Pediatric Radiology

, Volume 43, Issue 1, pp 93–102

Characteristic imaging features of desmoplastic small round cell tumour

  • Vandan Caur Arora
  • Anita P. Price
  • Stephen Fleming
  • Michael J. Sohn
  • Heather Magnan
  • Michael P. LaQuaglia
  • Sara Abramson
Original Article

DOI: 10.1007/s00247-012-2485-0

Cite this article as:
Arora, V.C., Price, A.P., Fleming, S. et al. Pediatr Radiol (2013) 43: 93. doi:10.1007/s00247-012-2485-0

Abstract

Background

Desmoplastic small round cell tumour (DSRCT) is a rare malignant neoplasm. Its radiological features have rarely been described.

Objective

To assess the CT parameters characteristic of DSRCT. We also report our experience with combined FDG PET/CT in staging and follow–up for DSRCT.

Materials and methods

The pretreatment diagnostic CT’s of 65 patients with DSRCT were evaluated. Pertinent imaging findings were catalogued, with histopathology or serial follow-up studies as reference standard. Combined FDG PET/CT examinations of 11 of these patients who underwent pretreatment imaging were also reviewed.

Results

Sixty-two patients presented with primary intra-abdominal disease; three had primary extra-abdominal tumours at presentation. The most common imaging finding of patients with intra-abdominal DSRCT was multiple peritoneal soft tissue masses, with a dominant mass in the retrovesical or rectouterine location in more than half of the cases. Forty percent had metastatic disease to the liver, lungs, spleen or bones at diagnosis. FDG PET/CT accurately detected 97.4% of all DSRCT lesions.

Conclusion

DSRCT typically presents as a large abdominopelvic mass with widespread peritoneal involvement predominantly in young males. Familiarity with its radiological features can help guide diagnosis and treatment. Functional imaging with PET/CT offers advantage over anatomical imaging for accurate disease staging.

Keywords

Desmoplastic small round cell tumoursChildren

Copyright information

© Springer-Verlag 2012

Authors and Affiliations

  • Vandan Caur Arora
    • 1
  • Anita P. Price
    • 1
  • Stephen Fleming
    • 1
  • Michael J. Sohn
    • 1
  • Heather Magnan
    • 2
  • Michael P. LaQuaglia
    • 3
  • Sara Abramson
    • 1
  1. 1.Department of RadiologyMemorial Sloan-Kettering Cancer CenterNew YorkUSA
  2. 2.Department of Pediatric OncologyMemorial Sloan-Kettering Cancer CenterNew YorkUSA
  3. 3.Department of SurgeryMemorial Sloan-Kettering Cancer CenterNew YorkUSA