Primary subcutaneous extra skeletal Ewing’s sarcoma: a case report on a very rare clinical entity
DOI:
https://doi.org/10.18203/2349-2902.isj20213635Keywords:
Ewing’s sarcoma, Extra skeletal, ImmunohistochemistryAbstract
Primary extra skeletal Ewing’s sarcoma is a very rare clinical entity. Patients are normally females in the second decade of their life. Clinically it normally presents as a fleshy mass which is mobile, not attached to the underlying structures. Diagnosis is difficult, final diagnosis being made by histopatholoical, immunohistochemistry and cytoskeletal studies combined. Ewing’s sarcoma is a tumour which normally affects the bones, and to a much lesser extent (10%) the soft tissues. Primary extra skeletal Ewing’s sarcoma affecting the back (as in the case we are discussing) is an extremely rare clinical entity with lesser than 50 cases reported worldwide. The entity being so rare doesn’t have any much literature for review.
References
Yagnik VD, Dawka S. Extra skeletal Ewing’s sarcoma/peripheral primitive neuroectodermal tumor of the small bowel presenting with gastrointestinal perforation. Clin Exp Gastroenterol. 2019;12:279-85.
Sumegi J, Nishio J, Nelson M, Frayer RW, Perry D, Bridge JA. A novel t (4; 22) (q31; q12) produces an EWSR1-SMARCA5 fusion in extra skeletal Ewing sarcoma/primitive neuroectodermal tumor. Mod Pathol. 2011;24(3):333-42.
Hübner G. Zur Feinstruktur von formalinfixiertem Biopsie- und Autopsiematerial nach Paraffineinbettung. Virchows Arch Abt A Path Anat. 1970;351:155-67.
Dangoor A, Seddon B, Gerrand C. UK guidelines for the management of soft tissue sarcomas. Clin Sarcoma Res 6, 20 (2016). https://doi.org/10.1186/s13569-016-0060-4.
Javery O, Krajewski K, O’Regan K. A to Z of Extra skeletal Ewing Sarcoma Family of Tumours in Adults: Imaging Features of Primary Disease, Metastatic Patterns, and Treatment Responses. AJR. 2011;197:1015-22.
Ahmad R, Mayol BR, Davis M, Rougraff BT. Extra skeletal Ewing's sarcoma. Cancer. 1999;85(3):725-31.
Srivastava S, Arora J, Parakh A, Goel RK. Primary extra skeletal Ewing's sarcoma/primitive neuroectodermal tumor of breast. Indian J Radiol Imaging. 2016;26(2):226-30.
Angervall L, Enzinger FM, Angervall L, Enzinger FM. Extraskeletal neoplasm resembling Ewing’s sarcoma. Cancer. 1975;36:240-51.
Guiter G, Gamboni M, Zakowsk M. The Cytology of Extra skeletal Ewing Sarcoma. Am Cancer Society. 1999;87:141-8.
Xie CF, Liu MZ, Xi M. Extraskeletal Ewing's sarcoma: a report of 18 cases and literature review. Chin J Cancer. 2010;29(4):420-4.
Hesla,AC.; Papakonstantinou, A.; Tsagkozis, P. Current Status of Management and Outcome for Patients with Ewing Sarcoma. Cancers 2021, 13, 1202. https://doi.org/10.3390/ cancers13061202.
Grier H, Krailo M, Tarbell N. Addition of Ifosfamide and Etoposide to Standard Chemotherapy for Ewing’s Sarcoma and Primitive Neuroectodermal Tumor of Bone. N Eng J Med. 2013;348:694-701.
Dunst J and Schuck A: Role of radiotherapy in Ewing tumors. Pediatr Blood Cancer. 2004;42:465-70.
