Multimodality imaging, particularly MRI for primary assessment and PET/CT for metastases, is crucial for managing extraskeletal Ewing sarcoma, which has a 5-year survival exceeding 80%.
Extraskeletal Ewing sarcoma
Multimodality imaging
Extraskeletal Ewing sarcoma (EES) is a rare subtype in the Ewing sarcoma family of tumors (ESFT), which also includes Ewing sarcoma of bone (ESB) and, more recently, primitive neuroectodermal tumors. Although these tumors often have different manifestations, they are grouped on the basis of common genetic translocation and diagnosis from specific molecular and immunohistochemical features. While the large majority of ESFT cases occur in children and in bones, approximately 25% originate outside the skeleton as EES. Importantly, in the adult population these extraskeletal tumors are more common than ESB. Imaging findings of EES tumors are generally nonspecific, with some variation based on location and the tissues involved. A large tumor with central necrosis that does not cross the midline is typical. Despite often nonspecific findings, imaging plays an important role in the evaluation and management of ESFT, with MRI frequently the preferred imaging modality for primary tumor assessment and local staging. Chest CT and fluorine 18 fluorodeoxyglucose PET/CT are most sensitive for detecting lung and other distant or nodal metastases. Management often involves chemotherapy with local surgical excision, when possible. A multidisciplinary treatment approach should be used given the propensity for large tumor size and local invasion, which can make resection difficult. Despite limited data, outcomes are similar to those of other ESFT cases, with 5-year survival exceeding 80%. However, with metastatic disease, the long-term prognosis is poor. ©RSNA, 2022
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Alexandra Wright
Madhura Desai
Candice W. Bolan
Radiographics
University of Wisconsin–Madison
The University of Texas MD Anderson Cancer Center
The University of Texas Health Science Center at Houston
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Wright et al. (Fri,) conducted a review in Extraskeletal Ewing sarcoma. Multimodality imaging was evaluated. Multimodality imaging, particularly MRI for primary assessment and PET/CT for metastases, is crucial for managing extraskeletal Ewing sarcoma, which has a 5-year survival exceeding 80%.
www.synapsesocial.com/papers/6a14e48f0b551a597239130b — DOI: https://doi.org/10.1148/rg.210226