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Characteristic imaging features of desmoplastic small round cell tumour

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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.

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Conflict of interest

The authors declare no conflicts of interest.

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Authors and Affiliations

  1. Department of Radiology, Memorial Sloan-Kettering Cancer Center, 1275 York Ave, New York, NY, 10065, USA

    Vandan Caur Arora, Anita P. Price, Stephen Fleming, Michael J. Sohn & Sara Abramson

  2. Department of Pediatric Oncology, Memorial Sloan-Kettering Cancer Center, New York, NY, USA

    Heather Magnan

  3. Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, NY, USA

    Michael P. LaQuaglia

Authors
  1. Vandan Caur Arora
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  2. Anita P. Price
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  3. Stephen Fleming
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  4. Michael J. Sohn
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  5. Heather Magnan
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  6. Michael P. LaQuaglia
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  7. Sara Abramson
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Corresponding author

Correspondence to Sara Abramson.

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Arora, V.C., Price, A.P., Fleming, S. et al. Characteristic imaging features of desmoplastic small round cell tumour. Pediatr Radiol 43, 93–102 (2013). https://doi.org/10.1007/s00247-012-2485-0

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  • DOI: https://doi.org/10.1007/s00247-012-2485-0

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