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

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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.

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References

  1. Stocker JT, Ishak KG (1978) Undifferentiated (embryonal) sarcoma of the liver: report of 31 cases. Cancer 42:336–348

    Article  CAS  PubMed  Google Scholar 

  2. Chocarro G, Amesty MV, Hernández F et al (2013) Embryonal sarcoma of the liver. Pediatr Surg Int 29:1261–1266

    Article  CAS  PubMed  Google Scholar 

  3. Ismail H, Dembowska-Bagińska B, Broniszczak D et al (2013) Treatment of undifferentiated embryonal sarcoma of the liver in children — single center experience. J Pediatr Surg 48:2202–2206

    Article  PubMed  Google Scholar 

  4. Upadhyaya M, McKiernan P, Hobin D et al (2010) Primary hepatic sarcomas in children — a single-center experience over 19 years. J Pediatr Surg 45:2124–2128

    Article  PubMed  Google Scholar 

  5. Walther A, Geller J, Coots A et al (2014) Multimodal therapy including liver transplantation for hepatic undifferentiated embryonal sarcoma. Liver Transpl 20:191–199

    Article  PubMed  Google Scholar 

  6. Wildhaber BE, Montaruli E, Guérin F et al (2014) Mesenchymal hamartoma or embryonal sarcoma of the liver in childhood: a difficult diagnosis before complete surgical excision. J Pediatr Surg 49:1372–1377

    Article  PubMed  Google Scholar 

  7. Merli L, Mussini C, Gabor F et al (2015) Pitfalls in the surgical management of undifferentiated sarcoma of the liver and benefits of preoperative chemotherapy. Eur J Pediatr 25:132–137

    Article  Google Scholar 

  8. Roebuck DJ, Aronson D, Clapuyt P et al (2007) 2005 PRETEXT: a revised staging system for primary malignant liver tumours of childhood developed by the SIOPEL group. Pediatr Radiol 37:123–132

    Article  PubMed  Google Scholar 

  9. Kim D-Y, Kim K-H, Jung S-E et al (2002) Undifferentiated (embryonal) sarcoma of the liver: combination treatment by surgery and chemotherapy. J Pediatr Surg 37:1419–1423

    Article  PubMed  Google Scholar 

  10. Wu JT, Book L, Sudar K (1981) Serum alpha fetoprotein (AFP) levels in normal infants. Pediatr Res 15:50–52

    Article  CAS  PubMed  Google Scholar 

  11. Chung EM, Lattin GE, Cube R et al (2011) From the archives of the 457 AFIP: pediatric liver masses: radiologic-pathologic correlation. Part 458 2. Malignant tumors. Radiographics 31:483–507

  12. Buetow PC, Buck JL, Pantongrag-Brown L et al (1997) Undifferentiated (embryonal) sarcoma of the liver: pathologic basis of imaging findings in 28 cases. Radiology 203:779–783

    Article  CAS  PubMed  Google Scholar 

  13. Ros PR, Olmsted WW, Dachman AH et al (1986) Undifferentiated (embryonal) sarcoma of the liver: radiologic-pathologic correlation. Radiology 161:141–145

    Article  CAS  PubMed  Google Scholar 

  14. Qiu L-L, Yu R-S, Chen Y (2011) Sarcomas of abdominal organs: computed tomography and magnetic resonance imaging findings. Semin Ultrasound CT MR 32:405–421

    Article  PubMed  Google Scholar 

  15. Iqbal K, Xian ZM, Yuan C (2008) Undifferentiated liver sarcoma — rare entity: a case report and review of the literature. J Med Case Reports 2:20

    Article  PubMed Central  Google Scholar 

  16. Wei ZG, Tang LF, Chen ZM et al (2008) Childhood undifferentiated embryonal liver sarcoma: clinical features and immunohistochemistry analysis. J Pediatr Surg 43:1912–1919

    Article  PubMed  Google Scholar 

  17. Stringer MD, Alizai NK (2005) Mesenchymal hamartoma of the liver: a systematic review. J Pediatr Surg 40:1681–1690

    Article  PubMed  Google Scholar 

  18. Lauwers GY, Grant LD, Donnelly WH et al (1997) Hepatic undifferentiated (embryonal) sarcoma arising in a mesenchymal hamartoma. Am J Surg Pathol 21:1248–1254

    Article  CAS  PubMed  Google Scholar 

  19. O’Sullivan MJ, Swanson PE, Knoll J et al (2001) Undifferentiated embryonal sarcoma with unusual features arising within mesenchymal hamartoma of the liver: report of a case and review of the literature. Pediatr Dev Pathol 4:482–489

    Article  PubMed  Google Scholar 

  20. Shehata BM, Gupta NA, Katzenstein HM et al (2011) Undifferentiated embryonal sarcoma of the liver is associated with mesenchymal hamartoma and multiple chromosomal abnormalities: a review of eleven cases. Pediatr Dev Pathol 14:111–116

    Article  PubMed  Google Scholar 

  21. Chung EM, Cube R, Lewis RB et al (2010) From the archives of the AFIP: pediatric liver masses: radiologic-pathologic correlation part 1. Benign tumors. Radiographics 30:801–826

    Article  PubMed  Google Scholar 

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Correspondence to Flaviu Gabor.

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Gabor, F., Franchi-Abella, S., Merli, L. et al. Imaging features of undifferentiated embryonal sarcoma of the liver: a series of 15 children. Pediatr Radiol 46, 1694–1704 (2016). https://doi.org/10.1007/s00247-016-3670-3

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  • DOI: https://doi.org/10.1007/s00247-016-3670-3

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