Imaging features of undifferentiated embryonal sarcoma of the liver: a series of 15 children

Pediatr Radiol. 2016 Nov;46(12):1694-1704. doi: 10.1007/s00247-016-3670-3. Epub 2016 Aug 26.

Abstract

Background: Undifferentiated embryonal sarcoma of the liver is a rare malignant mesenchymal tumour occurring mostly in children ages 6-10 years. The discrepancy between its solid appearance on US and cystic-like appearance on CT has been described.

Objective: To study the imaging particularities and similarities among our cases of undifferentiated embryonal sarcoma and to report the errors in initial diagnoses.

Materials and methods: We conducted a retrospective study of 15 children with undifferentiated embryonal sarcoma diagnosed or referred to our hospital during 1997-2015 and analysed the clinical, biological and imaging data.

Results: We identified eight boys and seven girls ages 9 months to 14 years. Ten children presented with abdominal pain. Alpha-fetoprotein was slightly increased in one. Initial US and CT had been performed for all, while additional MRI had been done in two children. Initial CT demonstrated a hypoattenuated mass in all. Rupture was seen in five and intratumoural bleeding in seven children. Tumour volumes reduced during neoadjuvant chemotherapy in 10 children.

Conclusion: Undifferentiated embryonal sarcoma might be suggested in a non-secreting unifocal tumour with well-defined borders, fluid-filled spaces on US, hypoattenuation and serpiginous vessels on CT, and if there are signs of internal bleeding or rupture on CT or MRI.

Keywords: Children; Computed tomography; Cystic mesenchymal tumour; Liver tumour; Magnetic resonance imaging; Sarcoma; Ultrasound.

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Female
  • Humans
  • Infant
  • Liver / diagnostic imaging
  • Liver Neoplasms / diagnostic imaging*
  • Magnetic Resonance Imaging*
  • Male
  • Neoplasms, Germ Cell and Embryonal / diagnostic imaging*
  • Retrospective Studies
  • Sarcoma / diagnostic imaging*
  • Tomography, X-Ray Computed*
  • Ultrasonography*