1.7kg Rare Embryonal Sarcoma of the Liver in a 74-Year-Old: A Case Report and Literature Review

Abstract

Introduction: Undifferentiated embryonal sarcoma (UESL) of the liver is an extremely rare, highly aggressive, malignant tumour of which less than 100 cases have been reported worldwide. It has a peak incidence of 6-10 years with less than 20 reported cases in adults >40 years. Method: Data was collected on a 74-year-old male with a background of asbestos exposure, smoking and basal cell carcinoma who presented as an emergency admission with abdominal pain, weight loss and haematemesis. A literature review was conducted briefly during his admission to identify management plans for such sarcomas, and completed in detail after his emergency treatment. His Computed Tomography (CT) abdomen&pelvis showed a heterogenous, multilocular mass within the omentum, anterior to the stomach and colon; arising from hepatic segments II and III. The patient underwent an emergency laparotomy (+left lateral hepatectomy). Macroscopically, an 18 x 14 x 7 cm cystic tumour weighing 1.7 kg with a cut surface showing necrotic and haemorrhagic material was found. Microscopically, a high-grade tumour with a primitive mesenchymal appearance comprising spindle cells, high mitotic activity (>20/10 mitoses per high power field) and PAS positive eosinophilic, hyaline globules was described in keeping with a primary embryonal sarcoma of the liver. Results: The patient was discharged on post-operative day 5 with no mortality/morbidity and 6month surveillance scan was clear. Conclusion: UESL in adults represents a diagnostic and therapeutic challenge. A significant improvement in survival has been noted with a combined treatment modality of complete surgical resection with or without adjuvant chemotherapy but further research is needed.