Embryonal sarcoma of the liver

Pitfalls in the Surgical Management of Undifferentiated Sarcoma of the Liver and Benefits of Preoperative Chemotherapy

“…2,6 In our experience, and in the literature, children younger than 1 year, or older than 10 years can also be diagnosed with USL. 1,3,5 Some USL in infants are believed to arise from benign mesenchymal hamartoma, which happened once in our series. 13 Imaging: US/CT/MRI could show a mixed cystic and solid with or without intra tumoral bleeding.…”

“…Since its first pathologic description in 1978 by Stocker and Ishak, and its reported 38% overall survival (OS), 1 progress has been made with the help of multimodal therapies: chemotherapy, surgery, radiotherapy, and even primary or rescue LT, with reported OS ranging from 50 to 100%. [2][3][4][5][6][7][8][9] Because of its mixed and cystic appearance, possible intratumoral bleeding, rupture, and the lack of biologic markers, these tumors may be mistaken as mesenchymal hamartoma, hepatic hematoma, hepatoblastoma, which may lead to urgent and incomplete resection or inappropriate preoperative management. 5,10 In Europe or in America, USLs are treated according to the soft tissue sarcoma group protocols, which may include optional neoadjuvant chemotherapy, complete surgical resection, and adjuvant chemotherapy based on cyclo/ ifosfamide, vincristine, dactinomycin, plus or minus doxorobucin, and radiotherapy in case of incomplete resection or rupture (clinicaltrial.gov NCT00379457).…”

“…Some studies, however, advocate primary surgery as a standard approach. 4 Despite, an increasing number of descriptive publications on this rare entity, [3][4][5]9 none addressed the role of neoadjuvant chemotherapy, and the importance of good recognition of USL before taking any surgical action.…”

Undifferentiated Embryonal Sarcoma of the Liver in an Adult: Case Report

“…1 Generally, non-specific clinical signs such as weight loss, nausea, fatigue, vomiting, right upper quadrant pain and jaundice can be seen. 5,6 UES is not related to hepatic cirrhosis, and laboratory findings are non-specific. In CT examination, it has usually large solitary multi-cystic nature showing septation areas of necrosis and hemorrhage and well-defined cyst .…”

“…While it is often located in the right lobe it may also be seen in the left lobe and in the bilobar place. 6 Approximate tumor size is 10 cm. Tumor may have pseudocapsule.…”