Magnetic resonance and computed tomography imaging features of epithelioid sarcoma in children and young adults with pathological and clinical correlation: a report from Children’s Oncology Group study ARST0332

  • M. Beth McCarvilleEmail author
  • Simon C. Kao
  • Tuan V. Dao
  • Christopher Gaffney
  • Cheryl M. Coffin
  • David M. Parham
  • Andrea Hayes-Jordan
  • Sheri L. Spunt
Original Article



To correlate imaging features of epithelioid sarcoma in children and young adults enrolled in Children’s Oncology Group study ARST0332 with clinical and pathological findings.

Materials and methods

Fifteen patients (6 males; median age 16.1 years, range 6.5–24.8 years) with epithelioid sarcoma enrolled in ARST0332 had preoperative imaging (MRI, n=10; CT, n=5) that was reviewed by two radiologists who recorded numerous features including presence and percentage of tumor necrosis, presence of surrounding edema, and lymph node involvement. Discrepancies between reviewers were adjudicated by concurrent re-review. We correlated imaging findings with histological assessment of percentage tumor necrosis, proximal- vs. classic-type histology, lymph node involvement and recurrence.


Eleven patients (11/15, 73%) had proximal-type histology tumors. Ten of 14 tumors (71%) had imaging evidence of necrosis. Among the nine tumors with imaging and histological estimates of percentage necrosis, agreement was within 30% (in six tumors there was ≤10% difference between pathology and imaging). All 10 tumors imaged with MRI had surrounding edema. Four patients had biopsy-proven nodal involvement; all had necrotic nodes on imaging. There were four false-positives for nodal involvment by imaging. Twelve patients (12/15, 80%) had recurrences (local only, n=1; local and distant, n=1; distant only, n=10).


Proximal-type histology was prevalent in this young cohort with preoperative imaging. Necrosis is common in primary tumors and involved nodes. There is good agreement between histological and imaging estimates of primary tumor necrosis. Surrounding tumor edema is common in this tumor, which is known to spread along fascial planes.


Adolescents Children Computed tomography Epithelioid sarcoma Magnetic resonance imaging Young adults 



The authors thank Anne Speights and Edwina Anderson for their assistance in data collection and management, Fran Laurie and Richard Hanusik for their assistance with imaging processing and transfer from QARC, and Adriane Matthews for administrative assistance. This project was supported by U10CA98543, U10CA98413, U10CA180886, U10CA180899 and St. Baldrick’s Foundation.

Compliance with ethical standards

Conflicts of interest

Dr. Spunt received funding support from the National Cancer Institute/CureSearch as principal investigator of the study.


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of Diagnostic Imaging, MS 220St. Jude Children’s Research HospitalMemphisUSA
  2. 2.Department of RadiologyUniversity of Iowa College of MedicineIowa CityUSA
  3. 3.Department of RadiologyChildren’s Hospital of Orange CountyOrangeUSA
  4. 4.Department of RadiologyTexas Children’s HospitalHoustonUSA
  5. 5.Department of Pathology, Microbiology, and ImmunologyVanderbilt University School of MedicineNashvilleUSA
  6. 6.Department of Pathology and Laboratory Medicine, Children’s Hospital of Los AngelesUniversity of Southern California Keck School of MedicineLos AngelesUSA
  7. 7.Department of SurgeryUniversity of Texas, MD Anderson Cancer CenterHoustonUSA
  8. 8.Department of PediatricsStanford University School of MedicinePalo AltoUSA

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