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Extraskeletal Ewing Sarcoma from Head to Toe: Multimodality Imaging Review.

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Summary

Extraskeletal Ewing sarcoma (EES) is a rare cancer, often found in adults outside the bone. Early detection and multidisciplinary treatment are key for managing this Ewing sarcoma family of tumors (ESFT).

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Area of Science:

  • Oncology
  • Radiology
  • Pathology

Background:

  • Extraskeletal Ewing sarcoma (EES) is a rare subtype of Ewing sarcoma family of tumors (ESFT).
  • While ESFT commonly affects children and bone, 25% of cases originate outside the skeleton as EES, being more prevalent than bone tumors in adults.
  • EES tumors share common genetic translocations and molecular features with other ESFTs.

Purpose of the Study:

  • To review the imaging findings, diagnostic modalities, and management strategies for extraskeletal Ewing sarcoma (EES).
  • To highlight the role of imaging in the evaluation and staging of EES.
  • To discuss the treatment approaches and prognosis for EES.

Main Methods:

  • Review of imaging findings for EES across various locations.
  • Discussion of the utility of MRI, CT, and PET/CT in diagnosis and staging.
  • Analysis of current treatment protocols including chemotherapy and surgery.

Main Results:

  • EES imaging findings are generally nonspecific, often presenting as large tumors with central necrosis.
  • MRI is crucial for primary tumor assessment and local staging.
  • Chest CT and FDG PET/CT are sensitive for detecting metastases.

Conclusions:

  • A multidisciplinary approach is essential for managing EES due to potential for large size and local invasion.
  • Outcomes for EES are comparable to other ESFTs, with high survival rates for localized disease.
  • Metastatic EES carries a poor long-term prognosis.