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Radiologic-Pathologic correlation


Two Cases of Spinal, Extraosseous, Intradural Ewing's sarcoma/Peripheral Neuroectodermal Tumor: Radiologic, Pathologic, and Molecular Analysis.

Stacey K MardekianAshish GandheMarkku MiettinenSvetlana PackMark T CurtisZiedulla Abdullaev
Departments of Pathology, and Radiology, Thomas Jefferson University Hospital, Philadelphia, PA, Laboratory of Pathology, National Cancer Institute, United States
Date of Submission: 29-Nov-2013, Date of Acceptance: 08-Jan-2014, Date of Web Publication: 30-Jan-2014.
Corresponding Author:
Corresponding Author

Mark T Curtis

E-mail: mark.curtis@jefferson.edu

Corresponding Author:
Corresponding Author

Mark T Curtis

E-mail: mark.curtis@jefferson.edu

DOI: 10.4103/2156-7514.126050 Facebook Twitter Google Linkedin

ABSTRACT


Extraosseous Ewing's sarcoma/peripheral neuroectodermal tumors (ES/PNETs) are rare neoplasms that account for approximately 10%-15% of soft tissue sarcomas in children and 5% of soft tissue sarcomas in adults. Primary spinal, extraosseous, intradural ES/PNETs are even less common. The diagnosis of ES/PNET is extremely challenging, because the tumor can have a nonspecific radiologic appearance, and the histologic features are shared by many other "small round cell tumors." Thus, ES/PNET should be included in the radiologic and pathologic differential diagnosis, even in older patients and in unusual tumor sites. We report two cases of spinal, extraosseous, intradural ES/PNETs in adults who presented with back pain. Magnetic resonance imaging revealed contrast enhancing, intradural lesions in the area of the conus medullaris. The tumor in Case 1 was partially intramedullary, while the tumor in Case 2 was exclusively extramedullary. In both cases, the radiologic and intraoperative surgical impression favored ependymoma. The diagnosis of ES/PNET was established in both cases by histopathologic, immunohistochemical, and molecular analysis.
Keywords: Ewing Sarcoma/peripheral Neuroectodermal Tumor, Intradural Spinal Neoplasms, Peripheral Neuroectodermal Tumors

Cited in 5 Documents

  1. Hirokazu Takami, Ravi Kumar, Desmond A. Brown and William E. Krauss (2018) Histologic Features and Prognosis of Spinal Intradural Extramedullary Ewing Sarcoma: Case Report, Literature Review, and Analysis of Prognosis. World Neurosurgery 115:448. doi: 10.1016/j.wneu.2018.04.015
  2. Wei Qi, Xiaofeng Deng, Tie Liu, Yingzhi Hou, Chenlong Yang, Liang Wu, Jingyi Fang, Xianzeng Tong, Jun Yang and Yulun Xu (2016) Comparison of Primary Spinal Central and Peripheral Primitive Neuroectodermal Tumors in Clinical and Imaging Characteristics and Long-Term Outcome. World Neurosurgery 88:359. doi: 10.1016/j.wneu.2015.12.033
  3. Arcot Rekha, Prabhu Purushothaman, Rufus Ranjitsingh Edwin and Anukiran Ravichandran (2016) Ewings Sarcoma-Primitive Neuroectodermal Tumour-Rare Extraosseous Presentation. CRCM 05(03):95. doi: 10.4236/crcm.2016.53017
  4. Konstantinos Paterakis, Alexandros Brotis, Anastasia Tasiou, Vasiliki Kotoula, Eftychia Kapsalaki and Marianna Vlychou (2017) Intradural extramedullary Ewing's sarcoma: A case report and review of the literature. Neurologia i Neurochirurgia Polska 51(1):106. doi: 10.1016/j.pjnns.2016.11.006
  5. Joshua T. Scantland, Mercia J. Gondim, Andrew S. Koivuniemi, Daniel H. Fulkerson and Chie-Schin Shih (2018) Primary Spinal Intradural Extraosseous Ewing Sarcoma in a Pediatric Patient: Case Report and Review of the Literature. Pediatr Neurosurg 53(4):222. doi: 10.1159/000488767

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