A novel image-based system for risk stratification in patients with desmoplastic small round cell tumor Journal Article


Authors: Saltsman, J. A. 3rd; Price, A. P.; Goldman, D. A.; Hammond, W. J.; Danzer, E.; Magnan, H.; Slotkin, E.; Tap, W. D.; Heaton, T. E.; Modak, S.; LaQuaglia, M. P.
Article Title: A novel image-based system for risk stratification in patients with desmoplastic small round cell tumor
Abstract: Background: Desmoplastic small round cell tumor (DSRCT) is an aggressive soft tissue sarcoma affecting children and young adults with 5-year overall survival (OS) of approximately 20%. Despite generally poor prognosis, long-term survival does occur. However, no evidence-based system exists to risk-stratify patients at diagnosis. Methods: We retrospectively reviewed all DSRCT cases diagnosed at our institution between January 2000 and September 2016. Demographics, diagnostic imaging, and clinical data were reviewed. Univariate and multivariate Cox proportional hazard modeling was used to evaluate associations between imaging characteristics and OS. Results: There were 130 patients (85% male; median age at presentation: 21.2 years) with confirmed DSRCT and sufficient imaging and clinical information for analysis. Median 5-year OS was 28% (95% CI: 19%–37%). In univariate analysis, shorter OS was associated with presence of liver lesions (hazard ratio [HR] 2.1, 95% CI: 1.28–3.45), chest lesions (HR 1.86, 95% CI: 1.11–3.1), and ascites (HR 1.69, 95% CI: 1.06–2.7). In multivariate analysis, liver involvement and ascites were predictive and were used to stratify risk (intermediate = no liver involvement or ascites; high = either liver involvement or ascites; very high = both liver involvement and ascites). Intermediate-risk patients had a 5-year survival of 61% (95% CI: 40%–76%) versus 16% (95% CI: 6%–29%) among high-risk patients and 8% (95% CI: 1%–29%) among very high risk patients. Conclusion: Patients with DSRCT can be risk-stratified at diagnosis based on specific imaging characteristics. Type of study: Retrospective study with no comparison group. Level of evidence: Level IV. © 2018 Elsevier Inc.
Keywords: adolescent; adult; child; cancer surgery; major clinical study; overall survival; ascites; cancer staging; cytoreductive surgery; cohort analysis; diagnostic imaging; retrospective study; high risk patient; sarcoma; anatomy; thorax injury; liver injury; desmoplastic small round cell tumor; intermediate risk patient; surgical oncology; pediatric surgery; cancer prognosis; long term survival; dsrct; human; male; female; priority journal; article
Journal Title: Journal of Pediatric Surgery
Volume: 55
Issue: 3
ISSN: 0022-3468
Publisher: W.B. Saunders Co-Elsevier Inc.  
Date Published: 2020-03-01
Start Page: 376
End Page: 380
Language: English
DOI: 10.1016/j.jpedsurg.2018.02.068
PUBMED: 29605262
PROVIDER: scopus
PMCID: PMC6126997
DOI/URL:
Notes: Article -- Export Date: 1 May 2020 -- Source: Scopus
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Citation Impact
MSK Authors
  1. Anita P Price
    39 Price
  2. Shakeel Modak
    185 Modak
  3. Heather Magnan
    27 Magnan
  4. William Douglas Tap
    176 Tap
  5. Emily Kanaya Slotkin
    22 Slotkin
  6. Debra Alyssa Goldman
    112 Goldman
  7. Todd Erin Heaton
    24 Heaton
  8. Enrico Danzer
    5 Danzer