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Optimum treatment for primary intracranial Ewing sarcoma.

Sasha G Howell1,2, Kristopher A Lyon1,2, David Garrett1,2

  • 1Department of Neurosurgery, Baylor Scott & White Medical CenterTempleTexas.

Proceedings (Baylor University. Medical Center)
|July 18, 2020
PubMed
Summary

This case report details a rare adult primary intracranial Ewing sarcoma (ES) in the pineal region with spinal metastasis. It highlights current management strategies and potential treatments for this aggressive neuroectodermal tumor.

Keywords:
ChemotherapyEwing sarcomapineal region tumorradiation therapy

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Area of Science:

  • Neuro-oncology
  • Pediatric Oncology
  • Skeletal Oncology

Background:

  • Ewing sarcoma (ES) is a high-grade neuroectodermal neoplasm predominantly affecting children and young adults.
  • While typically involving bone, extraosseous ES can occur, most commonly in paravertebral regions.
  • Intracranial presentation of ES is exceptionally rare.

Observation:

  • A unique case of an adult with primary extraosseous Ewing sarcoma originating in the pineal region is presented.
  • The tumor extended into the third and fourth ventricles.
  • Multiple drop metastases were observed throughout the spine.

Findings:

  • This atypical presentation of Ewing sarcoma involved the brain and spine.
  • The case illustrates the use of contemporary chemotherapeutic and adjuvant management approaches.
  • It provides insights into potential therapeutic strategies for metastatic disease in this rare context.

Implications:

  • This report expands the understanding of rare intracranial Ewing sarcoma presentations.
  • It underscores the importance of considering ES in the differential diagnosis of pineal region tumors, even in adults.
  • The case offers valuable information for refining treatment protocols for advanced and metastatic Ewing sarcoma in atypical locations.