Flaviu Gabor1, Stephanie Franchi-Abella2, Laura Merli3, Catherine Adamsbaum2,4,5, Daniele Pariente2. 1. Department of Pediatric Radiology, Bicêtre Hospital, 78, rue du Général Leclerc, Le Kremlin-Bicêtre, 94275, France. flaviugabor@yahoo.com. 2. Department of Pediatric Radiology, Bicêtre Hospital, 78, rue du Général Leclerc, Le Kremlin-Bicêtre, 94275, France. 3. Unit of Hepato-Biliary and Transplant Surgery, Department of Surgery and Transplantation Centre, Bambino Gesù Children's Hospital, Rome, Italy. 4. Faculty of Medicine, Paris Sud University, Le Kremlin-Bicêtre, France. 5. LTCI, CNRS, Télécom Paris Tech, Université Paris-Saclay, Paris, France.
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.
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.
Authors: Bahig M Shehata; Nitika A Gupta; Howard M Katzenstein; Charlotte K Steelman; Mark L Wulkan; Kenneth W Gow; Julie A Bridge; Brian D Kenney; Karen Thompson; Jean-Pierre de Chadarévian; Carlos R Abramowsky Journal: Pediatr Dev Pathol Date: 2010-10-06
Authors: P C Buetow; J L Buck; L Pantongrag-Brown; W H Marshall; P R Ros; M S Levine; Z D Goodman Journal: Radiology Date: 1997-06 Impact factor: 11.105