Extraskeletal Ewing sarcoma

Last revised by Laura Porter on 02 Aug 2021

Extraskeletal Ewing sarcoma (EES) is included in the Ewing sarcoma family of tumors (ESFT) along with Ewing sarcoma of bone, primitive neuroectodermal tumor (PNET), peripheral neuroepithelioma, and thoracopulmonary PNET (Askin tumor). When compared with Ewing sarcoma of bone, extraskeletal Ewing sarcoma is rare.

EES is generally found in younger patients (in 85% between the age of 20 months and 30 years). Osseous Ewing sarcoma is estimated to be about 5 to 6 times more prevalent than EES 2, however, in adults at least half of primary cases are extraskeletal 1.

When compared with Ewing sarcoma of bone, EES displays several differences 8-11:

  • no distinct male predilection, but instead a more equal distribution between sexes
  • slightly older age group than found in osseous Ewing sarcoma by 5-10 years, the average age is 20 years of age
  • the trunk is affected more frequently than the lower extremities

A rapidly growing, solitary, superficial or deep mass in the soft tissues is generally seen between 5 and 10 cm at the initial presentation 8. Depending on where the tumor is located, adult patients generally present with a painless mass or vague abdominal or chest pain 1. If the mass is seen to be paravertebral, the patient can present with symptoms of cord compression 10. Between 35% and 43% of adult patients have metastatic disease at presentation 3, 5. The most common site of metastasis is the lung 4.

  • paravertebral region (~30%)
  • lower extremities (~25%)
  • chest wall (~20%)
  • retroperitoneum (~10%)
  • pelvis and hip (~10%)
  • upper extremities (3%) 6, 8, 10, 12, 13
  • intracranial 18

Extraskeletal Ewing sarcoma imaging features are generally nonspecific.

  • normal appearance
  • large soft-tissue mass (50%)
  • calcification (25%)
  • presence of adjacent bone erosion, cortical thickening, osseous invasion, or periosteal reaction 2

  • nonspecific soft-tissue mass of similar attenuation to muscle (87%)
  • low attenuation can be due to hemorrhage or necrosis
  • calcification (25-30%)
  • osseous involvement of bone surface with cortical erosion or periosteal reaction (40%) 14

A common feature is disorganized high-flow vascular channels with low signal intensity with all pulse sequences (90%). Additionally, a pseudocapsule with somewhat well-defined margins can be seen (36%) 14.

  • T1
    • soft tissue mass with heterogenous signal intensity (~90%)
    • similar intensity to skeletal muscle
  • T1 C+ (Gd)
    • heterogeneous but prominent enhancement
  • T2
    • intermediate to high signal intensity (100%)
    • fluid levels may be evident

Bone scintigraphy and FDG PET shows increased radionuclide uptake 12.

  • most frequently hypoechoic15
  • increased Doppler blood flow14

Local disease starts with neoadjuvant chemotherapy as the standard of care before definitive radiation or surgery. EES is aggressive with a high propensity for local recurrence and distant metastasis. Recurrence occurs in ~30% (range 22-36%) 3, 4.  Adult outcomes may be worse than in children, because of the inability of adults to tolerate aggressive chemotherapy16, 17.

General imaging differential considerations include

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Cases and figures

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