Histologic Features and Prognosis of Spinal Intradural Extramedullary Ewing Sarcoma

Case Report, Literature Review, and Analysis of Prognosis

Hirokazu Takami, Ravi Kumar, Desmond A. Brown, William E. Krauss

Research output: Contribution to journalArticle

2 Citations (Scopus)

Abstract

Background: Ewing sarcoma is a malignant neoplasm that primarily involves extremity long bones, the pelvis, and soft tissues of children. The intradural extramedullary space is an uncommon location for Ewing sarcoma, and there is therefore a paucity of information regarding the features and natural history of this particular presentation. There is controversy regarding preoperative diagnosis, surgical management, and postoperative care, and there are no accepted standards. Methods: Herein we present a 61-year-old man with an L1-3 intradural extramedullary Ewing sarcoma who underwent gross total resection of the tumor via laminectomy. Pathologic analysis revealed a diagnosis of Ewing sarcoma based on the characteristic immunohistochemistry and the presence of EWSR1-FLI1 fusion transcript by reverse-transcription polymerase chain reaction. Results: There have been 28 cases of intradural extramedullary Ewing sarcoma reported in the literature. Out of 29 cases including the current case, 18 cases (62.1%) were men. The median and mean ages were 32 and 34.0 years, respectively, with a peak age group of 10–20 years. Twenty-two of 29 (72.4%) cases involved the lumbar spine, followed by the cervical spine (n = 7, 24.1%). Long-term prognosis was poor, with 1-, 2-, 3-, and 5-year progression-free survival rate of 75.0%, 56.3%, 37.5%, and 18.8%, respectively. The 1-, 2-, 3-, and 5-year overall survival rate was 89.5%, 80.5%, 80.5%, and 43.0%, respectively. Conclusions: Herein, we present imaging and pathologic findings of the case with review of the literature.

Original languageEnglish (US)
JournalWorld Neurosurgery
DOIs
StateAccepted/In press - Jan 1 2018

Fingerprint

Ewing's Sarcoma
Spine
Survival Rate
Laminectomy
Postoperative Care
Natural History
Pelvis
Reverse Transcription
Disease-Free Survival
Neoplasms
Extremities
Age Groups
Immunohistochemistry
Bone and Bones
Polymerase Chain Reaction

Keywords

  • Ewing sarcoma
  • EWSR1-FLI1 fusion
  • Literature review
  • Rare diseases
  • Reverse-transcription polymerase chain reaction
  • Spinal intradural extramedullary tumors

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology

Cite this

Histologic Features and Prognosis of Spinal Intradural Extramedullary Ewing Sarcoma : Case Report, Literature Review, and Analysis of Prognosis. / Takami, Hirokazu; Kumar, Ravi; Brown, Desmond A.; Krauss, William E.

In: World Neurosurgery, 01.01.2018.

Research output: Contribution to journalArticle

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abstract = "Background: Ewing sarcoma is a malignant neoplasm that primarily involves extremity long bones, the pelvis, and soft tissues of children. The intradural extramedullary space is an uncommon location for Ewing sarcoma, and there is therefore a paucity of information regarding the features and natural history of this particular presentation. There is controversy regarding preoperative diagnosis, surgical management, and postoperative care, and there are no accepted standards. Methods: Herein we present a 61-year-old man with an L1-3 intradural extramedullary Ewing sarcoma who underwent gross total resection of the tumor via laminectomy. Pathologic analysis revealed a diagnosis of Ewing sarcoma based on the characteristic immunohistochemistry and the presence of EWSR1-FLI1 fusion transcript by reverse-transcription polymerase chain reaction. Results: There have been 28 cases of intradural extramedullary Ewing sarcoma reported in the literature. Out of 29 cases including the current case, 18 cases (62.1{\%}) were men. The median and mean ages were 32 and 34.0 years, respectively, with a peak age group of 10–20 years. Twenty-two of 29 (72.4{\%}) cases involved the lumbar spine, followed by the cervical spine (n = 7, 24.1{\%}). Long-term prognosis was poor, with 1-, 2-, 3-, and 5-year progression-free survival rate of 75.0{\%}, 56.3{\%}, 37.5{\%}, and 18.8{\%}, respectively. The 1-, 2-, 3-, and 5-year overall survival rate was 89.5{\%}, 80.5{\%}, 80.5{\%}, and 43.0{\%}, respectively. Conclusions: Herein, we present imaging and pathologic findings of the case with review of the literature.",
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AB - Background: Ewing sarcoma is a malignant neoplasm that primarily involves extremity long bones, the pelvis, and soft tissues of children. The intradural extramedullary space is an uncommon location for Ewing sarcoma, and there is therefore a paucity of information regarding the features and natural history of this particular presentation. There is controversy regarding preoperative diagnosis, surgical management, and postoperative care, and there are no accepted standards. Methods: Herein we present a 61-year-old man with an L1-3 intradural extramedullary Ewing sarcoma who underwent gross total resection of the tumor via laminectomy. Pathologic analysis revealed a diagnosis of Ewing sarcoma based on the characteristic immunohistochemistry and the presence of EWSR1-FLI1 fusion transcript by reverse-transcription polymerase chain reaction. Results: There have been 28 cases of intradural extramedullary Ewing sarcoma reported in the literature. Out of 29 cases including the current case, 18 cases (62.1%) were men. The median and mean ages were 32 and 34.0 years, respectively, with a peak age group of 10–20 years. Twenty-two of 29 (72.4%) cases involved the lumbar spine, followed by the cervical spine (n = 7, 24.1%). Long-term prognosis was poor, with 1-, 2-, 3-, and 5-year progression-free survival rate of 75.0%, 56.3%, 37.5%, and 18.8%, respectively. The 1-, 2-, 3-, and 5-year overall survival rate was 89.5%, 80.5%, 80.5%, and 43.0%, respectively. Conclusions: Herein, we present imaging and pathologic findings of the case with review of the literature.

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