Large extrapulmonary Ewing's Sarcoma a diagnostic challenge: case report
Sarcoma de Ewing extrapulmonar de gran tamaño, un reto diagnóstico. Reporte de caso
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Ewing's sarcoma is a type of primary bone tumor, typically found in long and flat bones. It is less frequently located in the thoracic wall. It is noteworthy that the occurrence of a significantly large tumor in the rib cage is uncommon. Additionally, it is important to highlight that the prognosis is inversely proportional to the size of the tumor. Clinical case: We present the case of an 11-year-old Afro-descendant patient with no pathological history, presenting with fever, pleuritic pain, and dyspnea. Imaging studies, including a biopsy, revealed a tumor in the right costal arch (13 x 11 cm). Biopsy confirms Ewing's sarcoma. Chemotherapy is initiated with significant improvement by week 6. Pediatric oncological surgery successfully resects the tumor (6x7 cm) in the seventh costal arch with no complications. The patient continues with antineoplastic management and shows clinical improvement by week 12. Conclussion: Emphasizing the importance of clinical-pathological correlation, immunohistochemical studies are crucial for a definitive diagnosis, considering the histological characteristics specific to this sarcoma. Few cases in the literature report initial tumor size and its reduction post-chemotherapeutic therapy, highlighting the favorable outcome observed in the patient by week 12 of chemotherapy.
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Ewing J. Diffuse endothelioma of bone. Proc NY Path Soc. 1921;21:17–24
Ries LAG, Smith MA, Gurney JG, Linet M, Tamra T, Young JL, et al, editors. Cancer incidence and survival among children and adolescents: United States SEER Program 1975-1995. National Cancer Institute. SEER Program. NIH Pub No 99-4649.Bethesda, MD, 1999.
Muñoz Villa A. Tumores óseos malignos. En: Madero López L, Muñoz Villa A, editores. Hematología y oncología pediátricas 1ª ed. Madrid: Ediciones Ergón; 1997. p. 557-72.
Casey D, et al. Irinotecan and temozolamide for Ewing sarcoma: The Memorial Sloan.
Kettering Experience. Pediatric Blood Cancer. Disponible en: https://doi.org/10.1002/pbc.22206
López Guerra JL, Márquez-Vega C, Ramírez-Villar GL, et al. Prognostic factors for overall survival in pediatric patients with Ewing sarcoma of bone treated according to multidisciplinary protocol. Clin Transl Oncol. 2012 Apr;14(4):294-301 Disponible en: https://doi.org/10.1007/s12094-012-0798-y
Jagodzińska-Mucha P, Ługowska I, Świtaj T, et al. The clinical prognostic factors and treatment outcomes of adult patients with Ewing sarcoma. Int J Clin Oncol. 2020 Nov;25(11):2006-2014. Disponible en: https://doi.org/10.1007/s10147-020-01741-
Umeda K, Miyamura T, Yamada K, et al. Prognostic and therapeutic factors influencing the clinical outcome of metastatic Ewing sarcoma family of tumors: A retrospective report from the Japan Ewing Sarcoma Study Group. Pediatr Blood Cancer. 2021 Mar;68(3):e28844. Disponible en: https://doi.org/10.1002/pbc.28844
Karski EE, McIlvaine E, Segal MR, et al. Identification of Discrete Prognostic Groups in Ewing Sarcoma. Pediatr Blood Cancer. 2016;63(1):47-53. Disponible en: https://doi.org/10.1002/pbc.25709
Tortajada JF, García JAO, Claudio Morales L, Castell JG, Perales VM, Casas LM. Factores de riesgo para los tumores óseos malignos pediátricos. Anales de Pediatría. 2005;63:537-547. Disponible en: https://doi.org/10.1016/S1695-4033(05)70254-X
Yaw KM. Pediatric bone tumors. Semin Surg Oncol. 1999;16:173-83. Disponible en: https://doi.org/10.1002/(sici)1098-2388(199903)16:2<173::aid-ssu8>3.0.co;2-g
Delattre O, Zucman J, Plougastel B, et al. Gene fusion with an ETS DNA-binding domain caused by chromosome translocation in human tumors. Nature. 1992;359(6391):162-165. Disponible en: https://doi.org/10.1038/359162a0
Ewing J. Diffuse endothelioma of bone. Proc NY Pathol Soc. 1921;21:17-24
Paz-Gómez FJ. Tumor de células pequeñas redondas y azules: abordaje diagnóstico. Med Sur. 2004;11(1):13-19.
Mai Lan H, Hidalgo A, Gutiérrez F. Avances en Diagnósticos por Imágenes: Patología Pleural. Ediciones Journal 2009; pág 86.3.
Sánchez C. Tumores Torácicos. Tratado de Neumología Infantil. Cobos N, Pérez EG. Ergón 2003; pág. 912-9.
Scocci G, Deibert C, Böhm S, et al. Impact of surgery on survival in patients with Ewing sarcoma: meta-analysis. Eur J Cancer. 2007;43(13):1998-2005.
Urano F, Umezawa A, Yabe H, et al.: Molecular analysis of Ewing's sarcoma: another fusion gene, EWS-E1AF, available for diagnosis. Jpn J Cancer Res. 1998;89(7):703-11. Disponible en: https://doi.org/10.1111/j.1349-7006.1998.tb03274.x
Kitagawa R, Niikura Y, Becker A, Houghton PJ, Kitagawa K. EWSR1 maintains centromere identity. Cell Rep. 2023 Jun 27;42(6):112568. Disponible en: https://doi.org/10.1016/j.celrep.2023.112568
Patterson FR, Basra SK. Ewing’s sarcoma. In: Schwartz HS, editor. Orthopaedic knowledge update: musculoskeletal tumors 2. Rosemont: American Academy of Orthopaedic Surgeons; 2007. pp. 175-183.
Ulaner G, Hwang S, Landa J, Lefkowitz RA, Panicek DM. Tumores musculoesqueléticos y afecciones similares a tumores: errores comunes y evitables en las imágenes en pacientes con cáncer conocido o sospechado: Parte B: Imitaciones malignas de tumores benignos. En T. Ortopédico. 2013;37:877-882. doi: 10.1007/s00264-013-1824-6.
Sabate JM, Franquet T, Parellada JA, Monill JM, Oliva E. Malignant neuroectodermal tumors of the chest wall (Askin tumors): CT and MR findings in eight patients. Clinical Radiology 1994;49:634-8. Disponible en: https://doi.org/10.1016/S0009-9260(05)81882-3
Daniel A., Jr., Ullah E., Wahab S., Kumar V., Jr. Relevance of MRI in prediction of malignancy of musculoskeletal system-a prospective evaluation. BMC Musculoskelet. Disord. 2009;10:125. doi: 10.1186/1471-2474-10-125.
Wenaden A.E., Szyszko T.A., Saifuddin A. Imaging of periosteal reactions associated with focal lesions of bone. Clin. Radiol. 2005;60:439-456. doi: 10.1016/j.crad.2004.08.017.
Exner GU, Kurrer MO, Mamisch-Saupe N., Cannon SR Las tácticas y técnicas de la biopsia musculoesquelética. EFORT Abierto Rev. 2017;2:51-57. doi: 10.1302/2058-5241.2.160065.
Mitsuyoshi G., Naito N., Kawai A., Kunisada T., Yoshida A., Yanai H., Dendo S., Yoshino T., Kanazawa S., Ozaki T. Diagnóstico preciso de lesiones musculoesqueléticas mediante biopsia con aguja gruesa. J. Cirugía Oncol. 2006;94:21-27. doi: 10.1002/jso.20504.
Marcilla D., Machado I., Grunewald TGP, Llombart-Bosch A., de Alava E. Análisis (inmuno)histológico del sarcoma de Ewing. Métodos Mol. Biol. 2021;2226:49-64. doi: 10.1007/978-1-0716-1020-6_5.
Paz-Gómez FJ. Tumor de células pequeñas redondas y azules: abordaje diagnóstico. Med Sur. 2004;11(1):13-19.
Folpe AL, Goldblum JR, Rubin BP, Shehata BM, Liu W, Dei Tos AP, Weiss SW. Morphologic and immunophenotypic diversity in Ewing family tumors: a study of 66 genetically confirmed cases. Am J Surg Pathol. 2005;29(8):1025-1033. Disponible en: https://doi.org/10.1097/01.pas.0000167056.13614.62
Alava E, Kawai A, Healey JH, et al. EWS-FLI1 fusion transcript structure is an independent determinant of prognosis in Ewing's sarcoma. J Clin Oncol. 1998;16(4):1248-1255. Disponible en: https://doi.org/10.1200/JCO.1998.16.4.1248
Cripe TP. Sarcoma de Ewing: una ventana epónima a la historia. Sarcoma. 2011; 457532. Disponible en: https://doi.org/10.1155/2011/457532.
Ozaki, T. Diagnosis and treatment of Ewing sarcoma of the bone: A review article. Journal of Orthopaedic Science. 2015;20(2),250-263. Disponible en: https://doi.org/10.1007/s00776-014-0687-z
Shamberger RC, Grier HE Sarcoma de Ewing/tumor neuroectodérmico primitivo de la pared torácica. Semin Pediatr Cirugía. 2001;10:153-160. Disponible en: https://doi.org/10.1053/spsu.2001.24699.
Sabanathan S., Salama FD, Morgan WE, Harvey JA Tumores primarios de la pared torácica. Ana Torácico Cirugía. 1985;39:4-15. Disponible en: https://doi.org/10.1016/S0003-4975(10)62515-5.
Saenz NC, Hass DJ, Meyers P, Wollner N, Gollamudi S, Bains M, LaQuaglia MP. Sarcoma de Ewing pediátrico de la pared torácica. J Pediatr Cirugía. 2000;35: 550-555. Disponible en: https://doi.org/10.1053/jpsu.2000.0350550.
Hayes FA, Thompson EI, Meyer WH, Kun L, Parham D, Rao B, Kumar M, Hancock M, Parvey L, Magill L, et al. Terapia para el sarcoma óseo de Ewing localizado. J Clin Oncol. 1989;7:208–213. Disponible en: https://doi.org/10.1200/JCO.1989.7.2.208.
Hayes FA, Thompson EI, Meyer WH, Kun L, Parham D, Rao B, Kumar M, Hancock M, Parvey L, Magill L, et al. Terapia para el sarcoma óseo de Ewing localizado. J Clin Oncol. 1989;7:208–213. Disponible en: https://doi.org/10.1200/JCO.1989.7.2.208.
Collaud S, Stork T, Dirksen U, Pottgen C, Hegedus B, Schildhaus HU, Bauer S, Aigner C. Tratamiento quirúrgico para el sarcoma primario de la pared torácica: un estudio de una sola institución. J Cirugía Res. 2020;260:149-154. Disponible en: https://doi.org/10.1016/j.jss.2020.11.078.
DuBois SG, Krailo MD, Gebhardt MC, et al. Comparative evaluation of local control strategies in localized Ewing sarcoma of bone: a report from the Children's Oncology Group. Cancer. 2015;121(3):467-475. Disponible en: https://doi.org/10.1002/cncr.29065
