi: 10.1188/08.CJON.89-97. PMID: 18258578
Histopathology of childhood sarcomas, Intergroup Rhabdomyosarcoma Studies I and II: clinicopathologic correlation.
Newton WA Jr, Soule EH, Hamoudi AB, Reiman HM, Shimada H, Beltangady M, Maurer H
J Clin Oncol 1988 Jan;6(1):67-75. doi: 10.1200/JCO.1988.6.1.67. PMID: 3275751

Clinical prediction guides

A spontaneously ruptured primitive neuroectodermal tumor/extraosseous Ewing's sarcoma of the kidney with renal vein tumor thrombus.
Moustafellos P, Gourgiotis S, Athanasopoulos G, Karagianni E, Hadjiyannakis E
Int Urol Nephrol 2007;39(2):393-5. Epub 2007 Feb 20 doi: 10.1007/s11255-006-9073-x. PMID: 17310314
Adults with Ewing's sarcoma/primitive neuroectodermal tumor: adverse effect of older age and primary extraosseous disease on outcome.
Baldini EH, Demetri GD, Fletcher CD, Foran J, Marcus KC, Singer S
Ann Surg 1999 Jul;230(1):79-86. doi: 10.1097/00000658-199907000-00012. PMID: 10400040Free PMC Article
Histopathology of childhood sarcomas, Intergroup Rhabdomyosarcoma Studies I and II: clinicopathologic correlation.
Newton WA Jr, Soule EH, Hamoudi AB, Reiman HM, Shimada H, Beltangady M, Maurer H
J Clin Oncol 1988 Jan;6(1):67-75. doi: 10.1200/JCO.1988.6.1.67. PMID: 3275751

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