DOI QR코드

DOI QR Code

Extraosseous Ewing's Sarcoma Presented as a Rectal Subepithelial Tumor: Radiological and Pathological Features

  • Bae, Heejin (Department of Radiology, Yonsei University College of Medicine) ;
  • Chung, Taek (Department of Pathology, Yonsei University College of Medicine) ;
  • Park, Mi-Suk (Department of Radiology, Yonsei University College of Medicine) ;
  • Kim, Myeong-Jin (Department of Radiology, Yonsei University College of Medicine) ;
  • Lim, Joon Seok (Department of Radiology, Yonsei University College of Medicine) ;
  • Kim, Honsoul (Department of Radiology, Yonsei University College of Medicine)
  • 투고 : 2017.02.05
  • 심사 : 2017.03.20
  • 발행 : 2017.03.30

초록

Purpose: Extraosseous Ewing's sarcoma (EOE) of the rectum is extremely rare: only three cases have been reported in the literature and none of these reports described their imaging findings in detail. Herein, we describe the tumor imaging and pathological features in detail. Materials and Methods: We report a case of rectal EOE in a 72-year-old female who received local excision and was provisionally diagnosed with a rectal submucosal spindle cell tumor. We used immunohistochemistry, histopathology, and fluorescence in situ hybridization to characterize the tumor and provide a definitive diagnosis of EOE. Results: MRI revealed a well-demarcated submucosal tumor with heterogeneous enhancement and hemorrhagic foci in rectum. EOE was diagnosed by positive staining of tumor cells for CD99 and Fli-1 by immunohistochemistry and the presence of the EWSR1 gene translocation by fluorescence in situ hybridization. Although the patient underwent radiation treatment and surgery, the tumor recurred after 4 months as revealed by computed tomography and magnetic resonance imaging. Conclusion: Rectal EOE may present as a rectal submucosal tumor. The understanding of imaging and histological characteristics of this tumor are critical for accurate diagnosis and appropriate aggressive treatment.

키워드

참고문헌

  1. Fletcher CD, Bridge JA, Hogendoorn P, Mertens F. WHO classification of tumours of soft tissue and bone. 4th ed. Lyon, France: International Agency for Research on Cancer, 2013
  2. Vardy J, Joshua AM, Clarke SJ, Yarrow PM, Lin BP. Small blue cell tumors of the rectum. Case 1. Ewing's sarcoma of the rectum. J Clin Oncol 2005;23:910-912 https://doi.org/10.1200/JCO.2005.03.096
  3. Aboumarzouk OM, Coleman R, Goepel JR, Shorthouse AJ. PNET/Ewing's sarcoma of the rectum: a case report and review of the literature. BMJ Case Rep 2009;2009
  4. Javery O, Krajewski K, O'Regan K, et al. A to Z of extraskeletal Ewing sarcoma family of tumors in adults: imaging features of primary disease, metastatic patterns, and treatment responses. AJR Am J Roentgenol 2011;197:W1015-1022 https://doi.org/10.2214/AJR.11.6667
  5. Kim SK, Park YK. Ewing sarcoma: a chronicle of molecular pathogenesis. Hum Pathol 2016;55:91-100 https://doi.org/10.1016/j.humpath.2016.05.008
  6. Wei S, Siegal GP. Round cell tumors of bone: an update on recent molecular genetic advances. Adv Anat Pathol 2014;21:359-372 https://doi.org/10.1097/PAP.0000000000000036
  7. Murphey MD, Senchak LT, Mambalam PK, Logie CI, Klassen- Fischer MK, Kransdorf MJ. From the radiologic pathology archives: ewing sarcoma family of tumors: radiologicpathologic correlation. Radiographics 2013;33:803-831 https://doi.org/10.1148/rg.333135005
  8. Huh J, Kim KW, Park SJ, et al. Imaging features of primary tumors and metastatic patterns of the extraskeletal ewing sarcoma family of tumors in adults: a 17-year experience at a single institution. Korean J Radiol 2015;16:783-790 https://doi.org/10.3348/kjr.2015.16.4.783
  9. Kim H, Kim JH, Lim JS, et al. MRI findings of rectal submucosal tumors. Korean J Radiol 2011;12:487-498 https://doi.org/10.3348/kjr.2011.12.4.487
  10. Angervall L, Enzinger FM. Extraskeletal neoplasm resembling Ewing's sarcoma. Cancer 1975;36:240-251 https://doi.org/10.1002/1097-0142(197507)36:1<240::AID-CNCR2820360127>3.0.CO;2-H