medigraphic.com
Colegio de Medicos y Cirujanos República de Costa Rica

2015, Number 617

<< Back Next >>

Rev Med Cos Cen 2015; 72 (617)

Sarcoma de Ewing

Villalta FJC

Language: Spanish
References: 33
Page: 695-704
PDF size: 413.25 Kb.


ABSTRACT

Ewing´s sarcoma belongs to a group of neoplastic diseases known as the Ewing family of tumors, which also includes their extraosseous variant, the neuroectodermal tumor, Askin´s tumor and the atypical Ewing tumors. Its origin has been debated for a long time and has even been appointed to a possible endothelial and vascular origin as the one conferred by James Ewing in 1921. The truth of all this is that there´s sufficient scientific evidence from immunohistochemical, cytogenetic and molecular genetic studies supporting mesenchymal cell origin as a common progenitor for all the tumors of Ewing´s family sarcomas. Ewing’s sarcoma is genetically characterized in most cases with a cytogenetic abnormality, which is a chromosomal disorder, associated with a translocation of chromosome 22 t (11:22) (q24: q12). The Ewing family of tumors usually originates in the long bones of the extremities, mainly in the femur, but also in the tibia, fibula, humerus and the bones of the pelvis. The bones of the spine, hands and feet are usually less affected. It is reported mainly in males with a male / female ratio of 1: 6. It has a prevalence of 1-5 cases per 1,000,000 inhabitants giving 70% in children under 20 years (range 11-12 years in girls between 15-16 years in children.


REFERENCES

  1. Applebaum MA, Worch J, Matthay KK, et al. Clinical features and outcomes in patients with extraskeletal Ewing sarcoma. Cancer 2011; 117:3027.

  2. Askin FB, Rosai J, Sibley RK, et al. Malignant small cell tumor of the thoracopulmonary region in childhood: a distinctive clinicopathologic entity of uncertain histogenesis. Cancer 1979; 43:2438

  3. Bacci G, Capanna R, Orlandi M, et al. Prognostic significance of serum lactic acid dehydrogenase in Ewing’s tumor of bone. Ric Clin Lab 1985; 15:89.

  4. Campanacci M. Bone and Soft Tissue Tumors, 2nd, Springer Verlag, New York 1999. p.653

  5. Cangir A, Vietti TJ, Gehan EA, et al. Ewing’s sarcoma metastatic at diagnosis. Results and comparisons of two intergroup Ewing’s sarcoma studies. Cancer 1990; 66:887

  6. Casciato Dennis, Lowita Barry. Oncología Clínica 4ta edición. Editorial Marban 2001. Pag: 370-371

  7. Cotterill SJ, Ahrens S, Paulussen M, et al. Prognostic factors in Ewing’s tumor of bone: analysis of 975 patients from the European Intergroup Cooperative Ewing’s Sarcoma Study Group. J Clin Oncol 2000; 18:3108

  8. Ferrari S, Bertoni F, Mercuri M, et al. Ewing’s sarcoma of bone: relation between clinical characteristics and staging. Oncol Rep 2001; 8:553

  9. Ginsberg JP, Woo SY, Hicks MJ, Horowitz ME. Ewing’s sarcoma family of tumors: Ewing’s sarcoma of bone and soft tissue and the peripheral primitive neuroectodermal tumors. In: Priniciples and Practice of Pediatric Oncology, 4th, Pizzo PA, Poplack DG (Eds), Lippincott, Williams and Wilkins, Philadelphia 2002

  10. Glaubiger DL, Makuch R, Schwarz J, et al. Determination of prognostic factors and their influence on therapeutic results in patients with Ewing’s sarcoma. Cancer 1980; 45:2213

  11. Granowetter L, Womer R, Devidas M, et al. Dose-intensified compared with standard chemotherapy for nonmetastatic Ewing sarcoma family of tumors: a Children’s Oncology Group Study. J Clin Oncol 2009; 27:2536.

  12. Grier H, Krailo M, Link M, et al. Improved outcome in non-metastatic Ewing’s sarcoma (EWS) and PNET of bone with the addition of ifosfamide (I) and etoposide (E) to vincristine (V), Adriamycin (Ad), cyclophosphamide (C) and actinomycin (A): A Children’s Cancer Group (CCG) and Pediatric Oncology Group (POG) report (abstract). Proc Am Soc Clin Oncol 1994; 13:421.

  13. Hartman KR, Triche TJ, Kinsella TJ, Miser JS. Prognostic value of histopathology in Ewing´s sarcoma: longterm follow up of distal extremity primary tumors. Cancer 1991; 67: 163-171

  14. Jedlicka P. Ewing Sarcoma, an enigmatic malignancy of likely progenitor cell origin, driven by transcription factor oncogenic fusions. Int J Clin Exp Pathol 2010; 3:338

  15. Jürgens H, Exner U, Gadner H, et al. Multidisciplinary treatment of primary Ewing’s sarcoma of bone. A 6-year experience of a European Cooperative Trial. Cancer 1988; 61:23.

  16. Madero López Luis; Muñoz Villa Arturo. Hematología y Oncología Pediátricas. Editorial Ergon 1997. Pag: 557-562

  17. Mankin HJ. Ewing sarcoma. Curr Opinion Ortho 2000; 11:479.

  18. Mendenhall CM, Marcus RB Jr, Enneking WF, et al. The prognostic significance of soft tissue extension in Ewing’s sarcoma. Cancer 1983; 51:913

  19. Miser JS, Krailo MD, Tarbell NJ, et al. Treatment of metastatic Ewing’s sarcoma or primitive neuroectodermal tumor of bone: evaluation of combination ifosfamide and etoposide--a Children’s Cancer Group and Pediatric Oncology Group study. J Clin Oncol 2004; 22:2873

  20. Nesbit ME Jr, Gehan EA, Burgert EO Jr, et al. Multimodal therapy for the management of primary, nonmetastatic Ewing’s sarcoma of bone: a long-term follow-up of the First Intergroup study. J Clin Oncol 1990; 8:1664

  21. Oberlin O, Bayle C, Hartmann O, et al. Incidence of bone marrow involvement in Ewing’s sarcoma: value of extensive investigation of the bone marrow. Med Pediatr Oncol 1995; 24:343

  22. Panicek DM, Gatsonis C, Rosenthal DI, et al. CT and MR imaging in the local staging of primary malignant musculoskeletal neoplasms: Report of the Radiology Diagnostic Oncology Group. Radiology 1997; 202:237

  23. Parasuraman S, Langston J, Rao BN, et al. Brain metastases in pediatric Ewing sarcoma and rhabdomyosarcoma: the St. Jude Children’s Research Hospital experience. J Pediatr Hematol Oncol 1999; 21:370

  24. Pradhan A, Grimer RJ, Spooner D, et al. Oncological outcomes of patients with Ewing’s sarcoma: is there a difference between skeletal and extra-skeletal Ewing’s sarcoma? J Bone Joint Surg Br 2011; 93:531.

  25. Raney RB, Asmar L, Newton WA Jr, et al. Ewing’s sarcoma of soft tissues in childhood: a report from the Intergroup Rhabdomyosarcoma Study, 1972 to 1991. J Clin Oncol 1997; 15:574

  26. Rodriguez-Galindo Carlos, Navid Faruba, Khoury Joseph, Krasin Mathew. Pediatric Oncology: Pediatric Bone and soft Tissue Sarcomas: Ewing Sarcoma of tumors. Chapter 9 2006: 181-217

  27. Rud NP, Reiman HM, Pritchard DJ, et al. Extraosseous Ewing’s sarcoma. A study of 42 cases. Cancer 1989; 64:1548

  28. Shmooker Barry, Bickels Jacob, Jelinek James, Sugarbaker Paul, Malawer Martin. Bone and soft tissue sarcomas: Epidemiology, Radiology, Pathology and Fundamentals of surgical treatment. Chapter 1 2001. Pag: 1-37

  29. Smith MAS, Gurney JG, Ries LA. Cancer in adolescents 15 to 19 years old. In: Cancer incidence and Survival Among Children and Adolescents: United States SEER Program 1975-1995, Pub #99-4649, Smith MAS, Gurney JG, Ries LA, et al (Eds), National Cancer Institute, Bethesda, MD 1999

  30. Sneppen O, Hansen LM. Presenting symptoms and treatment delay in osteosarcoma and Ewing’s sarcoma. Acta Radiol Oncol 1984; 23:159.

  31. Syed Asad Ali et al. Case Report: Extra osseous primary Ewing´s Sarcoma. J Ayub Med Coll Abbottabad 2010;22(3)

  32. Widhe B, Widhe T. Initial symptoms and clinical features in osteosarcoma and Ewing sarcoma. J Bone Joint Surg Am 2000; 82:667.

  33. Wilkins RM, Pritchard DJ, Burgert EO Jr, Unni KK. Ewing’s sarcoma of bone. Experience with 140 patients. Cancer 1986; 58:2551.




2020     |     www.medigraphic.com