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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

  • Huh, Jimi (Department of Radiology, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Kim, Kyung Won (Department of Radiology, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Park, Seong Joon (Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Kim, Hyoung Jung (Department of Radiology, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Lee, Jong Seok (Department of Radiology, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Ha, Hyun Kwon (Department of Radiology, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Tirumani, Sree Harsha (Department of Imaging, Dana-Farber Cancer Institute, Brigham and Women's Hospital, Harvard Medical School) ;
  • Ramaiya, Nikhil H. (Department of Imaging, Dana-Farber Cancer Institute, Brigham and Women's Hospital, Harvard Medical School)
  • Received : 2015.01.03
  • Accepted : 2015.03.28
  • Published : 2015.08.01

Abstract

Objective: To comprehensively analyze the spectrum of imaging features of the primary tumors and metastatic patterns of the Extraskeletal Ewing sarcoma family of tumors (EES) in adults. Materials and Methods: We performed a computerized search of our hospital's data-warehouse from 1996 to 2013 using codes for Ewing sarcoma and primitive neuroectodermal tumors as well as the demographic code for ${\geq}18$ years of age. We selected subjects who were histologically confirmed to have Ewing sarcoma of extraskeletal origin. Imaging features of the primary tumor and metastatic disease were evaluated for lesion location, size, enhancement pattern, necrosis, margin, and invasion of adjacent organs. Results: Among the 70 patients (mean age, $35.8{\pm}15.6$ years; range, 18-67 years) included in our study, primary tumors of EES occurred in the soft tissue and extremities (n = 20), abdomen and pelvis (n = 18), thorax (n = 14), paravertebral space (n = 8), head and neck (n = 6), and an unknown primary site (n = 4). Most primary tumors manifested as large and bulky soft-tissue masses (mean size, 9.0 cm; range, 1.3-23.0 cm), frequently invading adjacent organs (45.6%) and showed heterogeneous enhancement (73.7%), a well-defined (66.7%) margin, and partial necrosis/cystic degeneration (81.9%). Notably, 29 patients had metastatic disease detected at their initial diagnosis. The most frequent site of metastasis was lymph nodes (75.9%), followed by bone (31.0%), lung (20.7%), abdominal solid organs (13.8%), peritoneum (13.8%), pleura (6.9%), and brain (3.4%). Conclusion: Primary tumors of EES can occur anywhere and mostly manifest as large and bulky, soft-tissue masses. Lymph nodes are the most frequent metastasis sites.

Keywords

Acknowledgement

Supported by : National Research Foundation of Korea (NRF)

References

  1. Cote GM, Choy E. Update in treatment and targets in Ewing sarcoma. Hematol Oncol Clin North Am 2013;27:1007-1019 https://doi.org/10.1016/j.hoc.2013.07.001
  2. Jain S, Xu R, Prieto VG, Lee P. Molecular classification of soft tissue sarcomas and its clinical applications. Int J Clin Exp Pathol 2010;3:416-428
  3. Murphey MD, Senchak LT, Mambalam PK, Logie CI, Klassen-Fischer MK, Kransdorf MJ. From the radiologic pathology archives: ewing sarcoma family of tumors: radiologic-pathologic correlation. Radiographics 2013;33:803-831 https://doi.org/10.1148/rg.333135005
  4. Javery O, Krajewski K, O'Regan K, Kis B, Giardino A, Jagannathan J, 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-W1022 https://doi.org/10.2214/AJR.11.6667
  5. Krasin MJ, Davidoff AM, Rodriguez-Galindo C, Billups CA, Fuller CE, Neel MD, et al. Definitive surgery and multiagent systemic therapy for patients with localized Ewing sarcoma family of tumors: local outcome and prognostic factors. Cancer 2005;104:367-373 https://doi.org/10.1002/cncr.21160
  6. Womer RB, West DC, Krailo MD, Dickman PS, Pawel BR, Grier HE, et al. Randomized controlled trial of interval-compressed chemotherapy for the treatment of localized Ewing sarcoma: a report from the Children's Oncology Group. J Clin Oncol 2012;30:4148-4154 https://doi.org/10.1200/JCO.2011.41.5703
  7. von Mehren M, Randall RL, Benjamin RS, Boles S, Bui MM, Casper ES, et al. Soft tissue sarcoma, version 2.2014. J Natl Compr Canc Netw 2014;12:473-483 https://doi.org/10.6004/jnccn.2014.0053
  8. Paulussen M, Bielack S, Jurgens H, Casali PG; ESMO Guidelines Working Group. Ewing's sarcoma of the bone: ESMO clinical recommendations for diagnosis, treatment and follow-up. Ann Oncol 2009;20 Suppl 4:140-142
  9. Somarouthu BS, Shinagare AB, Rosenthal MH, Tirumani H, Hornick JL, Ramaiya NH, et al. Multimodality imaging features, metastatic pattern and clinical outcome in adult extraskeletal Ewing sarcoma: experience in 26 patients. Br J Radiol 2014;87:20140123 https://doi.org/10.1259/bjr.20140123
  10. Jawad MU, Cheung MC, Min ES, Schneiderbauer MM, Koniaris LG, Scully SP. Ewing sarcoma demonstrates racial disparities in incidence-related and sex-related differences in outcome: an analysis of 1631 cases from the SEER database, 1973-2005. Cancer 2009;115:3526-3536 https://doi.org/10.1002/cncr.24388
  11. Kumar S, Pack S, Kumar D, Walker R, Quezado M, Zhuang Z, et al. Detection of EWS-FLI-1 fusion in Ewing's sarcoma/peripheral primitive neuroectodermal tumor by fluorescence in situ hybridization using formalin-fixed paraffin-embedded tissue. Hum Pathol 1999;30:324-330 https://doi.org/10.1016/S0046-8177(99)90012-6
  12. Bernstein M, Kovar H, Paulussen M, Randall RL, Schuck A, Teot LA, et al. Ewing's sarcoma family of tumors: current management. Oncologist 2006;11:503-519 https://doi.org/10.1634/theoncologist.11-5-503
  13. El Weshi A, Allam A, Ajarim D, Al Dayel F, Pant R, Bazarbashi S, et al. Extraskeletal Ewing's sarcoma family of tumours in adults: analysis of 57 patients from a single institution. Clin Oncol (R Coll Radiol) 2010;22:374-381 https://doi.org/10.1016/j.clon.2010.02.010
  14. Applebaum MA, Goldsby R, Neuhaus J, DuBois SG. Clinical features and outcomes in patients with Ewing sarcoma and regional lymph node involvement. Pediatr Blood Cancer 2012;59:617-620 https://doi.org/10.1002/pbc.24053
  15. Lee JA, Kim DH, Cho J, Lim JS, Koh JS, Yoo JY, et al. Treatment outcome of Korean patients with localized Ewing sarcoma family of tumors: a single institution experience. Jpn J Clin Oncol 2011;41:776-782 https://doi.org/10.1093/jjco/hyr033

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